Amniotic band syndrome with CNS involvement: a pediatric neurosurgeon’s dilemma—a case series and literature review

Mirna Sobana, Danny Halim, Ahmad Faried, Betha Egih Riestiano, Hendra Fajar Pajan, Andi Asadul Islam, Ahmad Data Dariansyah, Muhammad Arifin Parenrengi

Research output: Contribution to journalReview articlepeer-review

1 Citation (Scopus)

Abstract

Background: Amniotic band syndrome (ABS) is a rare congenital disease characterized by a broad spectrum of congenital anomalies resulting from the strangulated developing organ(s) by the detached fibrous amniotic band. The prevalence of CNS involvement in ABS is rare, but the mortality rate in these cases is high, while morbidity among the surviving patients is inevitable. Case report: Three-month-old male, 9-month-old female, and newborn female babies were presented with head lump(s), severe facial cleft, syndactyly, and finger amputation. The patient’s head imaging confirmed meningoencephalocele as the cause of the head lump in 2 patients; meanwhile, a porencephalic cyst was identified as the origin of head lumps in the other patient. VP shunt placement surgery was performed as the initial management in 2 patients, while one patient directly underwent meningoencephalocele resection surgery. Craniofacial and limb reconstructions were planned as the follow-up management in all cases. Unfortunately, one patient died of complications from suspected aspiration, while another never returned for follow-up treatment. Conclusion: Here, we report 3 ABS cases with CNS involvement. Despite the severe disfigurement and disability, the inexistence of fatal malformation might lead to long-term survival. The treatment of malformation(s) that might predispose to another fatal condition and surgery(-ies) to improve functional outcomes and patient’s social acceptability should be prioritized in managing the surviving ABS patients.

Original languageEnglish
Pages (from-to)2003-2017
Number of pages15
JournalChild's Nervous System
Volume39
Issue number8
DOIs
Publication statusPublished - Aug 2023

Keywords

  • Amniotic band syndrome
  • Detached fibrous amniotic band
  • Facial cleft
  • Finger amputation
  • Head lump
  • Strangulated developing organ
  • Syndactyly

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