ABSTRACT
PURPOSE: Persistent cloaca is one of the most severe types of anorectal malformation. Appropriate initial drainage is difficult due to their various malformations and hydrocolpos or dilated urinary bladder. Corrective surgery also differs among individual patients. We describe our experiences with the surgical management of children with persistent cloaca. METHODS: We retrospectively reviewed 16 children diagnosed with persistent cloaca at Asan Medical Center. RESULTS: Sixteen patients were managed in their neonatal period. Twelve patients had enlarged bladder or vagina at birth. Three patients, who did not undergo cystostomy or vaginostomy at first operation, had earlier complications after surgery or required drainage tube insertion. One patient who did not undergo hydrocolpos drainage died of sepsis and complications. Nine patients underwent corrective surgery; posterior sagittal anorectovaginourethroplasty using the Pena method. Three patients required additional operations due to complications after surgery. CONCLUSION: Patients found to have anatomical malformations before colostomy, as well as hydrocolpos and bladder enlargement, require a vaginostomy with or without a cystostomy to reduce complications. Follow-up is required in patients with hydrocolpos and bladder enlargement to determine whether vaginal drainage improves dilated bladder. Continuous long-term follow-up examination is required to determine the long-term results of corrective surgery.
Subject(s)
Child , Humans , Anus, Imperforate , Cloaca , Colostomy , Cystostomy , Drainage , Follow-Up Studies , Hydrocolpos , Parturition , Retrospective Studies , Sepsis , Urinary Bladder , VaginaABSTRACT
Persistent cloaca is a very rare congenital anomaly with a single common perineal opening for the genital urinary and gastrointestinal tract, which is caused by abnormal formation of the urorectal septum. It has an incidence of 1 in 50,000 to 1 in 125,000 births and is much more common in females and in twin pregnancies. Pathologic findings of persistent cloaca include dilated bowel, hydrocolpos, urethral obstruction, hydronephrosis and oligohydramnios caused by obstruction of the bladder, vagina and intestine. Failure of the paired m llerian ducts to fuse also usually results in duplication of the uterus and vagina. Currently, the diagnosis depends on the prenatal sonography but the diagnosis may be very difficult due to the complex nature of the anomaly and variable appearances. We present a case of persistent cloaca with one opening confirmed by autopsy after therapeutic termination which was initially diagnosed by prenatal sonography.
Subject(s)
Female , Humans , Pregnancy , Autopsy , Cloaca , Diagnosis , Gastrointestinal Tract , Hydrocolpos , Hydronephrosis , Incidence , Intestines , Oligohydramnios , Parturition , Pregnancy, Twin , Urethral Obstruction , Urinary Bladder , Uterus , VaginaABSTRACT
Persistent cloaca is a very rare congenital anomaly with a single common perineal opening for the genital, urinary and gastrointestinal tracts, which arises from defects in the embryonal process of descent of urorectal septum or fusion to cloacal membrane. Anomalies associated with persistent cloaca are found in the upper urinary tract, gastrointestinal tract, cardiovascular system, central nervous system and respiratory system, which are not easily detected through ultrasound during pregnancy. It has been reported that if those anomalies are diagnosed early, they can be corrected surgically. We present a case of persistent cloaca with hydrocolpos confirmed by autopsy after emergency cesarean section which was initially presented as a huge abdominal cystic mass and bilateral hydronephrosis by ultrasound at 34 weeks of gestation.