Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed

Neel, Nada; Tarabay, Mohmoud Salem

doi:10.4103/ua.ua_193_17

Cited by 3 publications

(8 citation statements)

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“…Mortality is very high due to obstruction of the urinary tract or a combination of renal, pulmonary, and cardiac complications [18]. These could explain the occurrence of deaths in our cases.…”

Section: Discussionmentioning

confidence: 53%

“…These could explain the occurrence of deaths in our cases. According to Neel and Tarabay [18], patient survival also depends on the optimal management and level of advancement of pediatric surgery, and therefore pediatric health care in general [19]. In other words, OEIS syndrome patient survival is an indicator of the quality of care in pediatric surgery [16] [18].…”

Section: Discussionmentioning

confidence: 99%

“…According to Neel and Tarabay [18], patient survival also depends on the optimal management and level of advancement of pediatric surgery, and therefore pediatric health care in general [19]. In other words, OEIS syndrome patient survival is an indicator of the quality of care in pediatric surgery [16] [18]. In developing countries like ours, the prognosis is very poor [12] [14], because despite successful surgery, the risk of anal and urinary incontinence remains high [18].…”

Section: Discussionmentioning

confidence: 99%

“…In other words, OEIS syndrome patient survival is an indicator of the quality of care in pediatric surgery [16] [18]. In developing countries like ours, the prognosis is very poor [12] [14], because despite successful surgery, the risk of anal and urinary incontinence remains high [18]. There are views that the physical, emo-Open Journal of Pediatrics tional, and financial burden is too great to justify correction [4].…”

Section: Discussionmentioning

confidence: 99%

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Oeis Syndrome (Cloacal Exstrophy): About Two Cases Treated at the Mother and Child Teaching Hospital in Cotonou (Benin)

Gbénou¹,

Assan²,

Akodjènou³

et al. 2020

OJPed

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Introduction: OEIS syndrome, the most severe form of exstrophy-epispadias complex, is characterized by the existence of an omphalocele, a bladder exstrophy, an anal imperforation and a spina bifida. Case Presentation: Two clinical cases admitted at the first day of life in neonatology department are reported; one was born by caesarean section, weighing 2500 g and the other one vaginally with a weight at 1890 g. The first child's mother was a primigravid, primiparous and the second mother was multiparous. Prenatal ultrasound was performed in one case. The clinical examination found two exstrophied hemi-bladders with two productive ureteral meatus, separated by a double intestinal orifice, a manifestation of anal imperforation, omphalocele, spina bifida and lumbar vertebral malformations; which corresponds to a typical form of OEIS syndrome. One of the babies had an associated clubfoot. The karyotype could not be performed. Both were dead; the first in the early postoperative period, the second one at 16 days of life without surgery. Conclusion: OEIS syndrome is a rare condition and represents a therapeutic challenge in developing countries. Promoting prenatal diagnosis is essential.

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Section: Discussionmentioning

confidence: 53%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Oeis Syndrome (Cloacal Exstrophy): About Two Cases Treated at the Mother and Child Teaching Hospital in Cotonou (Benin)

Gbénou¹,

Assan²,

Akodjènou³

et al. 2020

OJPed

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“…Surgical repair is always challenging due to the complexity of these malformations and requires that multidisciplinary teams include neonatologists, pediatric surgeons, pediatric urologists, pediatric orthopedists, pediatric neurosurgeons, and genetic and pediatric endocrinologists. 6 , 7 …”

Section: Discussionmentioning

confidence: 99%

Omphalocele, Exstrophy of Cloaca, Imperforate Anus, and Spinal Defects Complex: A Case Report

et al. 2023

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Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defects complex is a rare malformationcomplex that includes omphalocele, cloacal exstrophy, imperforate anus and spinal defects with theincidence of 1 in 200,000 to 400,000 pregnancies and is even rarer in twin gestation. The etiology ofthis complex is still unclear. Most cases are sporadic. Prenatal screening must be done for diagnosisand appropriate multidisciplinary management of cases. In severe cases, termination of pregnancy isconsidered. We present a 4-day first twin child with underdeveloped ambiguous genitalia deliveredvia emergency lower section cesarean section at 32+3 weeks of gestation with giant liver containingomphalocele, cloacal exstrophy, imperforate anus and meningocele with severe pulmonary arteryhypertension 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.

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Asociación poco frecuente del complejo OEIS con un defecto diafragmático

Torres,

Montaña-Jiménez,

Ramirez-Corredor

et al. 2023

Andes pediatr

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El complejo OEIS (onfalocele, extrofia cloacal, ano imperforado y defectos espinales) forma parte de los defectos de pared abdominal. Es una entidad infrecuente, que conlleva alta morbimortalidad y requiere de manejo multidisciplinario. Objetivo: Reportar un caso de asociación poco frecuente entre el complejo OEIS y hernia diafragmática; discutir su patogénesis y posible asociación con otras malformaciones de línea media. Caso Clínico: Neonato pretérmino, con diagnóstico antenatal de onfalocele. Al nacer destacó onfalocele gigante con amnios íntegro que contenía todo el hígado y asas, extrofia vesical con vejiga abierta y orificios uretrales expuestos, útero didelfo, epispadias, no se palparon gónadas, mediante imágenes se logró evidenciar extrusión de los tejidos blandos de pelvis, hernia diafragmática izquierda (Bochdalek), malformaciones óseas, mielomeningocele y mielocistocele. Con dichos hallazgos, se planteó complejo OEIS con presentación simultánea de pentalogía Cantrell o deleción 1p36, en el estudio genético se descartaron cromosomopatías, pero no se pudo completar estudio más específico. Se realizó colostomía, cierre de cloaca y afrontamiento a línea media de placa vesical, con adecuada evolución postoperatoria. Dada estabilidad respiratoria, se difirió corrección quirúrgica de hernia diafragmática. Se dio de alta después de una larga hospitalización, falleciendo por patología respiratoria aguda intercurrente. Conclusiones: Dentro de las asociaciones clínicas del complejo OEIS la hernia diafragmática es infrecuente, lo que podría corresponder a una forma inusual del complejo, debido a presentación simultánea con pentalogía Cantrell o deleción 1p36. A pesar de ser una malformación de presentación esporádica, es importante tener conocimien- to de sus características y variantes.

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Omphalocele, exstrophy of cloaca, imperforate anus, and spinal defect complex, multiple major reconstructive surgeries needed

Cited by 3 publications

References 9 publications

Oeis Syndrome (Cloacal Exstrophy): About Two Cases Treated at the Mother and Child Teaching Hospital in Cotonou (Benin)

Oeis Syndrome (Cloacal Exstrophy): About Two Cases Treated at the Mother and Child Teaching Hospital in Cotonou (Benin)

Omphalocele, Exstrophy of Cloaca, Imperforate Anus, and Spinal Defects Complex: A Case Report

Asociación poco frecuente del complejo OEIS con un defecto diafragmático

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