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.