RÉSUMÉ
Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defects complex is a rare malformation complex that includes omphalocele, cloacal exstrophy, imperforate anus and spinal defects with the incidence of 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestation. The etiology of this complex is still unclear. Most cases are sporadic. Prenatal screening must be done for diagnosis and appropriate multidisciplinary management of cases. In severe cases, termination of pregnancy is considered. We present a 4-day first twin child with underdeveloped ambiguous genitalia delivered via emergency lower section cesarean section at 32+3 weeks of gestation with giant liver containing omphalocele, cloacal exstrophy, imperforate anus and meningocele with severe pulmonary artery hypertension and non-visualization of right kidney and ureter, absence of uterus, fallopian tubes and right ovary. Separation and repair of the cecum and bladder were done. The ladd procedure was performed. Ileostomy was created and single-stage repair of the abdominal wall was done. Keywords: anorectal malformations; bladder exstrophy; case reports; neural tube defects; umbilicus.
Sujet(s)
Malformations multiples , Imperforation anale , Exstrophie vésicale , Hernie ombilicale , Enfant , Animaux , Humains , Grossesse , Femelle , Imperforation anale/diagnostic , Imperforation anale/chirurgie , Imperforation anale/complications , Hernie ombilicale/diagnostic , Hernie ombilicale/chirurgie , Hernie ombilicale/complications , Cloaque/chirurgie , Cloaque/malformations , Césarienne/effets indésirables , Malformations multiples/diagnostic , Malformations multiples/chirurgie , Exstrophie vésicale/diagnostic , Exstrophie vésicale/chirurgie , Exstrophie vésicale/étiologieRÉSUMÉ
Continuous urinary leakage, along with normal deliberate voiding, must suggest diagnosis of ectopic ureter, specifically in girls. Combination of a duplicated collecting system with distal, infra-sphincteric, vaginal insertion of ureter is an uncommon congenital anomaly and rare cause of urinary incontinence. We present a case report of a 7-year-old girl who presented to the urology department with urinary incontinence despite successful toilet training and history of recurrent urinary tract infections. Right duplex collecting system was seen on ultrasound. Magnetic resonance urography revealed a near complete right duplex collecting system with ectopic insertion of ureter into vagina, and aplastic uterus with bilateral normal ovaries suggestive of Mayer-Rokitansky-Kuster-Hauser syndrome. Surgical treatment consisted of "common sheath" reimplantation of ectopic ureters into bladder, with complete resolution of symptoms. This case suggests to us that congenital abnormalities of the genitourinary tract should be considered in case of urinary incontinence and recurrent urinary tract infections.