# Case Report: A “senior” with serpentine-like syndrome—treatment of combined brachyoesophagus, intrathoracic stomach and cervical rachischisis

**Authors:** Judith Lohmann, Anna Freund, Tobias Klein, Daniel Acero, Thomas Boemers

PMC · DOI: 10.3389/fped.2024.1378234 · Frontiers in Pediatrics · 2024-09-10

## TL;DR

This case report describes a 5-year-old with a rare syndrome involving stomach and spine malformations, highlighting treatment challenges and the need for further research.

## Contribution

The novelty lies in presenting the oldest known patient with serpentine-like syndrome and emphasizing comprehensive care strategies.

## Key findings

- A 5-year-old patient is the oldest documented case of serpentine-like syndrome.
- The report reviews treatment approaches and outcomes from previously published cases.
- The syndrome is associated with high mortality and complex anatomical anomalies.

## Abstract

Serpentine-like syndrome, characterized by the combination of intrathoracic stomach, a notably short esophagus anomaly, splenic abnormalities, and cervical spine malformations, has been associated with a high mortality rate since its identification in 2008. This report presents the case of a remarkable patient who recently celebrated her fifth birthday, marking her as the oldest documented individual with this syndrome to date. Highlighting the significance of comprehensive evaluations for concurrent malformations, the report discusses potential treatment modalities and challenges inherent in managing patients with this intricate syndrome. A comprehensive review of previously published cases is provided, comparing surgical interventions, causes of death, and age at the time of demise. This report underscores the importance of ongoing research and collaborative efforts to optimize outcomes for individuals afflicted with serpentine-like syndrome.

## Linked entities

- **Diseases:** serpentine-like syndrome (MONDO:0034895)

## Full-text entities

- **Diseases:** stomach (MESH:D013272), short esophagus anomaly (MESH:D004938), death (MESH:D003643), splenic abnormalities (MESH:D013158), Serpentine-like syndrome (MESH:C537586), malformations (MESH:C564254), rachischisis (MESH:D016135), cervical spine malformations (MESH:D002575)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11420036/full.md

## References

12 references — full list in the complete paper: https://tomesphere.com/paper/PMC11420036/full.md

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Source: https://tomesphere.com/paper/PMC11420036