Editorial Full text online at http://www.jiaps.com
47 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 48

Review Article Full text online at http://www.jiaps.com
Anatomical basis for Wilms tumor surgery
R. B. Tröbs
Department of Pediatric Surgery, Catholic Foundation Marien Hospital Herne, Ruhr-University of Bochum, Herne,
Germany
Address for correspondence: Dr. Ralf-Bodo Tröbs, Department of Paediatric Surgery, Catholic Foundation Marien Hospital Herne,
Ruhr-University of Bochum, D-44627 Herne, Widumer Str. 8, Germany. E-mail: ralf-bodo.troebs@marienhospital-herne.de
ABSTRACT
Wilms tumor surgery requires meticulous planning and sophisticated surgical technique. Detailed anatomical
knowledge can facilitate the uneventful performance of tumor nephrectomy and cannot be replaced by
advanced and sophisticated imaging techniques. We can define two main goals for surgery: (1) exact staging
as well (2) safe and complete resection of tumor without spillage. This review aims to review the anatomical
basis for Wilms tumor surgery. It focuses on the surgical anatomy of retroperitoneal space, aorta, vena cava
and their large branches with lymphatics. Types and management of vascular injuries are discussed.
KEY WORDS: Aorta, gerota’s fascia, Wilms tumor, inferior mesenteric artery, superior mesenteric artery, surgery,
vascular injury
DOI: *****
InTRodUcTIon stage. Thus, less overall treatment was required. These
findings were confirmed by the Third Wilms Tumor
The word surgery has Greek roots and means nothing (WT) Study of the United Kingdom Children’s Cancer
more than “handwork.” This handwork is largely based Study Group.[1,2]
on the science of dissection known as “anatomy.” From
this point of view, pediatric surgery is anatomy applied Removal of a large nephroblastoma can still be a
to the living body of a child. The aim of my presentation demanding undertaking. Large trials in WT surgery
is to review the anatomical basis of Wilms tumor (WT) have identified various anatomy-based complications.
surgery. However, institutional series and case reports give
additional important details. Surgical problems in
Treatment of children with a nephroblastoma is an WT surgery can involve the staging and removal of
interdisciplinary undertaking. In the last 40 years, the nephroblastoma itself, as well as hemorrhage
attempts have been made to decrease the morbidity and injuries to vessels and adjacent structures. The
of treatment while maintaining an excellent survival nomenclature of anatomical structures and eponyms
rate. The pediatric surgeon or urologist plays a key used in this paper follows the recommendations of the
role within the team. In particular, the duration and Federative Committee on Anatomical Terminology.[3]
amount of chemotherapy and radiation therapy can be
influenced by the surgical technique. Zones, spaces and planes
We can define two main goals for surgery (1) exact Wilms Tumor is an embryonic renal tumor located
staging and (2) safe and complete resection of the tumor within the retroperitoneal space. Recently, Skandalakis
without spillage. The Société Internationale d’Oncologie wrote “This space is a vast territory lacking any accurate
Pédiatrique (SIOP) investigators pioneered the concept
of pretreatment of renal tumors before nephrectomy.
The SIOP trials showed that pre-treatment resulted
Presentation held at the 34th Annual Conference of the Indian Association
in safer surgery, reduced tumor rupture rates, and
of Pediatric Surgeon, December 5th, Guwahati, Assam
an increased portion of children with a lower tumor
49 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Tröbs: Basis for Wilms tumor surgery
knowledge and accepted map”.[4] clearly shows the existence of three renal compartments
in a child. The ruptured right lower pole is shown
According Farthmann et al. (1989) the retro peritoneum with separated blood collections in the perirenal space
can be divided into three zones and four parts: the (asterisks) as well as in both pararenal spaces (stars).
central zone (containing the aorta, inferior vena cava, The rate of perirenal infiltration of nephroblastomas
pancreas, and duodenum); two lateral zones (the remains unclear. To obtain clear margins, a rim of
kidneys, ureters, and ascending/descending colon), healthy tissue including covering fibrous tissue and
and the pelvic zone (rectosigmoid, iliac vessels, and fat has to be resected with the tumor. Strict orientation
urogenital organs).[4,5] between different retroperitoneal planes is required to
achieve this. Standard textbooks recommend that the
The retro-peritoneal space is defined as the space
tumor plane should be developed outside the perirenal
between the posterior parietal peritoneum and
fascia.[7-10] In other words, resection of the tumor-bearing
the transversalis fascia. This anatomic highway is
kidney during radical uretero-nephrectomy also requires
responsible for the dissemination of pathologic entities
removal of the intact Gerota’s and Zuckerkandl’s fascia
originating in the retro peritoneum, and is comprised
that cover the kidney. This procedure is known as a
of connective tissue layers.
perifascial nephrectomy. The uretero-nephrectomy
Gerota’s fascia is often used as a general term to specimen shown in Figure 3 demonstrates an upper
describe renal fascia. In 1883, Zuckerkandl described pole tumor removed with a sheath of surrounding fat
the posterior renal fascia but did not recognize the and perirenal fascia.
presence of the anterior layer of renal fascia. In his work
Beiträge zur Kenntnis des Befestigungsapparates der At the upper pole of the kidney, a fascial septum
Niere, the Romanian Gerota documented the presence separates the adrenal gland from the kidney.[4] The
of the anterior fascia and clearly assigned Zuckerkandl’s
name to the posterior fascia.[6]
Three compartments of retro peritoneal space are
related to the kidney: the perirenal space as well as the
anterior and posterior pararenal spaces [Figure 1]. The
perirenal space is the home of the kidneys. The renal
fascia, a collagenous connective tissue of mesodermal
origin that envelopes the kidney, is responsible for this
compartmentalization. The kidney is enveloped by the
anterior and posterior laminae of the renal fascia and
fatty tissue inside and outside the fascia. There is some
medial fixation with the adventitial covering of renal
vessels and aorta or inferior vena cava (IVC).[4]
Figure 2: Peri- and pararenal hematoma after traumatic renal rupture
(CT Scan)
For example, Figure 2 depicts a case of traumatic renal
rupture. The distribution of perirenal blood and urine
Figure 1: Peri- and pararenal compartments (ap – Anterior, pp – Figure 3: Nephrectomy specimen. Upper pole tumor covered by
Posterior Pararenal) Gerota’s fascia
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 50

Tröbs: Basis for Wilms tumor surgery
adrenal gland for small or lower pole tumor can be another artery is revealed underneath that could be the
spared.[9] However, in many cases, when the tumor is superior mesenteric artery, aorta, or left or right renal
large and adrenal gland is attached it must be removed artery. This artery should not be ligated until its exact
to achieve adequate margins. identity has been established.[20,21] When in doubt,
this can be done by cross-clamping the vessel with a
Vessels – Keys To sUccessfUl sURgeRy vascular clamp. It can be clearly stated that attempts
at early ligation of the hilar vessels cannot be justified
Generally, three types of vessels have to be taken into until the renal vasculature is clearly identified.
account: arteries, veins, and lymphatic vessels. Modern
imaging techniques allow clear outlining of many The vessels most at risk during excision of right renal
aspects of the visceral blood supply. The aorta and its tumors are IVC and contralateral renal vein. In large
branches are quite small in infants and toddlers; thus right-sided tumors, the IVC enters the tumor mass and
they can be mistaken for the renal artery. In a three- is hidden from vision. Under this circumstance, the
year-old child the aorta at the level of the kidneys has a right renal artery is in close proximity to the left renal
diameter of 6 to 7 mm and the renal artery measures 2.8 vein, which can thus be damaged. [20] If unrecognized,
mm.[11] WT surgery is a safe and partially standardized such damage can lead to venous infarction and loss of
procedure. However, major bleeding and vascular renal function.
injuries can occur. The frequency of vascular injuries
has been reported as 1.5% in the NWTS-3 and 4.[12, 13]
Severe hemorrhage occurs at a higher rate. Further, there
aoRTa and ITs BRanches
exists a hidden surgical mortality.[14]
Modern imaging techniques allow noninvasive clear
The vascular pedicle of both kidneys is an important outlining of the visceral blood supply. The angio-MR
anatomical area and should be analyzed. The left renal [Figure 4] demonstrates the arterial branches of the aorta
vein passes in front of the aorta in the majority of in a child with a left-sided WT.
individuals. The right artery reaches the kidney behind
the IVC. However, variations in surgical importance can It shows that the coeliac trunk (Coe T) and SMA
occur. In up to three per cent cases, a retro aortic left originate from the aorta in close proximity to the origins
renal vein has to be taken into account. Circum aortic of the renal arteries. The distance between the origins
veins also occur in a relevant number of patients. [15] of these aortal tributaries can be 1 cm or less.[22]
Ligature and cutting of the large renal vessels is one of For surgical practice it is helpful to arrange the aortic
the most important steps of nephrectomy. Early ligature
of the vein has the theoretical advantage of preventing
hematogenous tumor spread. [8, 16, 17] However, this was
never confirmed by a prospective trial. On the other
hand, primary ligature of the artery is recommended
for prevention of tumor swelling and rupture. [7,10,18]
There is no consensus in literature regarding the
sequence of vessel ligature. Selected international
standard publications recommend early control of the
hilum. However, this is often not feasible with extremely
large tumors; mobilization of the tumor mass must
first occur to allow exposure of the hilar vessels.[16,19]
The current SIOP 2001/German Society of Pediatric
oncology protocol recommends initial ligature of the
artery. When feasible, we follow this recommendation.
During removal of left renal tumors, damage to the aorta,
superior mesenteric artery (SMA), and right renal artery
has been reported to occur. These vessels are in close
proximity to the tumor mass, and if the aorta and IVC
separate by tumor or lymphatic infiltration, they are
threatened during removal of the left kidney. The left Figure 4: Major Branches of Abdominal Aorta (MRI). R RA – Right
renal vein is usually identified first. Once it is divided Renal Artery, L RA - Left Renal Artery
51 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Tröbs: Basis for Wilms tumor surgery
branches in the three planes they occupy [Figure 5]: (1) constitutes an arch from the left transverse colon to the
blood supply to the gastrointestinal tract in front of the sigmoid colon secondary to the marginal artery.[26,27]
aorta; (2) to three paired glands on both sides and (3) to
diaphragm and the four lumbar arteries. [4] VenoUs dRaInage
Iatrogenic injury to the aorta and its branches has The left and right kidneys show a difference in venous
been inconstantly reported. These reports indicate that drainage. In right-sided nephroblastoma with cava
patients with left sided large tumors are at particular risk thrombus, ligature and dissection of the left renal
for this type of injury. Although attempts were always vein is possible. In the majority of patients, sufficient
made to repair the intraoperative vascular injury, three venous collaterals via the phrenic, adrenal, hemiacygos,
of six patients described in literature died as a result of testicular, lumbal and ureteral veins are present
the vessel injury. [23, 24] All these children had left sided [Figure 7].[28] In contrast, the anatomy of the right kidney
nephroblastoma; four cases had injuries to the SMA, does not allow this maneuver.[29]
one had both SMA and coeliac trunk, and one the aorta
injury. It has been reported that after unnoticed ligation In humans, embryogenesis of the renal and post renal
of SMA, though the bowel may initially appear viable, segments of IVC involves the sequential appearance of
full thickness necrosis develops later. When in doubt, a three paired venous channels: the posterior cardinals
Doppler flow study can provide essential information. as well as the sub cardinal and supra cardinal
veins.[30] The development of anastomotic channels
Lacerations of the SMA require surgical repair.[23,25] between these channels and subsequent regression of
Fullen and coworkers attempted to classify SMA segments of this system might be impaired, leading to
injuries, and found that at least injuries proximal of variants like the persistence of the left sub cardinal
the middle colic artery urgently require repair, whereas
those of the distal part can be tolerated without ischemia
or with ischemia of only a small segment of the
bowel.[25] When possible, end-to-end anastomosis of the
vessel with or without venous interposition or end-to-
side anastomosis to the aorta is the procedure of choice.
In contrast to SMA, dissection of the stem of inferior
mesenteric artery (IMA) is commonly tolerated without
disastrous consequences. Generally the marginal artery,
also known as arcade of Riolan arch, (synonymously
marginal artery of Drummond), sufficiently connects
SMA with IMA [Figure 6]. However, only small
collaterals exist at the splenic flexure.[22] In addition,
the ascending arch of the left colic artery (AALCA) is
present in at least two third of cases. This arterial branch
Figure 6: Marginal Arcade and AALCA Connect SMA with IMA.[26,27]
Figure 5: Three Planes of Aortic Branches.[4] Figure 7: Venous Drainage of Right Kidney
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 52

Tröbs: Basis for Wilms tumor surgery
vein leading to a high confluence of the large veins. Surg Gynecol Obstet 1992;175:507-14.
13. Ritchey ML, Shamberger RC, Haase G, Horwitz J, Bergemann T,
Other rare anomalies of the IVC include duplication,
Breslow NE. Surgical complications after primari nephrectomy
left-sidedness of the vein and interruption of the IVC for Wilms tumor: report from the National Wilms Tumor Study
with azygos/ hemiazygos continuation.[15,30,31] Group. J Am Coll Surg 2001;192:63-8.
14. Stehr M, Deilmann K, Haas RJ, Dietz HG. Surgical complications
Lack of recognition of retro peritoneal venous anomalies in the treatment of Wilms tumor. Eur J Pediatr Surg 2005;15:414-9.
15. Aljabri B, MacDonald PS, Satin R, Stein LS, Obrand DI, Steinmetz
can have potentially disastrous consequences because OK. Incidence of major venous and renal anomalies relevant to
anomalous venous structures tend to be dilated and aortoiliac surgery as demonstrated by computed tomography. Ann
tortuous. Intraoperative lacerations can cause life- Vasc Surg 2001;15:615-8.
16. Ehrlich RM. Complications of Wilms tumor surgery. Urol Clin
threatening hemorrhage.
North Am 1983;10:399-406.
17. Leape LL, Breslow NE, Bishop HC. The surgical treatment of
lymph nodes and Vessels Wilms tumor: results of the National Wilms Tumor Study. Ann
Surg 1978;187:351-6.
18. Nephroblastom SIOP 2001/GPOH. Therapieoptimierungsstudie.
Surgeons might not be able to tell from gross inspection 19. Shamberger RC. Renal Tumors. In: Carachi R, Grosfeld JL, Azmy
whether or not lymph nodes contain tumors.[32] Random AF, editors. The Surgery of childhood tumors. 2nd ed. Berlin,
sampling of nodes in each area should be performed Heidelberg: Springer; 2008. p. 171-99.
20. Gough DCS. Wilms Tumor. In: Gearhart JP, Rink RC, Mouriquand
since occult lymph node metastases affect the tumor
PD, editors. Pediatric urology. Philadelphia, London, New York:
stage and therapy. Failure to sample lymph nodes WB Saunders; 2001. p. 875-84.
results in a poorer prognosis. Postoperative chylous 21. Katmawi-Sabbagh S, Cuckow P. Mistaken ligation of the right
ascites has been infrequently described after WT renal artery: a risk in the surgical management of massive left-
sided Wilms tumor. J Indian Assoc Pediatr Surg 2007;12:156-7.
nephrectomy. [33,34] The usual causes are radical
22. Loeweneck H, Feifel G. Bauch. In, Lanz T v, Wachsmuth W, editors.
lymphadenectomy and operative injury to the cisterna Praktische Anatomie. Vol. 2. Berlin, Heidelberg, New York: 1993.
chyli or their major tributaries. Resection of the supra 23. Ritchey ML, Lally KP, Haase GM, Shochat SJ, Kelalis PP. Superior
hilar nodes carries a particular risk of injuring the mesenteric artery injury during nephrectomy for Wilms tumor. J
Pediatr Surg 1992;27:612-5.
cisterna. Meticulous lymphostasis is of paramount 24. Roth H, Weirich A, Ludwig R, Daum R, Zimmermann H. Die
importance in such cases of lymph node removal. Resektion des Nephroblastoms: Probleme und Komplikationen
– Auswertungen zur Nephroblastomstudie SIOP 9/GPOH.
Langenbecks Arch Chir suppl 1996;113:1078-83.
RefeRences 25. Fullen WD, Hunt J, Altemeier WA. The clinical spectrum of
penetrating injury to the superior mesenteric arterial circulation.
1. De Kraker J, Jones KP. Treatment of Wilms tumor: an international J Trauma 1972;12:656-63.
perspective. J Clin Oncol 2005;23:3156-7. 26. Lange JF, Komen N, Akkerman G, Nout E, Horstmannshoff H,
2. Tröbs RB, Hänsel M, Friedrich T, Bennek J. A 23-year-experience Schlesinger F, et al. Riolan’s arch: confusing, misnomer, and
with malignant renal tumors in infancy and childhood. Eur J obsolete. An literature survey of the connection(s) between the
Pediatr Surg 2001;11:92-8. superior and inferior mesenteric arteries. Am J Surg 2007;193:742-8.
3. Federative Committee on Anatomical Terminology. Terminologica 27. Lange MM, Buunen M, van de Velde CJ, Lange JF. Level of arterial
anatomica. Stuttgart, New York: Thieme; 1998. p. 163-6. ligation in rectal cancer surgery: low preferred over high tie. A
4. Skandalakis JE, Skandalakis LJ, Zoras O. Posterior aspect of the review. Dis Colon Rectum 2008;51:1139-45.
abdominal viscera and retroperitoneum. In, Merlini MP, Martin 28. Giuliani L. Radikale Chirurgie der Nierentumoren. In: Hohenfellner
RF, editors. Multiorgan resection for cancer. Stuttgart, New York: R, editor. Ausgewählte urologische OP-Techniken. Stuttgart, New
Thieme; 2006. p. 26-46. York: Thieme; 1994. p. 1.27-1.48.
5. Farthmann EH, Kirchner R, Fraedrich G. Organ- und 29. Narasimhan KL, Chowdhary SK, Marwaha RK, Rao KL. Renal
Gefäßverletzungen des zentralen Retroperitoneums. Chirurg failure following unilateral nephrectomy in Wilms tumor. Ind J
1989;60:657-64. Urol 2000;16:157-60.
6. Chesbrough RM, Burkhard TK, Martinez AJ, Burks DD. Gerota 30. Mathews R, Smith PA, Fishman EK, Marshall FF. Anomalies of
versus Zuckerkandl: The renal fascia revisited. Radiology the inferior vena cava and renal veins: embryologic and surgical
1989;173:845-6. considerations. Urology 1999;53:873-80.
7. Sigel A, Chlepas S. Nephroblastoma. In, Eckstein HB, Hohenfellner 31. Zucconi WB, Guelfguat M, Solounias N. Approach to the
R, Williams DI, editors. Surgical pediatric urology. Philadelphia, educational opportunities provided by variant anatomy,
London, Toronto: WB Saunders, Georg Thieme; 1977. p. 199-209. illustrated by discussion of a duplicated inferior vena cava. Clin
8. Othersen BH, Hebra A, Tagge EP. Nephroblastoma and other renal Anat 2002;15:165-8.
tumors. In: Carachi R, Azmy AF, Grosfeld JL, editors. The surgery 32. Othersen HB Jr, DeLorimer A, Hrabovsky E, Kelalis P, Breslow
of childhood tumors. 1st ed. London, Sydney, Auckland: Arnold; N, D’Angio GJ. Surgical evaluation of lymph node metastases in
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9. Ross JH, Kay R. Surgical Considerations for patients with Wilms 33. Eberl W, Kwee T, Mau G. Chylous ascites after tumor nephrectomy
tumor. Seminars Urol Oncol 1999;17:33-9. with retroperitoneal lymphadenectomy (article in German).
10. Duffy PG, Cuckow P. In: Spitz L, Coran AG, editors. Operative Monatsschr Kinderheilkd 1991;139:47-50.
Pediatric Surgery. 6th ed. London: Hodder Arnold; 2006. p. 703-8. 34. Weiser AC, Lindgren BW, Ritchey ML, Franco I. Chylous
11. Dragendorff O. Die Gefäße des Stammes und der Gliedmaßen. In: ascites following surgical treatment for Wilms tumor. J Urol
Peter K, Wetzel G, Heiderich F, editors. Handbuch der Anatomie 2003;170:1667-9.
des Kindes. Vol. 2. München: Verlag JF Bergmann; 1938. p. 323-98.
12. Ritchey ML, Kelalis PP, Breslow N, Etzioni R, Evans I, Haase GM, Source of Support: Nil, Conflict of Interest: None declared.
et al. Surgical complications after nephrectomy for Wilms tumor.
53 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Review Article Full text online at http://www.jiaps.com
Psychological issues in cleft lip and cleft palate
Avinash De Sousa, Shibani Devare, Jyoti Ghanshani
Consultant Psychiatrist, Address Missing?????
Address for correspondence: Dr. Avinash De Sousa, Carmel, 18, St. Francis Avenue, Off S.V. Road, Santacruz West,
Mumbai 400-054, Maharashtra, India. E-mail: avinashdes999@yahoo.co.uk
ABSTRACT
Vocational and social issues affect rehabilitation and development of patients with cleft lip and cleft palate.
However, psychological problems like lowered self esteem and difficulties in social interaction have also
been noted in them. Not many pediatric reconstructive surgery teams have a psychiatrist on their panel. It
is likely that psychological problems are higher in incidence than literature actually suggests. Hence it is
very essential that such cases are identified by the surgical team to maximize positive outcome of surgery
and rehabilitation. This study discusses psychological issues revolving around cleft lip and cleft palate
along with lacunae in many psychological research studies.
KEY WORDS: Cleft lip, cleft palate, psychological issues
DOI: *****
InTRodUcTIon contribute in the development of psychosocial issues
among individuals with any form of facial anomaly in
Research shows, learning to live with a change in general. Research has shown that attractive children
appearance of one’s face as a result of injury or disease are seen by others as brighter, having more positive
is a difficult task.[1] It is additionally challenging for social behavior and receive more positive treatment
children with congenital craniofacial conditions and than their less attractive counterparts.[4,5] Self perception
their parents to adjust. Evidence shows, amongst the plays a pivotal role in influencing an individual’s
various craniofacial developmental abnormalities, cleft self esteem and psychological adjustment affected by
lip and cleft palate occur most commonl, affecting one cleft lip and palate anomaly.[6-8] Additionally, parental
in 700 live births.[2] The role of physical appearance has influence also shapes ones psychosocial perception.
proved that a healthy physical appearance, regardless The attitudes, expectations and degree of support
of facial or physical characteristics, is considered shown by parents can influence a child’s perception
attractive.[3] Significant literature has shown, in addition of their cleft impairment.[9,10] Parents of children with
to coping with their physical appearance, children clefts may be more tolerant of misbehavior in their
with cleft anomaly in general have to deal with their child and are more likely to spoil their child by being
more superficial psychological issues/ psychosocial overprotective.[4,11] Additionally, peer interaction also
limitations. Existing multispecialty care is primarily plays an important role in maintaining psychosocial
aimed at physical rehabilitation with the psychological limitations. Many children with cleft lip and palate
issues of care often being neglected.[4] This paper may have a less attractive facial appearance or speech
discusses the various psychosocial issues amongst than their peers. A high incidence of teasing over facial
children and adults with cleft lip and cleft palate. appearance is reported among those with cleft lip and
palate.[12-17] A self report research study on determining
oVeRVIew of psychosocIal IssUes In clefT lIp the psychosocial functioning related to cleft lip and
and clefT palaTe palate, showed participants with cleft lip and palate
reported greater behavioral problems; were teased often
Various physiological and sociocultural factors and less happy with their facial appearances.[12]
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 54

Sousa, et al.: Psycological issues in cleft lip and palate
The study concludes that having been teased was a are underachievers along with the evidence of
significant predictor of poor psychological functioning behavioral inhibition, concern regarding appearance,
amongst individuals with cleft lip and palate. However, and decreased expectations by teachers and parents.
it found limited evidence to suggest that individuals
may encounter psychosocial problems as a result of psychologIcal facToRs In clefT sURgeRy
having a cleft lip and palate, with overall adjustment
and functioning appearing to be reasonably good. It is evident that with various limitations that
individuals with cleft lip and palate experience, they are
socIal sTIgma and clefT dIsoRdeRs bound to encounter various psychological difficulties.
Moreover, these limitations build up over a period of
A social stigma is created within an individual when time because of the psychological problems faced.
he/she is negatively discriminated by labeling him/her For example, communication disorders in individuals
different from normal. An individual’s thoughts, feelings with cleft lip and palate seem not to result from
and behavior related to their physical appearance makes phonological defects but from psychological problems
their body image attitudes.[18] A negative response from that may influence the entire development of an affected
outsiders, actual or perceived, may adversely affect child.[24] Anxiety and depression have also been reported
self-image.[4,19,20] Also physical attractiveness plays an to be twice as prevalent in adults with cleft lip and
important role in the development and maintenance palate compared with normal controls.[25] Difficulties
of self beliefs. Research indicates that preference are also experienced in relation to behavioral problems
for attractive individuals subsequently influences and satisfaction with facial appearances.[12] Moreover,
self esteem, social competence, and future ratings of these psychological problems can be interrelated.
attractiveness.[3] Moreover, being physically attractive Anxiety, depression, and palpitations were reported
appears to be an advantageous trait regardless of age. about twice as often by subjects with cleft lip and
Physical attractiveness has shown to play a significant palate compared with controls, and these psychological
role in social set ups like developing relationships problems were strongly associated with concerns about
during various stages of life, school, courtships, work appearance, dentition, speech, and desire for further
etc. Social acceptance often depends on one’s physical treatment.[25] Additionally, findings in studies indicated
look. These associations between physical beauty and that psychological and behavioral problems depended
social acceptability indicate the difficulties for cleft lip on the type of cleft deformity. For example, children
and palate affected individuals.[4,9] with only cleft palate only showed greater problems
with parents, reported depression, anxiety, and
edUcaTIon and commUnIcaTIon pRoBlems learning related to speech than children with unilateral
cleft lip and palate or bilateral cleft lip and palate.
Evidence shows that communication problems related The latter two groups showed fewer problems and a
to cleft lip and palate are noticeable at a young age. A greater relationship of problem to facial appearance.[26]
research study on the development of children with These psychological difficulties are not just limited to
cleft lip and palate infants and toddlers, from birth till individuals/children with cleft lip abnormality, but also
the age of three, reveals that toddlers with cleft palate to their parents. Research studies have shown parents to
exhibit ‘at-risk/delayed’ development in the expressive experience mental crisis, based on their own previous
language domain at 36 months.[21] It is also observed that background, coping with present stress etc. in bringing
factors directly affecting the psychological development up a child with cleft lip.[4,27]
of a child born with cleft lip and palate include possible
speech and language disorders, facial disfigurement, saTIsfacTIon and IssUes afTeR sURgeRy
and hearing loss.[22] Research thus makes the association
of communication problems with cleft lip and palate Surgery, being the immediate option of dealing with
evident. To add on to the communication disadvantages certain issues related to disfigurement, is beneficial in
experienced by individuals with cleft lip and palate, it dealing with both physical and psychological issues.
becomes more difficult to deal with emotional issues Surgery usually results in increased self esteem, self
during their academic years. Studies on cleft have confidence and satisfaction with appearance. [28] It
shown relationships between (a) facial appearance and can be used in young patients to improve esthetic
teacher perception, (b) behavioral inhibition and lower appearance, an important factor in the psychological
school achievement, and (c) speech defectiveness and development of adolescents.[29] However, it is necessary
self-esteem.[23] for oneself to develop positive self skills to deal with
the post surgery situations. Any individual with
Research shows that a high percentage of cleft children facial differences, who has fostered these skills, can
55 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Sousa, et al.: Psycological issues in cleft lip and palate
achieve acceptance, develop positive social interaction background play a central role in influencing behavior.
skills, demonstrate social competence, and be less It appears that research studies of the effects of cleft
likely to exhibit significant adjustment problems.[30] related conditions on behavior should examine both
Unrealistic, high expectations post surgery may also internalizing and externalizing dimensions of behavior
lead to dissatisfaction, which may further affect an and consider that there may be subgroups of children
individuals self satisfaction.[4] This disappointment showing these different kinds of behavior across
and dissatisfaction can also be experienced by parents. different age levels.[22]
Evidence makes the need for disseminating valuable
information on the pros and cons of surgery essential. conclUsIon
A study on patient satisfaction observed that majority of
the patients expressed satisfaction on the care provided, Identifying the common psychosocial factors related to
30% of the parents expressed a need to make them cleft lip and palate remains a major challenge. Extensive
more involved treatment planning decision with most research data does suggest that psychological and
of them having no or inadequate knowledge on left clip psychosocial factors have an effect on behavior, but
treatment procedures.[31] there is limited evidence to suggest that individuals
experience psychosocial problems as a result of cleft
pRoBlems wITh clef T lIp and pal aTe lip and palate. More research is required to develop a
psychologIcal ReseaRch tool whereby bias in self reporting could be avoided.
Additionally, there is a need to evaluate patient and
Though several research studies have been carried family before surgery and help provide them with
out on various aspects of cleft lip and palate they are relevant information on post and pre surgery issues.
insufficient in providing information. Studies do predict
some amount of difficulties in psychosocial functioning RefeRences
among cleft lip palate individuals, however there is
1. Rankin M, Borah G. Perceived functional impact of abnormal
limited information on the severity and the duration facial appearance. Plast Reconstr Surg 2003;111:2140-6.
of the same. [12] There is no direct evidence of cleft lip 2. Turner SR, Rumsey N, Sandy JR. Psychological aspects of cleft
palate on behavior. Many studies have shown other lip and palate. Eur J Orthodont 1998;20:407-15.
environmental, confounding factors such as teasing, 3. Sawer DB, Magee L. Psychological aspects of reconstructive
and cosmetic plastic surgery: Clinical, empirical and ethical
leading to poor psychological functioning, more so than perspectives. Lippincott Williams and Wilkins; Maryland: 2006.
having a cleft lip and/or palate per se,[22] thus providing p. 3-11.
conflicting evidence when it comes to establishing 4. Turner SR, Thomas PW, Dowell T, Rumsey N, Sandy JR.
whether children and adults with cleft lip and palate Psychological outcomes amongst cleft patients and their families.
Br J Plast Surg 1997;50:1–9.
experience psychological problems as a result of their 5. Dion KK, Berscheid E, Walster E. What is beautiful is good. J Pers
cleft. Soc Psychol 1972;24:285-90.
6. Videbeck R. Self-concept and the reaction of others. Sociometry
Many studies investigating psychological issues of cleft 1960;23:231-9.
7. Fitts WH. The self concept and behavior: Counsellor recordings
lip and palate use self reported data,[4,12] thus indicating and tests. Research Monograph No.1, Nashville, Tenessee; 1972.
a possible error of predisposition to self perception p. 35-45.
Similar self reports by parents and individual with cleft 8. Strauss RP, Broder H, Helms RW. Perception of appearance and
lip and palate have been reviewed to identify the level speech by adolescent patients with cleft lip and palate and their
parents. Cleft Palate Journal 1988;25:335-41.
of satisfaction post surgery.[32] As observed, unrealistic 9. Bull R, Rumsey N. The social psychology of facial disfigurement.
expectations can also play a pivotal role in developing J Cross Cult Psychol 1988;17:99-108.
psychological distress. Research on determining the 10. Lansdown R, Lloyd J, Hunter J. Facial deformity in childhood:
needs of parents as well as patients on cleft lip and severity and psychological adjustment. Child Care Health Dev
1991;17:165-71.
palate would be useful in providing basic information 11. Harper DC, Richman LC. Personality profiles of physically
of cleft lip palate and its characteristics pre and post impaired adolescents. J Clin Psychol 1978;34:636-42.
surgery. 12. Hunt O, Burden D, Hepper P, Johnston C. The psychosocial
effects of cleft lip and palate: a systematic review. Eur J Orthodont
2005;27:274–85.
Additionally, facial growth would change along with
13. Bernstein NR, Kapp K. Adolescents with cleft palate: body-image
age and treatment interventions; longitudinal studies and psychosocial problems. Psychosomatics 1981;22:97–703.
which determine the facial growth changes and the 14. Heller A, Tidmarsh W, Pless IB. The psychosocial functioning
experience of surgery should be examined as possible of young adults born with cleft lip or palate. Clin Pediatr
1981;20:459–65.
factors influencing psychosocial functioning.[4] An
15. Noar JH. Questionnaire survey of attitudes and concerns of
individual’s personality traits i.e. level of confidence patients with cleft lip and palate and their parents. Cleft Palate
and environmental factors like upbringing, family Craniofac J 1991;28:279–84.
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16. Noar JH. A questionnaire survey of attitudes and concerns of Norwegian adults who had undergone standardised treatment
three professional groups involved in the cleft palate team. Cleft of complete cleft lip and palate. Part II. Self-reported problems
Palate Craniofac J 1992;29:92–5. and concerns with appearance. Scand J Plast Reconstr Surg Hand
17. Turner SR, Rumsey N, Sandy JR. Psychological aspects of cleft Surg 1995;29:329–36.
lip and palate. Eur J Orthodont 1998;20:407-15. 26. Pillemer FG, Cook KV. The psychosocial adjustment of pediatric
18. Clifford E. The cleft palate experience: new perspectives on craniofacial patients after surgery. Cleft Palate J 1989;26:201-7.
management. Springfield, Illinois: 1978. p. 22-30. 27. McWilliams BJ. Social and psychological problems associated
19. Goffman E. Stigma: Notes on the management of spoiled identity. with cleft palate. Clin Plast Surg 1982;9:317-26.
Prentice-Hall, Englewood Cliffs, New Jersey: 1968. p. 15-7. 28. Lefebvre AM, Munro I. The role of psychiatry in a craniofacial
20. Charon JM. Symbolic interactionism: an introduction, an team. Plast Reconstr Surg 1978;61:564-9.
interpretation, an integration. Prentice Hall, Englewood Cliffs, 29. Rachmiel A, Aizenbud D, Ardekian L, Peled M, Laufer D.
New Jersey: 1979. p. 11-4. Surgically-assisted orthopedic protraction of the maxilla in cleft
21. Neiman GS, Savage HE. Development of infants and toddlers lip and palate patients. Int J Oral Maxillofac Surg 1999;28:9-14.
with clefts from birth to three years. Cleft Palate Craniofac J 30. Kapp-Simon KA. Psychological interventions for adolescents with
1997;34:218–25. cleft lip and cleft palate. Cleft Palate Craniofac J 1995;32:104-8.
22. Richman LC, Eliason M. Psychological characteristics of children 31. Jeffery S, Boorman JG. Patient satisfaction with cleft lip and palate
with cleft lip and palate: intellectual, achievement, behavioral and services in a regional centre. Br J Plast Surg 2001;54:189-96. Young
personality variables. Cleft Palate J 1982;19:249–57. JL, O’Riordan M, Goldstein JA, Robin NH. What Information Do
23. Richman LC, Millard T. Brief report: cleft lip and palate: Parent of Newborns With Cleft Lip, Palate, or Both Want to Know?
longitudinal behavior and relationships of cleft conditions to Cleft Palate Craniofac J 2000;38: 55–8.
behavior and achievement. J Pediatr Psychol 1997;22:487–94.
24. Rosanowski F, Ulrich E. Phoniatric aspects in cleft lip patients.
Facial Plast Surg 2002;18:197-203. Source of Support: Nil, Conflict of Interest: None declared.
25. Ramstad T, Otten E, Shaw WC. Psychosocial adjustment in
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5. Editor’s Name : K. L. N. Rao
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Date: 10th August 2009 K. L. N. Rao
57 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Original Article Full text online at http://www.jiaps.com
Hirschsprung’s disease diagnosis: Comparison of
immunohistochemical, hematoxilin and eosin staining
Mehrdad Memarzadeh, Ardeshir Talebi, Masod Edalaty, Mehrdad Hosseinpour1,
Nasrin Vahidi2
Departments of Surgery, 1Pathology, Medical School, Isfahan University of Medical Sciences, 1Department of
Surgery, Medical School, Kashan University of Medical Sciences, 2Al-Zahra University Hospital
Address for correspondence: Dr. Mehrdad Hosseinpour, Trauma Research Center, Shahid Beheshti Hospital, Kashan University of
Medical Sciences, Kashan-Iran. E-mail: meh_hosseinpour@yahoo.com
ABSTRACT
Background: The diagnosis of Hirschsprung’s disease (HD) based on the absence of ganglion cells in
hemotoxilin and eosin (H and E) staining has limitations in the diagnosis of immature ganglion cells in
neonates. Methods: In this prospective study, 54 biopsies taken from suspectedHD patients (five mucosal
specimens and 49 full thickness specimens) were studied. In the laboratory, after preparing sections of
paraffin embedded tissues, H and E staining slides were compared with immunohistochemical (IHC) staining
including: S100, NSE, CD117, CD56, Cathepsin D, Vimentin, bcl2, GFAP, Synaptophysin and chromogranin.
Results: The study revealed 30 negative (absence of ganglion cells) cases (55.5%), 17 positive cases (31.04%)
and seven suspected cases (12.9%) ganglion cells on the H and E staining. On IHC staining with CD56 and
Cathepsin D, all of the 17 positive cases detected through H and E, were confirmed for having ganglion cells
and out of 30 cases reported negative on H and E staining, 28(93.3%) were reported negative and two (6.7%)
positive by IHC staining. Of the seven suspected cases H and E staining), IHC staining detectedganglion
cells only in five slides; two remained negative. Conclusions: IHC staining using CD56 and Cathepsin D
improved the accuracy of diagnosis in HD when used in addition to H and E staining technique, especially
for negative or suspicious slides. Among the IHC staining markers, S100, NSE, CD117, Vimentin, bcl2, GFAP,
Synaptophysin and chromogranin were suboptimal in comparison to CD56 and Cathepsin D.
KEY WORDS: Hirschprung Disease, immunohistochemical staining, megacolon
DOI: *****
InTRodUcTIon O n t h e o t h e r h a n d a l t h o u g h AC h E a c t i v i t y
is diagnostically the most useful set of enzyme–
H e m a t ox i l i n a n d E o s i n ( H a n d E ) s t a i n i n g , histochemical reactions, it is not sufficient. AChE stains
Acetylcholinesterase staining (AChE) are commonly the parasympathetic nerve fibers and trunks of fibers
used [1-5] in the diagnosis of Hirschprung’s disease (HD). that increase dramatically in the lamina propria mucosa
However, diagnosis is not possible with H and E every and sub mucous layer, but is not a specific marker for
times, because staining has limitations in the diagnosis ganglion cell.[8] AChE staining requires the experience
of pathologists and in some instances interpretation is
of immature ganglion cells in neonates and the sub
difficult. [9] There are reports of false positive and false
mucosal area in which the ganglion cells are small (three
negative results using this technique.[10]
to five cells per ganglion) and irregularly distributed
and so their identification is difficult and requires high Earlier, the importance of IHC studies has been
expertise.[2,6, 7] Some sections contain only a part of the emphasized in the diagnosis of immature ganglion cells,
cytoplasmic portion of the ganglion cell.The diagnosis hypoganglionosis and other suspicious situations. [11-13]
of those cells as ganglion cells is difficult, because its In this study, we compareIHC staining using neural
staining is very similar to the background of the slide. markers with H and E staining to find out the best
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 58

Memarzadeh, et al.: Application of immunohistochemical staining
diagnostic panel for HD. ResUlTs
maTeRIal and meThods In the microscopic study of 54 specimens, H and E
staining revealed absence of ganglion cells (negative)
This is a prospective study conducted in the period in 30 cases (55.5%), presence of cells (positive) in
2001 to 2004. Rectal biopsy specimens from 54 infants 17(31.04%) and suspected presence in seven cases
suspected to be having HD constituted the material (12.9%). In the study of specimens through IHC staining
for the study. There were five mucosal and 49 full with CD56 and Cathepsin D [Figure 1 and 2], all 17 cases
thickness biopsies. The specimens were kept in 10% detected positive through H and E, were confirmed
formalin solution. for having ganglion cells and of the 30 cases reported
negative through H and E staining, 28 (93.3%) were
In the laboratory, after preparing sections of paraffin reportednegative and two (6.7%) positive by IHC. Out
embedded tissues, H and E staining slides were of seven cases suspected of having ganglion cells on
compared with IHC staining including S 100, NSE, H and E staining, we could find ganglion cells in five
CD 117, CD 56, Cathepsin D, Vimentin, BCL2, GFAP, slides while two remained negative [Table 1].
Synaptophysin, chromogranin.
According to our selection criteria, other markers (S100,
The slides for IHC were processed as follows: NSE, CD 117, Vimentin, bcl2, GFAP, Synaptophysin,
• First sections of four µm were obtained and fixed on and chromogranin ) [14] used for IHC staining were
the slides with polyelizine. This was followed by suboptimal in comparison to CD56 and Cathepsin D
antigen retrieval for 10 minutes using heat and citrate [Figure 3].
buffer (pH is equal to six). Then H2O2% 3 and pure
methanol were added for five minutes and sections dIscUssIon
were washed with distilled water. Next, primary
antibody (with negative control) was added for 10 Detection of ganglion cells in H and E sections can be a
minutes and washing was performedSecondary difficult process for the pathologist.[15] The maturation
antibody (biotinylated link) was added for 10 of ganglion cells is incomplete at the time of birth,
minutes and washed Streptavidin – HRP was added especially in the sub mucosal area. [12] Immature
for 10 minutes and washed with P.B.S. After adding ganglion cells may be unipolar or bipolar and can be
substrate chromogen (D.A.B) for 10 minutes, counter mistaken for stromal cells.[12] Sub mucosal ganglion cells
was stained. All the antibodies were from DAKO.- Co
and the slides were scanned for ganglion cells.
The best staining method was appreciated based on the
degree of staining of ganglion cell versus its background
and clear detection of ganglion cells.
Figure 2: IHC staining of Cathepsin D. In CD56 staining the background
is stainable; but ganglion cells are not. (Magnification =10×40)
Table 1: Comparison of Detection of Ganglion Cells in H& E
and IHC staining
H and E H and E H and E
Positive (17) Negative (30) Suspicious (7)
Figure 1: IHC staining of CD56. In Catepsin D staining, the ganglion
cells are stainable; but the background is not Magnification =10×40) IHC Positive 17 2 5
IHC Negative 0 28 2
59 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2
Author Queries?????
Please Provide High quality Image of Figures 1-3

Memarzadeh, et al.: Application of immunohistochemical staining
Figure 3A: IHC staining of (A) Synaptophysin and (B) BCL2. (In Figure 3B: IHC staining of (A) Synaptophysin and (B) BCL2. (In
Synaptophysin staining, the ganglion cells and background are not Synaptophysin staining, the ganglion cells and background are not
stainable well, but in BCL2 staining, the ganglion cells and background stainable well, but in BCL2 staining, the ganglion cells and background
are both stainable. (Magnification 10 × 40) are both stainable. (Magnifications =10 × 40)
are smaller than myenteric plexus ganglion cells,[7] and compare IHC markers with H and E staining to find
pathologists have to prepare between 50 to 400 sections out the best diagnostic panel for detection of ganglion
of H and E stained slides to find ganglion cells.[16] On cells. As shown in figures 1 to 3, ganglion cell detection
the other hand, although AChE staining is the chosen and its staining with CD56 and Cathepsin D were better
technique for some pathologists[12] its diagnosis needs than Bcl2 and Synaptophysin.
experience and its interpretation is difficult in some
instances.[5,9] One of the problems is the interference To conclude, our study shows that IHC markers,
of red blood cell (RBC) acetyl cholinesterase due to including both Cathepsin D and CD56, especially for
hemorrhage in lamina propria.[15] Also, false positive[16] negative or suspicious slides are the best diagnostic
and false negative[15] reactions were reported using this panel for detection of ganglion cells. In Cathepsin
staining technique. D staining, the ganglion cells are stainable, but the
background is not. On the contrary, in CD56 staining
Previous studies have emphasized the importance the background is stainable, but ganglion cells are
of immunohistochemistry in the diagnosis of not, these two methods complement each other. The
HD. [5,12,13,16-18] The obstacles to widespread use of pediatric surgeon was therefore advised to disregard
enzyme-histochemical reactions for HD diagnosis the surgical operation for negative cases. This panel
are linked to technical difficulties in some pathology can detect smaller or immature ganglion cells and
laboratories such as fresh preparing of medium, toxicity also small cytoplasmic portions of those cells. Hence,
of reagents and storage difficulties. Park et al.[15] found the sensitivity and specificity are increased and false
that the main diagnostic pitfall was the interpretation negative and positive results are decreased.
of the enteric nervous plexuses in the transitional zone
and the detection of the indistinct or immature neurons RefeRences
indistinguishable from enteric glial cells or satellite
cells. They showed immunohistochemical study was 1. Gupta M, Beeram MR, Pohl JF, Custer MD. Ileal atresia associated
a very helpful diagnostic adjunct to delineating the with Hirschsprung disease (total colonic aganglionosis). J
Pediatric Surg 2005;40:e5-7.
immature neurons (BCL2), and the size of the enteric 2. Martucciello G, Brizzolara A, Favre A, Lombardi L, Bocciardi
ganglia and neuromuscular innervation (S-100 protein, R, Sanguineti M, et al. Neural crest neuroblasts can colonise
Synaptophysin, and CD56). Another study [19] found aganglionic and ganglionic gut in vivo. Eur J Pediatr Surg
that Synaptophysin-positive synapses distribution 2007;17:34-40.
3. Meijers JH, van der Sanden MP, Tibboel D, van der Kamp AW,
in circular and longitudinal colonic muscles and Luider TM, Molenaar JC. Colonization characteristics of enteric
intermuscular ganglions can reflect functional neural crest cells: Embryological aspects of Hirschprung’s disease.
disturbances of large bowel motility and could be J Pediatr Surg 1992;27:811-4.
helpful in the description of the innervation status of 4. Sukhotnik I, Bernshteyn A, Mogilner JG. The basic biology of
apoptosis and its implications for pediatric surgery. Eur J Pediatr
colonic specimens in HD patients. Surg 2005;15:229-35.
5. Sams VR, Bobrow LG, Happerfield L, Keeling J. Evaluation of
Facing a wide diversity of opinions, we decided to PGP 9.5 in the diagnosis of Hirschsprung’s Disease. J Pathol
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 60

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1992;168:55-8. 14. Martucciello G, Pini Prato A, Puri P, Holschneider AM, Meier-Ruge
6. Petras R. Hirshsprung’s disease. In: Sternberg, SS. Diagnostic W, Jasonni V, et al. Controversies concerning diagnostic guidelines
surgical pathology, Williams and Wilkins; Philadelphia: 2004. p. for anomalies of the enteric nervous system: A report from the
1390-1. fourth International Symposium on Hirschsprung’s disease and
7. Rosai J. Large Bowel Disease. In: Ackerman’s Surgical Pathology. related neurocristopathies. J Pediatric Surg 2005;40:1527-31.
Philadelphia: Mosby; 2004. p. 777-9. 15. Park SH, Min H, Chi JG, Park KW, Yang HR, Seo JK.
8. Martucciello G. Hirschsprung’s disease, one of the most difficult Immunohistochemical studies of pediatric intestinal pseudo-
diagnosis in pediatric surgery: a review of the problems from obstruction. Am J Surg Pathol 2005;29:1017-24.
clinical practice to the bench. Eur J Pediatr Surg 2008;18:140-9. 16. Ariel I, Vinograd I, Lernau OZ, Nissan S, Rosenmann E. Rectal
9. Chen F, Winston JH 3rd, Jain SK, Frankel WL. Hirschsprung’s mucosal biopsy in aganglionosis and allied conditions. Hum
Disease in a young adult: report of a case and review of the Pathol 1983;14:991-5.
literature. Ann Diagn Pathol 2006;10:347-51. 17. Barshack I, Fridman E, Goldberg I, Chowers Y, Kopolovic J. The
10. Petras R. Hirschsprung’s disease. In: Sternberg, SS. Diagnostic loss of Calretinin expression indicates disease. J Clin Pathol
surgical pathology, Williams and Wilkins; Philadelphia: 2004. p. 2004;57:712-6.
1390-1. 18. Petchasuwan C, Pintong J. Immunohistochemical for intestinal
11. Davis BJ, Ornstein L. High resolution enzyme localisation with ganglion cells and nerve fibers: aid in the diagnosis of
a new diazo reagent, “hexazonium pararosaniline”. J Hist Cyt Hirschsprung’s Disease. J Med Assoc Thai 2000;83:1402-9.
1959;7:297. 19. Dzienis-Koronkiewicz E, Debek W, Chyczewski L. Use of
12. Hall CL, Lampert PW. Immunohistochemistry as an aid in synaptophysin immuno-histochemistry in intestinal motility
the diagnosis of Hirschsprung’s disease. Am J Clin Pathol disorders. Eur J Pediatr Surg 2005;15:392-8.
1985;83:177-81.
13. Mackenzie JM, Dixon MF. An Immunohistochemical study of the
enteric neural plexus in Hirschsprungs’s Disease. Histopathology Source of Support: Nil, Conflict of Interest: None declared.
1987;11:1055-66.
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61 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Original Article Full text online at http://www.jiaps.com
Flexible ureterorenoscopy and laser lithotripsy in children
Wen-Chan Yeow, Richard Pemberton, Andrew Barker
Department of Pediatric Surgery, Princess Margaret Hospital, Roberts Road, Subiaco WA 6008
Address for correspondence: Dr. Andrew Barker, Princess Margaret Hospital for Children, Roberts Road, Subiaco, WA 6008,
Australia. E-mail: Andrew.Barker@health.wa.gov.au
ABSTRACT
Background: Flexible ureterorenoscopy (FUR) and laser lithotripsy (LL) are techniques used in the
management of upper urinary tract disorders. These techniques, so far established in adults, are now being
used in children as well. We report our experience with 26 cases of pediatric upper urinary tract disorders
treated using these techniques. Methods: In the period from 1997 to 2006, FUR was performed in 26 children
(14 males and 12 females) in the age group of three months to 15 years with a mean age of 8.2 years. Twenty five
were stented prior to undergoing FUR and 24 presented with suspected upper tract stones (17 pelvicalyceal
and seven midureteric). Two cases showed JJ stent migration post-pyeloplasty. Results: Eight cases involved
diagnostic procedures. Six excluded the presence of renal calculi, one had focal medullary sponge kidney,
and one had calcified papillae. There were 15 cases of therapeutic FUR. Of these, 12 had LL with only one
had incomplete stone fragmentation which subsequently passed spontaneously. Other therapeutic procedures
included removal of migrated JJ stents and FUR with the basket removal of a midureteric calculus. Three
cases failed ureterorenoscopy due to technical difficulties. The overall success rate was 88.5% for FUR.
Conclusion: FUR and LL are valuable minimally invasive techniques for the examination and treatment of
pediatric upper urinary tract conditions. Preoperative stenting improves passage of the ureteroscope and
with progressive miniaturization of instruments, the lower weight limit will decrease.
KEY WORDS: Laser lithotripsy, pediatrics, ureteroscopy, urinary calculi
DOI: *****
InTRodUcTIon FUR and laser lithotripsy (LL) have been demonstrated
as safe procedures and to achieve a greater stone-
Flexible ureterorenoscopy (FUR) is a common procedure free rate when compared to ESWL, especially with
performed on adults for many purposes. Paediatric FUR stones greater than one centimeter.[2,3] The minimal
has become more widely practiced primarily due to the invasiveness of FUR, as compared to open surgery, also
availability of smaller caliber ureteroscopes. Its use in allows complete assessment of the urinary tract in cases
the pediatric population has been most beneficial in with suspected upper tract stones.
urolithiasis, though other upper urinary tract conditions
can also be treated. In 2000, van Savage et al. reviewed the management
of distal ureteric calculi in their pediatric population.
Many pediatric cases of urinary calculi occur in the They found that calculi 4mm or greater in size are
Aboriginal population in Australia.[1] Cheah et al. in unlikely to pass and thus will most likely require
their study of 93 Western Australian children found surgical intervention.[4] Our results with pediatric FUR
91.4% of calculi were located in the upper urinary tract and LL are described.
and only 8.6% were in the bladder.[1] Open nephro-
pyelolithotomy or ureterolithotomy, percutaneous meThods
nephrolithotomy or extracorporeal shock wave
lithotripsy (ESWL) would have been the only options The records of all children who had FUR in Western
available to treat these upper tract calculi. Australia from January 1997 to January 2006 were
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 62

Yeow, et al.: Running title missing?????
reviewed. The search found 26 children with 12 females bladder was opened for bladder diverticulum repair and
and 14 males. Their ages ranged from three months to ureteric reimplantation. He had suspected urolithiasis on
15 years (mean 8.2 years). There were seven Aboriginal ultrasound. Inspection revealed calcified papillae [Figure 1].
children.
Only eight children underwent FUR [Table 1]. In six, no
Ultrasound showed 24 children with urolithiasis, all calculi were seen in the upper renal tract at time of FUR.
upper tract calculi (17 pelvicalyceal and seven mid- However, three children had matrix stone seen to wash
ureteric). Four children also had intravenous pyelograms out after removal of the JJ stent. Calcified papillae and
(IVP) in their preoperative assessments. They presented focal medullary sponge kidney were diagnosed in two
with symptoms of pain, hematuria, or had recurrent children. Fifteen children had therapeutic procedures,
urinary tract infections. Some were being monitored 13 were for stone disease. One child had a mid ureteric
with serial ultrasounds, which demonstrated persisting calculus, which on FUR was felt to be small enough
and enlarging stone size. Two children underwent FUR to remove with a basket. Twelve underwent LL, with
to remove JJ stents from the ureter. all having successful fragmentation except one, who
on ultrasound during follow up still had three to four
Twenty three of the 24 children with upper tract stone mm fragments. These were treated conservatively and
disease had retrograde pyelogram and JJ stenting passed spontaneously.
performed prior to FUR. The JJ stent enabled ureteric
dilatation for at least two weeks before any attempt Two children had pyeloplasties performed. One child,
was made to pass the ureteroscope. FUR and Holmium: during removal of the JJ stent had the stent snap
YAG (Lumenis VersaPulse Powersuite 20W) LL was within the ureter. The ureteroscope was passed and the
performed in a manner similar to adults. A 7.5Fr Storz proximal part of the stent was located at the level of the
adult flexible cystoscope or a 7.5Fr Olympus flexible
ureteroscope was used. A 9.5/11.5Fr or 10/12Fr Cook
ureteral access sheath was used in all children who had
LL. The ureteroscope was advanced up to the kidney
under fluoroscopy over a guide wire and, during LL, a
second guide wire was left alongside the ureteroscope
for safety. With the ureteroscope in the renal pelvis, each
calyx was individually assessed for calculi. Once the
calculus was located a 200micron laser fiber was used
under direct vision to vaporize the stone to fragments
one mm or smaller in size.
Post LL, the children stayed overnight with a 3F
ureteric catheter draining the upper tract strapped to a
Foley indwelling urinary catheter, which was removed
the next day. The cases that were assessed to have
edematous ureters post procedure had a JJ stent left in Figure 1: Three-Month-Old Child with Preoperative Renal Calculi on
situ which was removed two weeks postoperatively. Ultrasound. Ureterorenoscopy Revealed Calcified Papillae
Table 1: Summary of fexible ureterorenoscopy outcomes
Patients were followed up in the outpatient clinic with
N (subtotal)
urinary tract ultrasounds. The average follow-up period
Therapeutic (15)
was 20 months. The Aboriginal children from the Laser lithotripsy:
northern regions of Western Australia were followed up Successful fragmentation 11
by their community general practitioners and visiting Incomplete fragmentation 1
pediatricians with regular urinary tract ultrasounds. Retrieval JJ stent 2
Ureteroscopic basket removal mid ureteric calculus 1
ResUlTs “Diagnostic” (8)
Negative for calculi 6
Twenty six patients were treated for upper urinary tract Focal medullary sponge kidney 1
disorders; 24 for presumed urolithiasis and the average Calcified papillae 1
stone size was 10.3mm (range three to 21mm). Twenty Failed (successfully converted to open) (3)
five children had JJ stenting before FUR. The child Ureter not accessible 1
not prestented was a three-month-old male who had Narrow calyx infundibulum 1
“open” ureterorenoscopy into his megaureter whilst the Poor vision 1
63 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Yeow, et al.: Running title missing?????
pelvic brim and snared with a basket to successfully without causing ureteric trauma especially at the orifice.
remove the fragment without complications. The
second child who had pyeloplasty had proximal stent In the earlier patients, when experience with FUR
migration and so there was no distal end to grasp at in children was less, all patients had post-operative
cystoscopy. The ureteroscope was passed and the JJ JJ stenting as well. More recently, if there was no
stent was found midureter and removed with a basket significant ureteric edema (assessed at time of
without complications. operation) and minimal ureteroscopic passages, a
ureteric catheter was inserted up to the renal pelvis and
There were three failed FUR cases. In a 2.5 year old child strapped to a bladder catheter overnight. This enabled
with a 12 by 21mm right renal calculus the ureter was too quick, simple removal as a single unit the next morning
edematous for guide wire insertion after her prestented prior to discharge.
JJ stent was removed at the time when ureteroscopy was
attempted. In a 10-year-old male, the ureteroscope was No complications were encountered other than the
unable to be manipulated successfully due to a narrowed failed FUR due to technical or anatomical difficulties
infundibulum of the calyx. The third case was an 11-year- precluding safe completion of the operation. The
old male who originally had calcium oxalate staghorn major complications reported in the literature include
and a lower pole calculus. His LL was abandoned due to stricture formation, ureteric perforation and ureter
reduced vision from the large stone burden being treated avulsion. We have no known long term complications
with lasertripsy. All three children were successfully for the current follow-up period.
converted to open pyelonephrolithotomy. The overall
success rate was 88.5% (23/26). conclUsIon
dIscUssIon FUR and LL are valuable minimally invasive techniques
for management of pediatric upper urinary tract
In our institution, computed tomography (CT) was disorders. Usually only one treatment is required. Calculi
not routinely performed for assessing urolithiasis in can be followed into the renal pelvis if they migrate up
the time period of this paper because of its radiation- the ureter during treatment and hard cysteine stones are
associated cancer risk. Instead, patients first undergo also treatable. FUR is also useful in other conditions such
an ultrasound and occasionally an IVP, if required, to as those requiring renal pelvis examination, biopsy and
confirm the diagnosis. Currently CT is performed more foreign body retrieval. Preoperative stenting improves
often using lower dose targeted non contrast scanning passage of the ureteroscope and with progressive
with helical CT for urolithiasis. miniaturization of the instruments; it will be able to be
performed in smaller children.
All the eight patients who underwent “diagnostic” FUR
were diagnosed as upper tract calculi preoperatively acKnowledgmenT
with calculi ranging from three to five mm. The FURs
were performed to treat the calculi with LL. The surgeon Thanks to Shane La Bianca, Julian Mander, John Stanley and
saw stone fragments when he removed the JJ stent in Rob Thomas for their technical expertise during the initial
three children just prior to FUR. In the six children learning curve.
(where no calculi were seen) we believe the calculi
were dislodged during their procedures. RefeRences
In our series, 25 patients had stenting performed prior 1. Cheah WK, King PA, Tan HL. A review of pediatric cases of urinary
tract calculi. J Pediatr Surg 1994;29:701-5.
to any attempts to pass the ureteroscope. We found this 2. Lam JS, Greene TD, Gupta M. Treatment of proximal ureteral
beneficial as the kidney was relieved of obstruction, calculi: holmium:YAG laser ureterolithotripsy versus
converting an emergency situation to an elective extracorporeal shock wave lithotripsy. J Urol 2002;167:1972-6.
3. Wollin TA, Teichman JM, Rogenes VJ, Razvi HA, Denstedt
operation. It also allowed passive ureteric dilatation.
JD, Grasso M. Holmium: YAG lithotripsy in children. J Urol
However, two general anesthetics are required. 1999;162:1717-20.
4. Van Savage JG, Palanca LG, Andersen RD, Rao GS, Slaughenhoupt
During ureteroscopies, the ureteric orifice can be BL. Treatment of distal ureteral stones in children: similarities to
difficult to intubate and traverse, as it is narrow. In the American urological association guidelines in adults. J Urol
2000;164:1089-93.
Singh et al. the tip (first 2cm, approximately 9Fr) of a 5. Singh A, Shah G, Young J, Sheridan M, Haas G, Upadhyay J.
hydrophillic 14Fr ureteral access sheath is used to dilate Ureteral access sheath fot the management of pediatric renal and
the ureteric orifice.[5] Our access sheaths used were ureteral stones: a single centre experience. J Uro 2006;175:1080-2.
smaller. The benefit with access sheath use is that the
Source of Support: Nil, Conflict of Interest: None declared.
ureteroscope can be passed multiple times, if required,
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 64

Original Article Full text online at http://www.jiaps.com
Is ligation of hernial sac during orchiopexy mandatory?
Nilay Biswas, Nilanjan Mitra, Hiralal Konar, Dipak Ghosh, Sukanta K. Das
Department of Pediatric Surgery, Medical College and Hospital, Kolkata, West Bengal, India
Address for correspondence: Dr. Veena Kumari, LHQ, Medical college & Hospital, 88 College Street, Kolkata-73, WestBengal, India.
E-mail: dr_veenasingh@yahoo.co.in
ABSTRACT
Aim: Traditionally, ligation of hernial sac during orchiopexy is considered mandatory to prevent postoperative
development of hernia. A prospective study was carried out to see if it is actually required based on the
fact that any peritoneal defect closes within 24 hours by metamorphosis of the in situ mesodermal cells.
Methods: Fifty cases of undescended testis, age ranging from eight months to 12 years were enrolled. All
of them underwent standard orchiopexy without ligation of the hernial sac. Results: Follow up of all cases
ranged between 1.5 years to three years. Not a single case was reported with evidence of hernia. Conclusions:
It is unnecessary to ligate the hernial sac during orchiopexy.
KEY WORDS: Ligation of hernial sac, orchiopexy, undescended testis
DOI: *****
InTRodUcTIon the ligation of the hernia sac. No special investigative
workup was done.
The conventional and accepted technique of orchiopexy
recommends that ligation of the hernial sac is mandatory The hernia sac was dealt with after complete mobilization
for prevention of postoperative development of hernia. of the testis through an inguinal incision [Figure 1].
However, it has been seen that during laparoscopic The sac was first opened up, divided and the proximal
orchiopexy there is no difference to simple suturing end of the divided sac was very gently peeled off with
when peritoneum is incised, hernia sac dissected and dissecting forceps as high as possible without damaging
left alone. This may be due to the fact that any peritoneal the cord structures [Figure 2]. It was left as such without
defect closes within 24 hours by metamorphosis of ligation. Standard orchiopexy was then performed by
the in situ mesodermal cells. We report the results of a making subdartos pouch.
study on nonligation of hernia sac during conventional
orchiopexy.
ResUlTs
maTeRIals and meThods All the patients were followed up for 1.5 years to three
years. No inguinal hernia was detected during the
This prospective study was conducted between April
regular follow-up in any child.
2004 and 2007. Fifty children with an age range of eight
months to 12 years with a diagnosis of undescended
testis were included. Of the 50 cases, 38 were unilateral dIscUssIon
and 12 bilateral cases. Of the 38 unilateral undescended
testis, 22 were right-sided and 16 left-sided. In all the Hernia sac has been routinely dissected and meticulously
cases, testis was palpable. Clinically, no cases presented freed from the cord structures and suture ligated
with hernia. After the baseline investigations, informed proximally during inguinal orchiopexy. This is done to
consent of the parents was taken and the procedure achieve adequate length of the cord to bring down the
explained to those parents who were able to understand. testes to the scrotum to its normal position and prevent
All children underwent standard orchiopexy without development hernia postoperatively.
65 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Author Queries?????
Please Provide High quality Image of Figures 1-2
Biswas, et al.: Do we really need to ligate hernial sac during orchiopexy?
Figure 1: Separation and gentle peeling of sac from cord structures Figure 2: Peeling of sac as high as possible
In cases of inguinal hernia in children, Mohta is very thin and tends to tear very easily.
et al. [1] suggested that nonligation of hernia sac during
herniotomy in children has no untoward effect on the Length of testicular vessel: It is found that the most
early complications and recurrence rate. The study important criteria for bringing down the testes in the
was based on the fact that peritoneal defect closes by scrotum is the length of the testicular vessels; in our
metamorphosis of the in situ mesodermal cells. Earlier, procedure extra length of the testicular vessel can be
Shulman et al.[2] showed that ligation of hernia sac in achieved by peeling off the peritoneum as high as
adult herniorrhaphy is a needless step. A prospective possible.
study of laparoscopic inguinal hernia repair in children
by Schier[3] showed that there is no difference to simple No ligation: Accidental ligation of the cord structures
suturing when peritoneum was incised and hernia sac is avoided.
resected. He stressed that an open internal inguinal
ring is not an inguinal hernia.[4] During laparoscopic Our experience suggests that routine ligation of the
orciopexy, Handa et al. [5] showed that closure of hernial sac is not mandatory. This reduces the operative
the internal ring is not necessary. Mobilization of time in all cases and eliminates the possibility of
undescended testes leaves a raw surface which coupled accidental ligation of cord structures.
with the presence of the pulled through spermatic cord
results in effective closure of the internal inguinal ring. RefeRences
In this study we did not ligate the hernia sac during 1. Mohta A, Jain N, Irniraya KP, Saluja SS, Sharma S, Gupta A. Non
ligation of hernial sac during herniotomy: A prospective study.
inguinal orchiopexy. After dissecting the hernia sac free
Pediatr Surg Int 2003;19:451-2.
from the cord, we simply gently peel off the proximal 2. Shulman AG, Amid PK, Lichtenstein IL. Ligation of hernial sac-
cut end of the hernia sac as high as possible. We have A needless step in adult hernioplasty. Int Surg 1993;78:152-3.
performed 50 cases of inguinal orchiopexy with this 3. Schier F. Laparoscopic inguinal hernia repair- A prospective
personal series of 542 children. J Pediatr Surg 2006;41:1081-4.
procedure and followed up for 1.5 to three years. We
4. Schier F. An open internal inguinal ring is not an inguinal hernia.
did not find any complication or untoward effect. So Pediatr Surg Int 2007;23:825.
we conclude that ligation of hernia sac is not necessary 5. Handa R, Kale R, Harjai MM. Laparoscopic orchiopexy: Is closure
in inguinal orhiopexy. We found a few other advantages of the internal ring necessary? J Postgrad Med 2005;51:266-8.
also: Time saving: Several minutes of operating time are
saved as we can avoid the holding of the proximal cut
end of the hernial sac with multiple small haemostatic
Source of Support: Nil, Conflict of Interest: None declared.
forceps and suture ligating it, especially when the sac
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 66

Case Report Full text online at http://www.jiaps.com
Bilateral giant juvenile fibro adenoma of breasts
Madhumita Mukhopadhyay, Rishavdeb Patra1, Sima Mondal, Asit Ghosh, A. K. Ray1
Departments of Pathology and 1Pediatric Surgery, IPGME&R and SSKM Hospital, Kolkata-20, India
Address for correspondence: Dr. Madhumita Mukhopadhyay, 7E, Dinobandhu Mukherjee Lane, Sibpur, Howrah-711102,
West Bengal, India. E-mail: madhumitamukhopadhyay@ymail.com
ABSTRACT
An 11-year-old girl with rapidly enlarging bilateral breast lumps is reported. It was diagnosed as a case
of juvenile fibro adenoma following fine needle aspiration cytology and confirmed on histopathological
examination of the excised specimens.
KEY WORDS: Breast tumor, fine needle aspiration cytology, giant juvenile fibro adenoma, phyllodes tumor
DOI: *****
InTRodUcTIon followed excision of bilateral breast lumps conserving
the normal breast tissues along with nipple and areola.
Bilaterally symmetrical giant juvenile fibro adenomas Cut surface of the lumps were solid, grayish white
are very rare in prepubertal girls.[1] The lesions are well and bulging with whorled like fashion. Hematoxylin
circumscribed, lobulated and can cause ulceration and eosin (H and E) stain showed [Figure 3] increased
of the overlying skin because of rapid growth. [2] cellularity of stromal and parenchymal component.
Early diagnosis and treatment is essential since the Stromal cellularity increased but there was no focal
tumors cause gross disfigurement and psychological periductal concentrate of cells; a characteristic of
detriment.[2] phyllodes tumor. The patient is on regular follow-up
and doing well at six months follow-up. In this patient,
case RepoRT aged only 11 years, the tumor was removed, keeping
surrounding breast tissue and preserving nipple and
An 11-year-old premenarche girl presented with areola. We do not plan prosthesis or augmentation now.
bilateral, rapidly increasing breast lumps for two
months [Figure 1]. There was a history of dull ache dIscUssIon
in the breasts. There was no history of trauma, nipple
discharge, fever, anorexia or weight loss. There was no Giant juvenile fibro adenoma in the prepubertal age
significant family history. Clinical examination revealed group is almost always benign and should be treated
mobile, slightly tender lobulated lumps. The right with breast conserving surgery.[3,4] Juvenile giant fibro
breast lump measured 22 cm x 20cm and left breast adenoma should be distinguished from phyllodes
lump measured 18cm x 16cm. Both the lumps were tumor. The distinction is very important because giant
solitary, well circumscribed and not fixed to the deeper juvenile fibro adenoma should be treated with excision
structures. There was an area of skin ulceration overlying of lumps and preservation of surrounding normal breast
the lump on the right side [Figure 1]. There was no tissue (as much as possible). In phyllodes tumor, a rim
axillary lymphadenopathy. Routine hematological and of normal tissue should be included in the excised
biochemical examinations were within normal limits. lump. This distinct type of fibro adenoma that tends to
Chest X Ray was normal. Ultrasonography of both the occur in adolescents shows hypercellularity of glands
breasts showed heterogeneous parenchymal pattern. and stroma.[5] A plethora of names exist to designate
the lesion such as age related term Juvenile fibro
FNAC showed aggregates of cohesive epithelial cells adenoma and size related like giant or massive fibro
[Figure 2]. A few bipolar nuclei and bare nuclei were adenoma.[6] Giant fibro adenomas constitute about 4%
also noted. There was no evidence of malignancy. Then of all fibro adenomas in the breast.[6] The occurrence
67 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Mukhopadhyay, et al.: Juvenile fibroadenoma of breast
Figure 1: Bilateral Breast Tumors Figure 2: FNAC from Breast Lumps (MGG Stain; 40 x 10) Show
Aggregate of Cohesive Epithelial Cells
in both the breasts is rare. Isolated case reports are
available in the English literature.[10,11] Sometimes it
is difficult to diagnose by FNAC. In our patient, the
diagnosis was made clinically, substantiated by FNAC
and confirmed by histopathology [12,13] Fortunately,
majority of these tumors can be removed completely by
simple mastectomy, preserving the nipple and areola, as
was done in our patient.[14] Juvenile giant fibro adenoma
may recur after complete excision and the chance of
recurrence becomes less after third decade.[15]
RefeRences
1. Moore RL, Mungara A, Shayan K, Wallace AM. Bilaterally
symmetric juvenile fibroadenomas and tubular breast deformity
in a prepubescent girl. J Pediatr Surg 2007;42:1133-6.
Figure 3: Histopathology (H and E Stain; Photomicrograph- 10 x 10) 2. Park CA, David LR, Argenta LC. Breast Asymmetry: Presentation
Shows Increased Cellularity of Stromal and Parenchymal Component. of a giant Fibroadenoma. Breast J 2006;12:451-61.
No Pperiductal Concentrates of Cells 3. Simmons RM, Cance WG, Iacicca MV. A Giant Juvenile
Fibroadenoma in a 12-Year- Old Girl: A Case for Breast
Conservation. Breast J 2000;6:418-20.
of fibro adenoma which is large and at the same time 4. Baxi M, Agarwal A, Mishra A, Agarwal G, Mishra SK.
Multiple bilateral Juvenile fibroadenomas of breast. Eur J Surg
hyper cellular should be differentiated from virginal 2000;166:828-30.
hypertrophy and phyllodes tumor. Sometimes it is 5. Dehner LP, Hill DA, Deschryver K. Pathology of the breast in
difficult to distinguish clinically juvenile giant fibro children, adolescents and young adults. Semin Diagn Pathol
adenoma from phyllodes tumor.[6] Although malignant 1999;16:235-47.
6. Rattan K, Kumar S, Dhull AK, Kaushal V, Kaur P. Giant
tumors of the breast are rare in this age group, two per Fibroadenoma Mimicking Phyllodes tumor in a Young Female:
cent of all primary malignant breast lesions occur under A Cytological Dilemma. The Internet Journal of Third World
the age of 25 years in females.[7] Medicine 2008;6:2.
7. Stehr KG, Lebeau A, Stehr M, Grantzow R. Fibroadenoma of the
breast in an 11-year –old girl. Eur J Pediatr Surg 2004;14:56-9.
Various other conditions like lipoma, hamartoma, cysts 8. Muttarak M, Chaiwun B. Imaging of giant breast masses with
should be kept in the list of differential diagnosis.[8] pathological correlation. Singapore Med J 2004;45:132-9.
Most of the times, physical examination, imaging like 9. Rosai J. Rosai and Ackerman’s Surgical Pathology. Vol. 2. 9th ed.
2004. p. 1771-2.
ultrasonography of breasts, mammography and magnetic
10. Ahuja A, Seth A. Juvenile Fibroadenoma of breast. Ind Pediatr
resonance imaging fail to make the exact diagnosis.[6] 2005;42:72.
The stromal hypercellularity should be evaluated more 11. Gobbi D, Dall’Igna P, Alaggio R, Nitti D, Cecchetto G. Giant
carefully in terms of degree and atypicality. It is also fibroadenoma of the breast in adolescents: report of 2 cases. J
Pediatr Surg 2009;44:e39-41.
rare for phyllodes tumor to occur in young patients.[9] 12. Tse G M, Ma T K, Pang L M, Cheung H. Fine needle aspiration
cytologic features of mammary phyllodes tumor. Acta Cytol
Giant huvenile fibroadenoma simultaneously occurring 2002;46:855-63.
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 68

Mukhopadhyay, et al.: Juvenile fibroadenoma of breast
13. Simi U, Moretti D, Iaccioni P, Arganini G, Roncella M, Miccoli P, surgery and a review of literature. World J Surg Oncol 2008;11:117.
Giacomini G. Fine needle aspiration cytopathology of phyllodes 15. Stacey EM. Sternbergs’s Diagnostic Pathology. 4th ed. Lippincott,
tumor. Differential diagnosis with fibroadenoma. Acta Cytol Williams Wilkins; 2004. p. 371-2.
1988;32:63-6.
14. Liang MI, Ramaswamy B, Patterson CC, McKelvey MT, Gordillo
G, Nuovo GJ, Carson WE 3rd. Giant breast tumors: surgical Source of Support: Nil, Conflict of Interest: None declared.
management of phyllodes tumors, potential for reconstructive
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69 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Case Report Full text online at http://www.jiaps.com
Double aortic arches, esophageal atresia and tracheal
compression
Yameen Majid, Monali Warade, Zarina Aziz, G. A. Karthik
Department of Radiodiagnosis, Narayana Hrudayalaya Institute of Medical, Sciences Bangalore, India
Address for correspondence: Dr. Yameen Majid, Department of Radiodiagnosis, Narayana Hrudayalaya Institute of Medical,
Sciences, Bangalore, India. E-mail: yameenmajid@gmail.com
ABSTRACT
We report a case of double aortic arch in a 12-month-old male infant well delineated on 64 slice computed
tomography scan. It formed a complete vascular ring around the trachea compressing it. The symptoms
resolved after the surgical division of the ring.
KEY WORDS: Conotruncal malformations, double aortic arch, heart defects, esophageal atresia, vascular rings,
64 Slice helical CT
DOI: *****
InTRodUcTIon recurrent episodes of dysphagia, cyanotic episodes and
respiratory tract infection. At six months the patient
Double aortic arch refers to the congenital anomaly was admitted again because of severe stridor and
in which both the aortic arches remain patent and dyspnoea. Clinical examination on patient showed mild
form a complete vascular ring encircling the trachea respiratory distress, respiratory rate of 55/min, pulse
and esophagus.[1] It is a very rare cause of complete rate of 122b/min, SPO2 OF 93% with oxygen by hood
vascular ring in which both the right and left aortic and a blood pressure of 75/50. On systemic examination
arches are present to various extents and usually result the patient showed normal air conduction on both sides
in noncardiac morbidity especially compression of with mild bilateral crackles.
trachea.[2] Not many cases are known in literature of
this anomaly. Further, a complete tracheal ring with Bronchoscopy revealed compression of the trachea along
both functional arches is the rarest of all types of with laryngomalacia. Echocardiography did not reveal
double aortic arch. We report a case of complete double any significant abnormality. A suspicion of vascular
aortic arch with classical manifestations and very well ring was made on clinical grounds and bronchoscopy
delineated on 64 slice computed tomography (CT) findings and a contrast CT of chest was advised.
with complete resolution of symptoms after surgical Contrast enhanced helical CT was done using 64-slice
correction. Light speed VCT (GE Medical systems, Milwaukee,
Wisconsin). Maximum intensity projection (MIP) and
case RepoRT volume rendered (VR) images were also obtained for
better delineation of the anatomy. A complete functional
A 12-month-old male infant presented with history double aortic arch forming a vascular ring around the
of stridor. He was born at 39 weeks gestation and caudal aspect of trachea at the level of D4 vertebra was
weighed 2.5 kg. APGAR score was normal at one and seen causing significant narrowing of the trachea at
five minutes. The baby was previously operated for that level for a short segment of 0.6cm at about 5mm
esophageal atresia and tracheo esophageal fistula proximal to the bifurcation. [Figures 1-4,]. The right
(EA-TEF) (type 1 - communication between the lower sub clavian and common carotid arteries showed
parts of esophagus with trachea) at the age of three separate origins from the right aortic arch and left sub
days at another hospital but the stridor persisted and clavian and common carotid arteries showed separate
worsened with time. In addition the patient developed origins from the left aortic arch. [Figures 2 and 4] The
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 70

Majid, et al.: Running title missing?????
Figure 1: Axial CT chest shows double aortic arch Figure 2: Oblique reformats two arches unite to form single descending
aorta
Figure 3: Virtual bronchoscopy demonstrates tracheal compression Figure 4: 3D reconstruction: Double aortic arch encircling trachea
Red – Aorta, Pink- Trachea
two arches were seen to unite approximately five mm has been reported by Lotz[4] in a six-month-old boy who
above the level of carina to form a single descending had recurrent respiratory difficulty. In our case double
aorta. [Figure 2] After five days, the patient was operated aortic arch was associated with esophageal atresia and
through a posterolateral thoracotomy and the presence tracheo esophageal fistula.
of double aortic arch was confirmed. Ligation of the left
arch was done, distal to the subclavian artery. He was The usual presentation of double aortic arch is due
discharged home one week after this procedure. He to tracheal compression leading to symptoms of
had persistent mild stridor in the postoperative period. upper airway obstruction in the form of stridor or
There was no stridor at six months follow-up. dyspnoea.[5] In infants the early symptoms may also
be harsh breathing noted by parents. The patients
dIscUssIon may also present with symptoms similar to lower
respiratory infections, so respiratory viral studies are
The first reference of double aortic arch goes back to usually done to exclude infectious etiology. However, as
1737 by Hommel. Not much literature is available on double arch predisposes patients to viral infections the
double aortic arch; however there have been few case diagnosis of an infection does not exclude the possibility
reports of double aortic arch in association with other of double aortic arch. A routine chest radiograph
anomalies. One such case was reported by Talwar et may reveal indentation of the tracheal air column.
al.[3] in 2009. In their case a complete double aortic arch Echocardiography is not usually done initially because
was associated with Tetralogy of Fallot and absent left of the presentation of the patient is with respiratory tract
pulmonary artery. Another case of double aortic arch infections, and usually the diagnosis is made on cross
71 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Majid, et al.: Running title missing?????
sectional imaging like Magnetic Resonance Imaging distal to sub clavian artery.
(MRI)[6] and CT or barium esophagogram. However,
echocardiography can reliably diagnose double aortic RefeRences
arch and also is important in evaluation of associated
cardiovascular anomalies. Barium esophagogram 1. Stewart JA, Kincaid OW, Edwards JE. An Atlas of Vascular Rings
usually shows bilateral indentation of the esophagus. and Related Malformations of the Aortic Arch System Springfield.
2. Gross RE. Arterial malformations which cause compression of
MRI and CT are best imaging modalities for diagnosis trachea or esophagus. Circulation 1955;11:124-34.
and characterization of double aortic arch and provide 3. Talwar S, Gupta A, Choudhary SK, Airan B. Absent left
in addition complete information on arterial branching pulmonary artery and double aortic arch in tetralogy of Fallot:
pattern, extent of tracheal and esophageal obstruction reconstruction using homograft saphenous vein or iliac artery.
Interact CardioVasc Thorac Surg 2009;8:277-9.
and preoperative planning. Bronchoscopy may be done 4. Lotz J, Macchiarini P. Double aortic arch diagnosed by magnetic
in some patients in the evaluation of airway pathologic resonance imaging. N Engl J Med 2004;351:e20.
condition which shows pulsatile compression of the 5. Kirklin JW, Clagett OT. Vascular rings producing respiratory
obstruction in infants. Proc Staff Meet Mayo Clin 1950;25:360-7.
posterior and lateral walls of the trachea
6. McMahon CJ, Bezold LI, Vick GW 3rd. Double aortic arch in
D-transposition of the great arteries: Confirmation of dominant
Double aortic arches are classified into two types arch by magnetic resonance imaging. Tex Heart Inst J 2000;27:
depending upon the patency of the two arches: type 1, 398-400.
which has both arches functioning, type 2 which has 7. Griswold HE Jr, Young MD. Double aortic arch: Report of two cases
and review of literature. Pediatrics 1949;4:751-68.
one of the arches atretic and is further classified into
four subtypes depending upon location of the atretic
segment.[7] In our case a completely functioning double
aortic arch was seen. Treatment is usually surgical, the
Source of Support: Nil, Conflict of Interest: None declared.
most common being surgical ligation of the small arch
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J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 72

Case Report Full text online at http://www.jiaps.com
Embryogenesis of esophageal atresia: Is localized vascular
accident a factor?
Hemonta K. R. Dutta, Shree Harsh
Department of Pediatric Surgery, Assam Medical College & Hospital, Dibrugarh, Assam, India
Address for correspondence: Dr. Hemonta Kr. Dutta, Assam Medical College & Hospital, Dibrugarh, Assam-786 002, India.
E-mail: hemontdut@yahoo.co.in
ABSTRACT
Although several theories on embryogenesis of esophageal atresia have been proposed, none could explain
the whole spectrum of this anomaly. We report a new variant of esophageal atresia in which the two blind
pouches were joined by an atretic band. Histology of the atretic part showed groups of striated muscle
arranged haphazardly without any lumen. The existing theories on etiology of esophageal atresia cannot
explain this variant. However, localized vascular accident during intrauterine life resulting in disturbances
in regional microcirculation could be a possible factor as demonstrated by Louw and Barnard in relation
to jejunoileal atresia.[1] This is contrary to the current understanding that disproportionate growth of the
horizontal esophageal folds results in esophageal atresia.
KEY WORDS: Embryogenesis, esophageal atresia, variant
DOI: *****
InTRodUcTIon joined by an atretic segment about 24 mm long and two
mm thick. The segment seemed to have a few muscle
The embryogenesis of esophageal atresia (EA) is fibers. The atretic segment was excised, blind ends
poorly understood. Theories such as intra embryonic opened and a primary anastomosis performed. The
pressure, vascular accidents, failure of recanalization, patient had an uneventful recovery and was discharged
disproportionate growth of the lateral epithelial folds on 12th post operative day.
etc were inadequate to explain the whole spectrum of
this malformation. The latest theory based on electron dIscUssIon
microscopic studies suggests that overgrowth of a dorsal
horizontal fold in the region of the tracheoesophageal The esophagus can be identified at three weeks of
separation would result in EA and tracheoesophageal gestation. In the subsequent two weeks, elongation
fistula (TEF).[1] This theory also proposes that late of the esophagus occurs mainly due to ascent of the
ischemia of an already formed esophagus could result larynx. Ventral tracheal primordium develops at this
in pure EA. stage. Rosenthal proposed that this process involves
apposition of two lateral longitudinal epithelial
case RepoRT f o l d s . [ 2 ] Fa i l u r e o f t h i s a p p o s i t i o n l e a d s t o
tracheoesophageal fistula formation while apposition
The author observed a new variant in a patient with too posteriorly results in EA. Smith proposed that
pure esophageal atresia. A two-day old male baby during the rapid phase of esophageal elongation, over
weighing 2.6 kg presented with frothing from mouth, thinning of the esophagus results in EA.[3] Tandler’s
high respiratory rate and a flat abdomen. A catheter theory of failure of recanalization resulting in atresia
through the mouth did not go beyond 10 cm from the may explain the rare congenital esophageal web, but
lower jaw. Water soluble contrast study showed a blind fail to explain the full thickness defect in EA.[4] The
upper pouch. A right posterolateral thoracotomy was presence of abnormal branchial vessels seen in some
done. It was observed that both the blind ends were cases of EA suggest a coincident rather than causal
73 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Dutta, et al.: Report of a new variant of esophageal atresia
Figure 2: Excised band
Figure1: Atretic band
still other variants such as pure esophageal atresia
that showed spontaneous recanalization,[12] which
could be the subtype II5 of type 2 in Kluth’s atlas of
esophageal atresia with 2 blind esophageal ends and a
cyst occupying the intervening space.
Sinha et al. reported another variant with a TEF between
the lower pouch and trachea, with a cystic dilatation in
the midportion.[13] The tracheal end of the fistula was
obstructed by a membranous septum at both ends of
a cystic dilatation, leading to a diagnosis of pure EA
(gasless abdomen). In the present variant, which was
not reported in the literature earlier, two blind ends of
the esophagus are connected by an atretic band. The
band was 24 mm long and histology of the part showed
presence of disorganized striated muscle groups without
any lumen. This is similar to type II atresia of the small
Figure 3: Histology of atretic part shows disorganized striated muscle bowel. Etiology of this variant could be explained on
the basis of the antenatal vascular accident theory
proposed by Louw in jejunoileal atresia.[14] Through
relationship.[5,6] Based on detailed saggital sections their classic experimental study in 1955, Louw and
and scanning electronic microscopic studies on chick Barnard demonstrated that intrauterine vascular insult
embryos, Kluth and Habenicht proposed the theory
to the developing bowel results in variable degree of
of esophagotracheal separation by dorsal and ventral
atresia.[2] This study was further supported by some
horizontal folds.[7] Overgrowth of the dorsal fold results
other experimental studies.[15-17] Similar observations
in distal fistulas continuity with the trachea. Although
noted in humans led to wide acceptance of this theory.
this theory comes close to explaining many of the [18-20]
Jejunoileal atresia, in rare instances, has been found
types of EA and TEF, it fails to explain embryogenesis
to be associated with esophageal, gastric, duodenal and
of some variant of EA. Variants of esophageal atresia,
colonic atresias,[18-20] which may point to a common
reported in literature, such as esophageal atresia with
etiological factor.
distal tracheoantral fistula associated with congenital
intrathoracic stomach and situs inversus (SI), [8]
esophageal atresia with triple fistula,[9] esophageal RefeRences
atresia with an abnormally long lower segment 1. Louw JH, Barnard CN. Congenital intestinal atresia, Observations
that entered the trachea in the region of the upper on its origin. Lancet 1955;2:1065-7.
thoracic aperture, a long atretic proximal portion of 2. Rosenthal AH. Congenital atresia of the esophagus with
the lower segment, and an additional membrane in tracheoesophageal fistula, Report of 8 cases. Arch Pathology
1931;12:756-72.
the lower segment,[10] esophageal atresia associated 3. Smith IE. The early development of the trachea and esophagus in
with esophageal heterotopic pancreas[11] etc could not relation to atresia of the esophagus and tracheoesophageal fistula.
be explained on the basis of these theories. There are Contributions to Embryology 1957;36:41-57.
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 74

Dutta, et al.: Report of a new variant of esophageal atresia
4. Tandler J. Zur Entwicklungsgeschichte des menschlichen 13. Sinha CK, Gangopadhyaya AN, Sahoo SP, Gopal SC, Gupta
duodenums im fruhen Embryonal stadium. Gegenbaurs DK, Sharma SP. A new variant of esophageal atresia and
Morphologisches Jahrbuch 1900;29:187-216. tracheoesophageal fistula and duodenal atresia: a diagnostic
5. Lister J. Vascular vagaries: some reflections on the significance dilemma. Pediatr Surg International 1997;12:186-7.
of blood vessels in normal and abnormal development. J R Coll 14. Louw JH. Congenital intestinal atresia and severe stenosis in the
Surg Edinb 1978;23 :331-40. newborn. South African Journal of Clinical Science 1952;3:109-
6. Greenwood RD, Rosenthal A. Cardiovascular malformations 29.
associated with tracheoesophageal fistula and esophageal atresia. 15. Tsujimoto K, Sherman FE, Ravitch MM. Experimental intestinal
Pediatrics 1976;57:87-91. atresia in the rabbit fetus sequential pathological studies. Johns
7. Spitz L. Esophageal Atresia: Past, Present and Future. J Pediatr Hopkins Med J 1972;131:287-97.
Surg 1996;31:19-25. 16. Abrams JS. Experimental intestinal atresia. Surgery 1968;64:185-
8. Luo CC, Lin JN, Lien R, Chu SM. A new variant of esophageal 91.
atresia with distal tracheo-antral fistula associated with 17. Santulli TV, Blanc WA. Congenital atresia of the intestine.
congenital intrathoracic stomach and situs inversus. J Pediatr Pathogenesis and treatment. Annals of Surgery 1961;154:939-48.
Surg 2003;38:E25-7. 18. De Lorimier AA, Fonkalsrud EW, Hays DM. Congenital atresia
9. Kane TD, Atri P, Potoka DA. Triple fistula: Management of a double and stenosis of the jejunum and ileum. Surgery 1969;65:819-27.
tracheoesophageal fistula with a third H-type proximal fistula. J 19. Evans CH. Atresias of the gastrointestinal tract. International
Pediatr Surg 2007;42;6:e1–3. abstracts in surgery 1951;92:1-8.
10. Lambrecht W, Kluth D. Esophageal atresia: A new anatomic 20. Lister J, Rickham PP. Intestinal atresia and stenosis, excluding the
variant with gasless abdomen. J Pediatr surg 1994;29:564-5. duodenum. In: Rickham PP, Lister J, Irving I, editors. Neonatal
11. Ozcan C, Celic A, Erdener A. A new variant of esophageal atresia Surgery. Butterworths, London: 1978. p. 381-400.
associated with esophageal heterotopic pancreas. J Pediatr
Surg 2002;37:116–8.
12. Gunsar C, Sencan A, KaracaI, Mir E. Isolated esophageal atresia
with spontaneous recanalization: Case report. J Pediatr Surg Source of Support: Nil, Conflict of Interest: None declared.
2002;37:1210-2.
Author Queries?????
Figs not cited in text
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75 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Case Report Full text online at http://www.jiaps.com
Urethral substitution with ileum in traumatic bladder neck-
vagina fistula
Lavanya Kannaiyan, Sudipta Sen
Department of Paediatric Surgery, Christian Medical College and Hospital, Vellore, Tamil Nadu-632 004, India
Address for correspondence: Dr. Sudipta Sen, Department of Paediatric Surgery, Christian Medical College and Hospital, Vellore,
Tamil Nadu–632 004, India. E-mail: paedsur@cmcvellore.ac.in
ABSTRACT
A five-year-old girl presented with post traumatic urinary incontinence secondary to rupture of the bladder
neck into the vagina. Operative repair included a midline exposure with resection of the symphysis pubis,
separation of the bladder neck from the vagina, repair of the torn bladder neck and urethral substitution
with ileum. Result: Normal continence and voiding.
KEY WORDS: Ileum, rectus muscle flap, traumatic urethrovaginal fistula, urethral reconstruction
DOI: *****
InTRodUcTIon A three cm segment of ileum was isolated on its
pedicle to reach the perineum. The ileal segment was
Pelvic trauma in the female child can result in total loss tubularized over an 8 feeding tube in the form of the
of the urethra and rupture of the bladder neck into the Yang- Monti tube. It was then anastomosed proximally
vagina.[1,2] We report a case where this was reconstructed to the urethral stump and distally to the introitus at
with bladder neck repair and urethral substitution with the site of expected urethral orifice. The lower half of
a monti tube, resulting in complete cure of incontinence the left rectus muscle was mobilized as a flap with its
without the need for intermittent catheterization. inferior attachment to the pubis left intact and carefully
preserving the inferior epigastric vessels. This muscle
case RepoRT flap was placed between the posterior bladder wall
and the vagina to prevent refistulization.[3] The anterior
A five-year-old girl presented with urinary incontinence vaginal wall was left unrepaired as the posterior and
following a history of pelvic trauma. She had a single lateral vaginal walls were intact and closure of the
orifice at the introitus from which she was continuously anterior vaginal wall may result in stenosis. The anterior
leaking urine. On endoscopic examination the bladder vaginal wall was left to reepithelialize over the rectus
neck was found opening into the vagina. Operative muscle interposed between the posterior bladder wall
reconstruction proceeded [Figure 1]. Operative exposure and vagina. An appendicular mitrofanoff was also
was obtained with a midline vertical incision from created opening at the umbilicus. We contemplate
the umbilicus to the vaginal orifice with division and closing the mitrofanoff port when the child desires it.
partial excision of the symphysis pubis. The remnant of
the proximal urethra and bladder neck were dissected At follow-up after 10 months, she is voiding normally
off the anterior vaginal wall at the area of the bladder with continent intervals of four to six hours. Residual
neck-vaginal fistula. The rupture in the posterior aspect urine measured after voiding was two ml. Ultrasound
of the bladder neck was then repaired anatomically and serum creatinine remain normal and the micturating
resulting in a normal appearing bladder neck with a cystourethrogram was satisfactory [Figure 2]. She uses
short proximal urethral stump which would not reach the mitrofanoff channel once a day to maintain its
the perineum. patency.
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 76

Kannaiyan, et al.: Running title missing?????
Figure 1: Schematic Diagram of the operative procedure. (a) Lower
Figure 2: Post operative micturating cystourethrogram. Bladder was
midline incision including the mons pubis and the anterior part of
filled via the mitrofanoff port and the patient voided via the reconstructed
the vulval outlet. Anterior pubic symphysis excised. (b) Pathological
bladder neck and ileal neourethra
anatomy exposed showing posterior rupture of bladder neck into
the anterior vagina. Distal urethra destroyed by injury. (c) Surgical
separation of bladder neck from the vagina. (d) Bladder neck repaired RefeRences
posteriorly and connected to monti tube neourethra. Left lower rectus
muscle flap placed between reconstruction and vagina to prevent 1. Dorairajan LN, Gupta H, Kumar S. Pelvic fracture-associated
refistulization. Appendicular mitrofanoff also created urethral injuries in girls: experience with primary repair. BJU
Int 2004;94:134-6.
2. Huang CR, Sun N, Wei-ping, Xie HW, Hwang AH, Hardy BE. The
dIscUssIon management of old urethral injury in young girls: analysis of 44
cases. J Pediatr Surg 2003;38:1329-32.
Traumatic posterior urethral loss in boys can be made up 3. Sen S, Zachariah N, Chacko J, Thomas G. Buttressing the divided
by mobilizing and rerouting the remaining urethra. The bladder neck by a rectus abdominis muscle flap to prevent
urethral recanalisation in paediatric urinary incontinence. Pediatr
short female urethra is often totally destroyed in pelvic
Surg Int 2003;19:124-6.
injury and has to be substituted.[1] Various methods have 4. Dabernig J. Neo-urethra preformation in free anterolateral thigh
been described including – bladder and buccal mucosa, flap. J Plast Reconstr Aesthet Surg 2006;59:779-80.
anterior bladder tube, ureters, appendix, anterior 5. Xu YM, Qiao Y, Sa YL, Zhang J, Zhang HZ, Zhang XR, Wu DL,
Chen R. One-stage urethral reconstruction using colonic mucosa
lateral thigh free flap, pedicled island skin, amnion graft: an experimental and clinical study. World J Gastroenterol
grafts, fallopian tube, and colonic mucosa.[5-11] Tapered 2003;9:381-4.
ileum has been used as a perineal mitrofanoff stoma 6. Kelemen Z, Romics I, Pajor L. Substitution of the distal female
for patients with urinary incontinence of congenital urethra with a vaginal flap and pedicled skin island. BJU Int
2002;89:459-61.
origin.[12] 7. Brandt FT, Albuquerque CD, Lorenzato FR. Female Urethral
reconstruction with amnion grafts. Int J Surg Investig 2000;1:1409-14.
We have successfully used the monti tube for posterior 8. Hemal AK, Dorairajan LN, Gupta NP. Posttraumatic complete and
urethral substitution in boys.[13] It is more difficult to partial loss of urethra with pelvic fracture in girls: an appraisal
of management. J Urol 2000;163:282-7.
bring the appendix to the perineum based on a single 9. Sheldon CA, Gilbert A. Use of the appendix for urethral
blood vessel. It was easier to bring the appendix to reconstruction in children with congenital anomalies of the
abdominal wall. The Monti-Yang tube is more versatile bladder. Surgery 1992;112:805-11.
in its blood supply and has further reach than the 10. Ahmed S, Neel KF. Urethral injury in girls with fractured pelvis
following blunt abdominal trauma. Br J Urol 1996;78:450-3.
appendix. In the girl reported herein, we have used a 11. Barroso U Jr, Duel B, Barthold JS, Gonzalez R. Orthotopic urethral
similar method of urethra substitution after separation substitution in female patients using the mitrofanoff principle .
of the bladder neck from the vagina and repairing J Urol 1999;61:251-3.
the posterior tear in the bladder neck resulting in 12. Kumar J, Sen S, Dange A, Kumar A. Use of ileum in reconstruction
of the congenitally abnormal posterior urethra in two children.
complete continence without the need for intermittent J P Urol 2007;3:333-6.
catheterization. The continence of the bladder neck
is due the anatomical repair of the injured bladder
in a previously normal bladder, hence, resulting in
a normally functioning bladder neck after the repair
with a loss of urethra which was substituted. We have
also used a rectus muscle flap to prevent vesicovaginal
Source of Support: Nil, Conflict of Interest: None declared.
refistulization.
77 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

Images Full text online at http://www.jiaps.com
Bathing trunk nevus
A. Y. Kshirsagar, K. S. Shukla, Y. P. Nikam, R. B. Garg, T. U. Sholapurkar
Department of Surgery, Krishna Institute of Medical Sciences, University, Karad, India
Address for correspondence: Dr. Ashok Y. Kshirsagar, Department of Surgery, Krishna Institute of Medical Sciences, University,
Malkapur, Karad – 415 110, Dist. Satara, Maharashtra, India. E-mail: drayk@indiatimes.com
KEY WORDS: Bathing trunk nevus, congenital nevomelanocytic nevus, giant congenital pigmented nevi
DOI: *****
We report a one-day-old male child born with bathing malignant melanoma. The exact cause is unknown.
trunk hairy nevus. Congenital hairy nevus denotes a There may be hereditary factors, autosomal dominance
pigmented surface lesion present at birth in one per cent or other multifactor determinants. Management depends
of newborns, but incidence of giant congenital pigmented upon size, location and malignant transformation.
nevi is less than 1 in 20,000 births. These nevi are Surgical excision is the mainstay of treatment. Cultured
significant because of association with leptomeningeal epidermal autograph has been used successfully to cover
melanocytosis or neurofibromatosis and predispose to postoperative large surface area defect.
Figure 1: Anterior view of child with costume hairy nevus
J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 78