Mayer-Rokitansky-Kuster-Hauser syndrome and anal canal stenosis: case report and review of literature.
Mayer-Rokitansky-Kuster-Hauser (MRKH) is a characteristic syndrome in which the mullerian structures are absent or rudimentary. It is also associated with anomalies of the genitourinary and skeletal systems. There are very few cases reported regarding its association with anorectal malformations, particularly perineal fistulas. To the best of our knowledge, there have not been any reported cases of anal canal stenosis in patients with MRKH. We describe a very rare association of MRKH with anal canal stenosis and multiple renal calculi. The patient underwent corrective surgery for the anomalies and removal of renal calculi. She has been under regular follow-up for the last few months and is doing well.
Department of Pediatric Surgery, Sri Aurobindo Institute of Medical Sciences, Indore (MP), Mumbai-400063 India. milindj79@yahoo.com
Milind
M
Joshi
Sangram
S
Singh
Tanmay
T
Vyas
Vighya
V
Chourishi
Anvesh
A
Jain
D058489:46, XX Disorders of Sex Development / Q000000981:diagnostic imaging / Q000196:embryology / Q000473:pathology / Q000601:surgery; D000015:Abnormalities, Multiple / Q000000981:diagnostic imaging / Q000196:embryology / Q000473:pathology / Q000601:surgery; D000293:Adolescent; D001003:Anal Canal / Q000002:abnormalities* / Q000601:surgery; D000714:Anastomosis, Surgical; D002908:Chronic Disease; D012809:Colon, Sigmoid / Q000601:surgery; D000013:Congenital Abnormalities; D003248:Constipation / Q000209:etiology; D003251:Constriction, Pathologic; D005260:Female; D006399:Hematocolpos / Q000209:etiology; D006801:Humans; D006869:Hydronephrosis / Q000209:etiology; D007668:Kidney / Q000002:abnormalities; D007669:Kidney Calculi / Q000209:etiology* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008572:Menarche; D009095:Mullerian Ducts / Q000196:embryology / Q000002:abnormalities; D019170:Somites / Q000002:abnormalities; D013131:Spine / Q000002:abnormalities; D019738:Surgically-Created Structures; D014463:Ultrasonography; D014599:Uterus / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000196:embryology / Q000473:pathology / Q000601:surgery; D014621:Vagina / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000196:embryology / Q000473:pathology / Q000601:surgery*