Focused Clinical Observation of Functional Outcomes in Children With <scp>OEIS</scp> Complex

Omphalocele, Cloacal Exstrophy, Imperforate Anus, Spinal Defects (OEIS) complex is an extremely rare congenital disorder, and data on long-term functional and developmental outcomes remain scarce. We aimed to evaluate ambulatory, urological, and intellectual outcomes in a cohort of patients with OEIS complex. We retrospectively reviewed 10 patients with OEIS complex born at our institution between 1991 and 2021. Spinal defects were classified radiologically or intraoperatively. Ambulatory and urinary outcomes were assessed at the most recent follow-up, and intellectual status was evaluated in patients who were aged ≥ 6 years. At a mean follow-up of 114 months (range, 4-348), five patients were independently ambulatory, two were ambulatory with orthoses, and two were nonambulatory. Two patients achieved dry time with clean intermittent catheterization, while seven remained incontinent. Among seven school-aged patients, five attended regular schools, one required special education, and one could not attend due to complications. Spinal anomalies included spinal lipomas (n = 6) and terminal myelocystoceles (n = 4). In this cohort, long-term ambulatory and intellectual outcomes were generally favorable in the absence of severe complications, whereas urinary continence remained challenging. Given the rarity of OEIS complex, additional multi-institutional and multidisciplinary studies with extended follow-up are needed.

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Publication Details

Journal
Congenital Anomalies
Published
2026-01-01
DOI
https://doi.org/10.1002/cga.70054
Primary Topic
Congenital gastrointestinal and neural anomalies
Type
article
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article

Focused Clinical Observation of Functional Outcomes in Children With OEIS Complex

Hiroko Sato, Ryutaro Fukuyama, Daisuke Hirokawa, Osamu Yazawa et al.
Congenital Anomalies
Congenital gastrointestinal and neural anomalies
article

Focused Clinical Observation of Functional Outcomes in Children With OEIS Complex

Hiroko Sato, Ryutaro Fukuyama, Daisuke Hirokawa, Osamu Yazawa, Takeshi Yamamoto, Kaoru Koyama, Hirokuni Honma
article en

Abstract

Omphalocele, Cloacal Exstrophy, Imperforate Anus, Spinal Defects (OEIS) complex is an extremely rare congenital disorder, and data on long-term functional and developmental outcomes remain scarce. We aimed to evaluate ambulatory, urological, and intellectual outcomes in a cohort of patients with OEIS complex. We retrospectively reviewed 10 patients with OEIS complex born at our institution between 1991 and 2021. Spinal defects were classified radiologically or intraoperatively. Ambulatory and urinary outcomes were assessed at the most recent follow-up, and intellectual status was evaluated in patients who were aged ≥ 6 years. At a mean follow-up of 114 months (range, 4-348), five patients were independently ambulatory, two were ambulatory with orthoses, and two were nonambulatory. Two patients achieved dry time with clean intermittent catheterization, while seven remained incontinent. Among seven school-aged patients, five attended regular schools, one required special education, and one could not attend due to complications. Spinal anomalies included spinal lipomas (n = 6) and terminal myelocystoceles (n = 4). In this cohort, long-term ambulatory and intellectual outcomes were generally favorable in the absence of severe complications, whereas urinary continence remained challenging. Given the rarity of OEIS complex, additional multi-institutional and multidisciplinary studies with extended follow-up are needed.

Congenital AnomaliesVol. 66(1)
Kanagawa Children's Medical Center (JP), Yokohama City University (JP)
Quality Education
Openalex Percentile: Top 3%
Congenital gastrointestinal and neural anomalies
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