Modern treatment of cloacal exstrophy

Ricketts, Richard R.; Woodard, John R.; Zwiren, Gerald T.; Andrews, Hilary; Broecker, Bruce H.

doi:10.1016/0022-3468(91)90993-4

Cited by 55 publications

(19 citation statements)

References 20 publications

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“…4 Ricketts et al treated 12 newborns with cloacal extrophy over a period of 10 years. 6 Stolar et al treated 14 patients with cloacal extrophy over a 23-year period, 7 and Cywes treated 24 infants with cloacal extrophy over 30-year period. 8 The exact embryogenesis of cloacal extrophy is not known, and many theories have been suggested, however, no single theory seems to adequately explain all the abnormalities seen in the condition, but the most accepted theory is that cloacal extrophy results from premature rupture of the cloacal membrane prior to caudal migration of the urorectal septum, and fusion of the genital tubercles.…”

Section: Discussionmentioning

confidence: 99%

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An Unusual Variant of Cloacal Extrophy

et al. 2002

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Section: Discussionmentioning

confidence: 99%

“…This should be done in the immediate newborn period with early orchidectomy. [4][5][6][7] In a large series of 20 patients with cloacal extrophy, 13 (65%) were genetically males, and all but one were given the female gender. 4 Males with adequate bilateral or unilateral phallic structures should, however, be raised as males.…”

Section: Discussionmentioning

confidence: 99%

An Unusual Variant of Cloacal Extrophy

et al. 2002

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“…Until the 1980s, most patients with this condition were left to die [10, 11]. In fact, one of the families in this series had been informed by an outside physician that the disorder was routinely fatal and was advised not to treat the patient.…”

Section: Discussionmentioning

confidence: 99%

“…This would indicate an extensive disruption of the caudal cell mass. However, cloacal exstrophy may also occur in the absence of a terminal myelocystocele [10, 11]. In addition, other forms of closed NTDs may occur [12, 13].…”

Section: Embryologymentioning

confidence: 99%

Long-Term Outcome of Terminal Myelocystocele Patients

Choi

McComb²

2000

Pediatr Neurosurg

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Introduction: A terminal myelocystocele, a closed form of a neural tube defect (NTD), can present as a large, fully epithelialized, cystic lumbosacral mass containing fat, cerebrospinal fluid (CSF) and neural tissue. The spinal cord terminates at a neural placode wherein the central canal opens into a CSF-filled cavity that is distinct from fluid in the subarachnoid space surrounding the spinal cord. This form of NTD, in our experience, was only associated with major caudal cell mass abnormalities, as these patients often have maldevelopment of the lower spine, pelvis, genitalia, bowel, bladder, kidney and the abdominal wall. This study will describe the clinical manifestations, surgical management and long-term outcome of our terminal myelocystocele patients. Methods: To characterize this rare entity, a 13-year retrospective review was undertaken at our institution. Results: Nine patients with terminal myelocystoceles were identified. In all cases, there were multiple congenital defects including cloacal exstrophy, imperforate anus, omphalocele, pelvic deformity, equinovarus or renal abnormality. Only 1 out of 9 patients has required a shunt for hydrocephalus. The main goal of neurosurgical intervention was to reduce the size of the mass, which can slowly enlarge over time. The spinal cord was also untethered, although these patients have no chance of bowel or bladder control. With a mean follow-up of 63 months, all patients remained neurologically stable. Impairment of lower extremity function is usually severe. However, some patients were ambulatory with the aid of a walker or orthotic device. All patients required a prolonged hospital stay as well as multiple operations prior to initial discharge. Conclusion: Experienced, multispecialty care is needed to optimize the long-term outcome of these complex patients.

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“…The focus of multidisciplinary care for exstrophy patients has shifted to quality of life because survival of these patients has approached 100% in the absence of lethal anomalies [4,5]. It is generally accepted that the current surgical approach for initial management of these patients includes omphalocele repair, diversion of the fecal stream, closure of the bladder, and osteotomies if necessary.…”

mentioning

confidence: 99%