TY - JOUR
T1 - Amniotic band syndrome with CNS involvement
T2 - a pediatric neurosurgeon’s dilemma—a case series and literature review
AU - Sobana, Mirna
AU - Halim, Danny
AU - Faried, Ahmad
AU - Riestiano, Betha Egih
AU - Pajan, Hendra Fajar
AU - Islam, Andi Asadul
AU - Dariansyah, Ahmad Data
AU - Parenrengi, Muhammad Arifin
N1 - Publisher Copyright:
© 2023, The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.
PY - 2023/8
Y1 - 2023/8
N2 - 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.
AB - 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.
KW - Amniotic band syndrome
KW - Detached fibrous amniotic band
KW - Facial cleft
KW - Finger amputation
KW - Head lump
KW - Strangulated developing organ
KW - Syndactyly
UR - http://www.scopus.com/inward/record.url?scp=85160821487&partnerID=8YFLogxK
U2 - 10.1007/s00381-023-06008-w
DO - 10.1007/s00381-023-06008-w
M3 - Review article
C2 - 37266681
AN - SCOPUS:85160821487
SN - 0256-7040
VL - 39
SP - 2003
EP - 2017
JO - Child's Nervous System
JF - Child's Nervous System
IS - 8
ER -