A female baby was born at 37 weeks and 6 days gestation by vaginal delivery with omphalocele, exstrophy of the cloaca, and imperforate anus, indicating the presence of OEIS complex, a rare combination of defects consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S), associated with lumbosacral lipoma. The most common associated spinal deformity is terminal myelocystocele, and spinal lipoma is rare. Constructive interference in steady-state magnetic resonance imaging clearly revealed double lipomas, a dorsal-type lipoma, located dorsal to the low-lying conus medullaris, and a filar-type lipoma, revealed by a thickened and fatty filum terminale. After recovery from abdominogenital repairs, debulking of the dorsal-type lipoma and untethering of the spinal cord by sectioning of the filar-type lipoma were performed at the age of 14 months. Neurosurgical treatment for occult spinal dysraphism should be undertaken after recovery from the initial series of major abdominogenital procedures.

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http://dx.doi.org/10.2176/nmc.49.487DOI Listing

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Background: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare, life-threatening congenital malformation primarily treated with abdominogenital repair. The optimal indication and timing of neurosurgical interventions for the associated spinal cord lesions remains insufficiently studied. We reviewed spinal dysraphism in OEIS to evaluate the best timing for neurosurgical intervention.

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Bladder exstrophy-epispadias-cloacal exstrophy complex (BEEC) is a spectrum of congenital urologic anomalies that involve the bladder, urethra, genitalia, and pelvic musculoskeletal system, and can affect urinary continence, sexual health, and fertility. BEEC includes a wide spectrum of anatomical abnormalities with different levels of severity: epispadias represents the mildest phenotype, classic bladder exstrophy (CBE) is the most common defect, and cloacal exstrophy (CE) - often referred to as omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex - is the most severe form. BEEC disorders cause significant health problems and affect the health-related quality of life (QoL) of affected individuals.

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Article Synopsis
  • OEIS complex is a rare birth defect involving omphalocele, bladder exstrophy, anal imperforation, and spina bifida, likely caused by multiple factors.
  • A case is reported involving a 29-year-old woman pregnant with dizygotic twins, where the condition was suspected at 22 weeks through ultrasound and confirmed with MRI.
  • The study highlights the importance of prenatal imaging in diagnosing OEIS complex for better counseling and management options before and after birth.
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Study Design: These diagnostic criteria were developed by an expert multidisciplinary and multi-institutional team based on analysis of peer-reviewed data, followed by electronic-Delphi consensus of a panel of 61 international pediatric specialists.

Results: After 2 Delphi rounds, a 92% or higher level of agreement was reached for each Delphi statement.

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Introduction And Importance: OEIS complex is a rare and complex anomaly of the genitourinary and intestinal tract. It includes Omphalocele, Exstrophy of cloaca, Imperforate anus and Spinal defects.

Presentation Of Case: We are reporting a case of OEIS complex.

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