Low anorectal malformation in females without fistula: A rare entity
V Upadhyaya, A Gangopadhyaya, A Pandey, D Gupta, A Upadhyaya
Keywords
anorectal malformation, female, fistula
Citation
V Upadhyaya, A Gangopadhyaya, A Pandey, D Gupta, A Upadhyaya. Low anorectal malformation in females without fistula: A rare entity. The Internet Journal of Surgery. 2006 Volume 12 Number 1.
Abstract
Anorectal malformations (ARM) are one of the most common congenital defects. Male and female patients are equally affected. Low anorectal malformations are common in males as well in females. Most of the male low ARM present without fistula. Low ARM in female without any fistula is very rare. We are presenting 3 cases of low ARM in female without fistula (anocutaneous, vestibular and vaginal). All these patients were treated by cut-back anoplasty. None of the patients were associated with any other congenital anomaly. Postoperative results were excellent.
Introduction
Imperforate anus has been a documented anomaly since antiquity. It has an incidence of 1 in 4000 newborns. Anorectal malformations affect all racial, social, cultural and economic groups equally. Low anorectal malformations without fistula are common in males whereas low anorectal malformations with fistula are common but low anorectal malformations without fistula are very rare in females. It has been consistently shown that low anorectal malformations predominate in female children1,2 (perineal fistulas, anteriorly placed anus, and ano-vestibular fistula). Low anorectal malformation of females without fistula is very rare and accounts for less than 1% cases of ARM in females. In one study, low ARM without fistula in females was reported up in to 4.5%3 whereas in one large series from India not a single case of low ARM without fistula in females was reported4. We are presenting three cases of low anorectal malformation without fistula.
Case history
All the patients presented at 2 -4 days of age with absence of the anal opening (Fig 1). On careful examination no fistulous opening was present in the perineum or in the fourchette. Abdominal distension was present. All the patients were operated by cut back anoplasty. The patients are in regular follow-up and anal dilation.
Figure 1
Discussion
ARMs occur quite commonly5, 6. Many systems of classification have been suggested; however, the terms high and low have been broadly recognized and applied worldwide. Pena reports the most common lesion to be ARM with recto-urethral fistula in males and cloacae amongst females7; however, the most commonly seen defect in the Liverpool series was anocutaneous fistula 8 and rectovestibular followed by perineal fistulas type of malformation in other series9. In males, high defects were more common whereas intermediate defects were commonest in females in the reports form India4. We are presenting three cases of low anorectal malformations in females without any fistula, their management and follow-up. The importance of this article is rarity of the disease, and such case has not been reported previously.
Conclusion
Low anorectal malformation without fistula in females is a very rare entity; management is very simple.
Correspondence to
Dr. Vijai D. Upadhyaya Department of Pediatric Surgery IMS, BHU Varanasi, 221005 U.P. India upadhyayavj@rediffmail.com