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Jiaps April June 09 17.08.09

  1. 1. Editorial Full text online at http://www.jiaps.com 47 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2
  2. 2. J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 48
  3. 3. 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
  4. 4. 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
  5. 5. 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
  6. 6. 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
  7. 7. 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 1999. p. 124-39. Wilms tumor. J Pediatr Surg 1990;25:330-1. 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
  8. 8. 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
  9. 9. 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
  10. 10. 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. J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 56
  11. 11. Sousa, et al.: Psycological issues in cleft lip and palate 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 FORM IV Statement about ownership and other particulars about newspaper (J Indian Assoc Pediatr Surg) to be published in the first issue every year after the last day of February 1. Place of publication : Mumbai 2. Periodicity of its publication : Quaterly (January-March, April-June, July-September, October-December) 3. Printer’s Name : Medknow Publications and Media Pvt. Ltd. Nationality : Indian Address : A-108/109, Kanara Business Center, Off Link Rd, Ghatkopar (E), Mumbai - 400075, India Phone: 91-22-6649 1818/1816, Fax: 91-22-6649 1817 4. Publisher’s Name : Medknow Publications and Media Pvt. Ltd. Nationality : Indian Address : A-108/109, Kanara Business Center, Off Link Rd, Ghatkopar (E), Mumbai - 400075, India Phone: 91-22-6649 1818/1816, Fax: 91-22-6649 1817 5. Editor’s Name : K. L. N. Rao Nationality : Indian Address : Department of Pediatric Surgery, Advanced Pediatric Center, Post Graduate Institute of Medical Education and Research, Chandigarh - 160012, India. 6. Names and addresses of individuals who own the newspaper and partners or shareholders holding More than one per cent of the total capital. : Indian Association of Pediatric Surgeons I, hereby declare that the particulars given above are true to the best of my knowledge and belief. Date: 10th August 2009 K. L. N. Rao 57 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2
  12. 12. 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
  13. 13. 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
  14. 14. 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
  15. 15. Memarzadeh, et al.: Application of immunohistochemical staining 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. Author Help: Online submission of the manuscripts Articles can be submitted online from http://www.journalonweb.com. For online submission, the articles should be prepared in two files (first page file and article file). Images should be submitted separately. 1) First Page File: Prepare the title page, covering letter, acknowledgement etc. using a word processor program. All information related to your identity should be included here. Use text/rtf/doc/pdf files. Do not zip the files. 2) Article File: The main text of the article, beginning with the Abstract to References (including tables) should be in this file. Do not include any informa- tion (such as acknowledgement, your names in page headers etc.) in this file. Use text/rtf/doc/pdf files. Do not zip the files. Limit the file size to 400 kb. Do not incorporate images in the file. If file size is large, graphs can be submitted separately as images, without their being incorporated in the article file. This will reduce the size of the file. 3) Images: Submit good quality color images. Each image should be less than 1024 kb (1 MB) in size. The size of the image can be reduced by decreas- ing the actual height and width of the images (keep up to about 6 inches and up to about 1200 pixels) or by reducing the quality of image. JPEG is the most suitable file format. The image quality should be good enough to judge the scientific value of the image. For the purpose of printing, always retain a good quality, high resolution image. This high resolution image should be sent to the editorial office at the time of sending a revised article. 4) Legends: Legends for the figures/images should be included at the end of the article file. 61 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2
  16. 16. 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
  17. 17. 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
  18. 18. 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
  19. 19. 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
  20. 20. 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

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