Mayer-Rokitansky-Kuster-Hauser syndrome with H-type anovestibular fistula

Mahajan, Jai Kumar; Venkatesh, M. A.; Bawa, Monika; Rao, K. L. N.

doi:10.1016/j.jpedsurg.2009.04.015

Cited by 8 publications

(3 citation statements)

References 11 publications

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“…A thorough evaluation for these associations should be made in any female with an H-type fistula. Other congenital anomalies reported by others in association with H-type fistulas include vertebral, urologic, cardiac, and genital anomalies [1,5,14].…”

Section: Discussionmentioning

confidence: 94%

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Management of H-type rectovestibular and rectovaginal fistulas

Lawal

Chatoorgoon

Bischoff

et al. 2011

Journal of Pediatric Surgery

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Section: Discussionmentioning

confidence: 94%

“…These include perineal repair, fistulectomy, abdominoperineal repair, limited or formal PSARP, vestibuloanal pull-through, anterior perineal anorectoplasty, and a transanal approach [1,2,[4][5][6][7][8][9]14]. The major complications are fistula recurrence (5%-30%) and wound dehiscence (0%-25%).…”

Section: Discussionmentioning

confidence: 99%

Management of H-type rectovestibular and rectovaginal fistulas

Lawal

Chatoorgoon

Bischoff

et al. 2011

Journal of Pediatric Surgery

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“…The inheritance pattern described is sporadic or autosomal dominant with low or incomplete penetrance [3]. The extension of the phenotype of the spectrum includes vesico-vaginal as well ano-vaginal fistula [4,5].…”

Section: Introductionmentioning

confidence: 99%

MURCS association with situs inversus totalis: Expanding the spectrum or a novel disorder

2015

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We are reporting a female patient with a MURCS association (Müllerian duct aplasia, unilateral renal agenesis, cervico-thoracic somite fusion defects), situs inversus totalis, short stature with normal development and intelligence. We are presenting the comparison with two other patients published with similar finding. Our patient is distinct in having all the characteristic features and represents the severe spectrum of this disorder. We present our argument favoring this to be a monogenic syndrome distinct from the other two entities and probably a ciliopathy.

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Diversities of H-type anorectal malformation: a systematic review on a rare variant of the Krickenbeck classification

Sharma

Gupta

2016

Pediatr Surg Int

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Congenital H-type fistula is a rare congenital rectourogenital connection with an external anal opening in a normal or ectopic position. A systematic review was done to study the anatomical types of congenital H-type fistula, embryology, clinical presentation, relative gender distribution, associated anomalies, investigative modalities, and recent advances in treatment of these lesions. A PubMed search included H-type anorectal malformation; H-type anorectal malformations; H-type anorectal; and H-type congenital anorectal that gave 9;43;76;26 abstracts, respectively. Relevant studies and cited articles were studied omitting duplicate search. The reported incidence is 0.1-16 % of all anorectal malformation. The H-type anorectal malformation is 2.5-6 times more common in females and usually associated with a normal anus. In males, the anomaly is usually a variant with an ectopic anus or a perineal fistula. Anatomical types include anovestibular; rectovestibular; rectovaginal fistula in females and rectourethral (bulbar, prostatic, bladder neck) and rectovesical fistula in males. Variants identified include H-type fistula with perineal fistula, perineal groove, H-type sinus, H-type canal, and acquired H-type fistula. This review compiles the available literature over last six decades. Various surgical corrective procedures have been described. The high recurrence decreases with a learning curve and experience.

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Mayer-Rokitansky-Kuster-Hauser syndrome with H-type anovestibular fistula

Cited by 8 publications

References 11 publications

Management of H-type rectovestibular and rectovaginal fistulas

Management of H-type rectovestibular and rectovaginal fistulas

MURCS association with situs inversus totalis: Expanding the spectrum or a novel disorder

Diversities of H-type anorectal malformation: a systematic review on a rare variant of the Krickenbeck classification

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