Rectovestibular fistula

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Rectovestibular fistula - Wikipedia

From Wikipedia, the free encyclopedia

A rectovestibular fistula, also referred to simply as a vestibular fistula, is an anorectal congenital disorder where an abnormal connection (fistula) exists between the rectum and the vulval vestibule of the female genitalia.

If the fistula occurs within the hymen, it is known as a rectovaginal fistula, a much rarer condition.^[1]

Presentation

Complications

If a colostomy is not performed immediately after birth, patients with rectovestibular fistulae may present later in life with complications including severe constipation and megacolon (abnormal dilation of the colon), requiring colostomy or further surgery.^[2]

Cause

A rectovestibular fistula occurs when the colon or rectum deviates anteriorly.^[1] This leads to the formation of an abnormal connection between the rectum and the vulval vestibule, immediately posterior to the hymen, so that an ectopic anus opens into the fourchette, or frenulum, of the labia minora.^[3] A common wall is shared between the rectum and vagina above the site of the fistula.^[2] Although the opening of the rectum into the anal canal is shorter and narrower than normal, the rectum itself is completely normal.^[2]

Diagnosis

The diagnosis of a rectovestibular fistula can be made in female newborns if the vulva is stained with meconium (the earliest form of stool in an infant).^[3] The opening of the anus may be difficult to see due to its small size and position, but it may be visible as a thickening of the median perineal raphe with an obvious anal dimple.^[3]^[2] Patients with rectovestibular fistulae are commonly misdiagnosed with rectovaginal fistulae.^[1]

Management

Colostomy is recommended by most surgeons, and has a good prognosis, with 90% of patients regaining normal bowel control.^[4] Since the rectal opening and anal orifice in a vestibular fistula tend to be short and narrow, a colostomy is usually performed to allow decompression of the bowel unless the orifice is wide enough to allow normal defecation.^[2] Colostomy is often followed by posterior sagittal anorectoplasty (PSARP), a surgical procedure to repair the anal orifice, at a later date.^[4]^[5] Some surgeons prefer to perform an immediate PSARP without a colostomy first, while others perform neither a colostomy nor a PSARP and instead opt for a simple dilatation of the orifice to allow stool to pass and the bowel to decompress.^[5] It has been suggested that only experienced surgeons should perform repair without an initial colostomy.^[1]

Epidemiology

Rectovestibular fistula is the most common defect of the rectum and anal canal in females.^[3]

References

^ ^a ^b ^c ^d Corman, Marvin L. (2005). Colon and Rectal Surgery. Lippincott Williams & Wilkins. pp. 575–576. ISBN 9780781740432.
^ ^a ^b ^c ^d ^e Puri, Prem (2003). Newborn Surgery (2nd ed.). CRC Press. p. 539. ISBN 9780340761441.
^ ^a ^b ^c ^d Rintala, Risto J. (2005). "Congenital anorectal anomalies". Paediatric Surgery (2nd ed.). CRC Press. pp. 157–158. ISBN 9780340809105.
^ ^a ^b Browne, Nancy Tkacz (2008). Nursing Care of the Pediatric Surgical Patient. Jones & Bartlett Learning. p. 305. ISBN 9780763768034.
^ ^a ^b Mattei, Peter (2011). Fundamentals of Pediatric Surgery. Springer Science+Business Media. p. 500. ISBN 9781441966438.

Congenital malformations and deformations of digestive system

Upper GI tract

Tongue, mouth and pharynx	Cleft lip and palate Van der Woude syndrome tongue Ankyloglossia Macroglossia Hypoglossia
Esophagus	EA/TEF Esophageal atresia: types A, B, C, and D Tracheoesophageal fistula: types B, C, D and E Esophageal inlet patch esophageal rings Esophageal web (upper) Schatzki ring (lower)
Stomach	Pyloric stenosis Hiatus hernia

Lower GI tract

Intestines	Intestinal atresia Duodenal atresia Meckel's diverticulum Hirschsprung's disease Intestinal malrotation Dolichocolon Enteric duplication cyst
Rectum/anal canal	Imperforate anus Rectovestibular fistula Persistent cloaca Rectal atresia

Accessory

Pancreas	Annular pancreas Accessory pancreas Johanson–Blizzard syndrome Pancreas divisum
Bile duct	Choledochal cysts Caroli disease Biliary atresia
Liver	Alagille syndrome Polycystic liver disease

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