# Understanding and managing a case of the omphalocele-exstrophy-imperforate anus-spinal defect complex

**Authors:** Khulud Mahmood Nurani, Waleed Abdalla, Khalid Mohamed Ayub

PMC · DOI: 10.1093/jscr/rjae166 · Journal of Surgical Case Reports · 2024-03-17

## TL;DR

This paper presents a case of a rare birth defect complex involving multiple organ systems and describes the surgical management and outcomes.

## Contribution

The paper contributes a detailed clinical case report of OEIS complex with comprehensive surgical and post-operative management insights.

## Key findings

- The neonate presented with multiple anomalies including exstrophy of the cecum, imperforate anus, and spina bifida.
- Surgical interventions such as Ladd’s bands release, ileostomy, and ureterostomy were successfully performed.
- The patient remained stable post-surgery, indicating the effectiveness of the surgical approach.

## Abstract

The omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare birth disorder involving a combination of gastrointestinal, musculoskeletal, renal, neural, and genitourinary system defects. We present a case report of a neonate with OEIS born by vertex spontaneous delivery to non-consanguineous parents. The major presenting defect was exstrophy of the cecum lying between two exstrophied halves of the bladder, an imperforate anus and spina bifida myelomeningocele. Explorative laparotomy revealed a duplex kidney system, attachment of the terminal ileum and cecum to the exstrophy, and a sausage-like atretic large bowel. Malrotation was also noted. Ladd’s bands were released followed by ileostomy and ureterostomy. Patient was stable post-operatively and in the incubator on room air. OEIS remains a challenging anomaly that requires optimal treatment to facilitate the quality of life. With appropriate surgical intervention, the functional and social treatment goals for this unusual congenital defect can be met.

## Linked entities

- **Diseases:** OEIS complex (MONDO:0017919), spina bifida (MONDO:0008449), imperforate anus (MONDO:0001046)

## Full-text entities

- **Diseases:** exstrophy (MESH:D001746), congenital defect (MESH:D000013), OEIS (MESH:C537748), spinal defect (MESH:D013122), Malrotation (MESH:C562456), exstrophy of the cecum (MESH:D002430), omphalocele (MESH:D006554), spina bifida myelomeningocele (MESH:D016135), imperforate anus (MESH:D001006), birth disorder (MESH:D025063), gastrointestinal, musculoskeletal, renal, neural, and genitourinary system defects (MESH:D014564)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

2 figures with captions in the complete paper: https://tomesphere.com/paper/PMC10948741/full.md

## References

10 references — full list in the complete paper: https://tomesphere.com/paper/PMC10948741/full.md

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Source: https://tomesphere.com/paper/PMC10948741