1. A tale behind the rectum
A. Mathew, V. Abraham, U. Shankar, S.V. Thomas, M.R. Jesudason and
B. Perakath
ABSTRACT
Tailgut cysts, also called benign retrorectal hamartomas, are uncommon developmental
cysts found behind the rectum. Here, we present a rare case of a tailgut cyst associated
with uterine anomaly, sacral and vertebral anomalies and vascular duplication, in a young
lady who presented with constipation and infertility.
KEYWORDS: tailgut cysts, retrorectal hemartomas
Introduction
Tailgut cysts are uncommon retrorectal cystic tumours. We
present a rare case of tailgut cyst with subdural extension
and associated uterine didelphys, hemisacral deficiency and
double inferior vena cava.
Case Report
A 24-year old lady presented with a history of low back pain
and difficulty in defecation worsening over the previous 5
years. She was also infertile.
Digital rectal examination revealed a large mass pushing
the rectum anteriorly; the overlying rectal mucosa was mobile.
The upper border of the mass was not palpable, nor was it
palpable per abdomen.
Magnetic resonance imaging of the abdomen and pelvis
revealed a cystic mass in the presacral and precoccygeal
region measuring 11 cm x 10 cm x 10 cm with solid
components. The lower part of the left hemisacrum was
deficient with part of the cyst extending into the sacral spinal
canal. She also had uterus didelphys with double inferior
vena cava and coronal clefts in the 4th
and 5th
lumbar vertebrae
(Figure 1)
Biochemistry, including serum beta human chorionic
gonadotropin and alpha-fetoprotein, were normal.
She underwent excision of the retrorectal cyst including
its sacral extension (Figures 2 and 3). Peroperatively, there
was a cerebrospinal fluid leak which was managed with a
lumbar subdural catheter.
Gross examination of the specimen showed an ovoid
cystic mass measuring 10 cm x 6 cm x 6cm with a weight of
150 g. The cut section revealed multiple loculations with many
smooth walled cysts containing pultaceous material. A focal
nodule was seen, which was also cystic on sectioning. The
microscopic appearance of the cyst lining was mucin
secreting columnar epithelium with adjacent smooth muscle
bundles and fibrous connective tissue on the external aspect.
The cyst within the nodule was lined by stratified squammous
epithelium enclosing lamellated keratin and a focus of
granuloma composed of epithelioid cells and foreign body
multinucleate giant cells. No immature elements were
present.
Department of Colorectal Surgery,
CMCH, Vellore, India
Correspondence:
Benjamin Perakath
Email- benjamin@cmcvellore.ac.in
CaseReport
© Tropical Gastroenterology 2008
Discussion
Tailgut cysts (also known as benign retrorectal harmartomas
or mucin-secreting cysts) are enteric developmental cysts of
the presacral space. Others in this category are the
epidermoid, dermoid, cystic rectal duplication and neurenteric
cysts.1
They are found in the female adult population and can
be asymptomatic or present with compression symptoms
(as in our case) or as pilonidal sinuses, anorectal fistulae or
recurrent retrorectal abscesses. Rare case reports of
malignant transformation2
and carcinoid tumour3
arising in a
tailgut cyst have been reported.
Whereas several cases of tailgut cysts per se, as well as
those with sacral defects have been reported2
, their
association with uterine didelphys, vertebral anomalies and
double IVC have not been reported. In 1981, Currarino4
described a syndrome in infants which consists of a triad of
presacral cystic masses, sacral agenesis and anorectal
malformation. These have an autosomal dominant
inheritance associated with mutations in the HLXB9 gene.5
These presacral masses comprised the anterior sacral
meningocele, cystic teratomas or enteric cysts.
Tropical Gastroenterology 2008.29;2:103–104
Figure 1: MRI of the tailgut cyst shows the sacral defect.

2. A literature search revealed that rectal duplication cysts6
and anterior sacral meningoceles1
have been associated
with uterus didelphys but only one other case report of a
tailgut cyst associated with uterine didelphys was found. This
was the case of a twelve-year old girl who had mental
retardation, and had hypothyroidism, sacral and coccygeal
agenesis but no chromosomal anomalies.7
Abnormalities of the lower urinary and genital tracts as a
result of Mullerian and Wollfian duct abnormalities have been
reported but none with retrorectal cysts viz. the Mayer
Rokitansky syndrome, which includes uterine didelphys,
imperforate vagina and renal agenesis.8
Double inferior vena cava in itself is a rarity. There are
isolated case reports of its association with renal aplasia9
,
congenital hepatic fibrosis and lung dysgenesis. No report
of double IVC with retrorectal cysts has been published till
date.
Embryologically, tailgut cysts are thought to be the
persistent part of the hind gut in the region of the embryonic
tail that normally involutes.1, 2
Pathologically, tailgut cysts are
usually multicystic or multiloculated and lined by a variety of
epithelia (stratified squammous, transitional, stratified
columnar, mucinous or ciliated columnar, ciliated
pseudostratified columnar or gastric). Well formed but
disorganised smooth muscle fibres are focally present in its
wall unlike the well formed continuous two layer muscle coat
seen in rectal duplication cysts. They are dissimilar to benign
cystic teratomas which contain distinct dermal appendages,
neural elements or mesenchymal derivatives like cartilage
or bone.1
Excision of the tailgut cyst results in its cure. This case
was reported to highlight the congenital anomalies that can
also be associated with this condition.
References
1. Dahan H, Arrivé L, Wendum D, Docou le Pointe H, Djouhri H,
Tubiana JM. Retrorectal developmental cysts in adults: clinical
and radiologic-histopathologic review, differential diagnosis, and
treatment. Radiographics. 2001;21:575–84.
2. Prasad AR, Amin MB, Randolph TL, Lee CS, Ma CK. Retrorectal
cystic hamartoma: report of 5 cases With Malignancy Arising in
2. Arch Pathol Lab Med. 2000;24:725–9.
3. Horenstein MG, Erlandson RA, Gonzalez-Cueto DM, Rosai J.
Presacral carcinoid tumors: a report of 3 cases and review of
literature. Am J Pathol. 1998;22:251–5.
4. Currarino G, Coln D, Votteler T. Triad of anorectal, sacral and
presacral anomlies. AJR Am J Roentgenol. 1981;137:395–8.
5. Kim IS, Oh SY, Choi SJ, Kim JH, Park KH, Park HK, et al. Clinical
and genetic analysis of HLXB9 gene in Korean patients with
Currarino syndrome. J Hum Genet. 2007;52:698–701.
6. Nour S, Kumar D, Dickson JA. Anorectal malformations with sacral
bony abnormalities. Arch Dis Child. 1989;64:1618–20.
7. Galluzzo ML, Bailez MM, Reusmann A, Gonzalez R, Davila MT.
Tailgut cyst (Retrorectal hamartoma): Report of a pediatric case.
Pediatr Dev Pathol 2007;22:1.
8. Stassart JP, Nagel TC, Prem KA, Phipps WR. Uterus didelphys,
obstructed hemivagina and ipsilateral renal agenesis: the
University of Minnesota experience. Fertil Steril.
1992; 57:756–61.
9. Gayer G, Zissin R Strauss S, Hertz M. IVC anomalies and right
renal aplasia on CT: a possible link? Abdom Imaging.
2003;28:395–9.
Figure 2: The figure shows the uterus didelphys displaced anteriorly
by the cyst.
Tropical Gastroenterology 2008.29;2:103–104
Figure 3: The figure shows the retrorectal cyst.