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Published on: June 24, 2020
[Amniotic band syndrome: pathogenesis, prenatal diagnosis and neonatal management]
L Sentilhes1, E Verspyck, S Patrier
1Clinique Gynécologique et Obstétricale, Pavillon Mère-Enfant, CHU Charles Nicolle, 1, rue de Germont, 76031 Rouen. loicsentilhes@hotmail.com
Insights
Amniotic band syndrome causes fetal malformations. While prenatal surgery is debated, Doppler studies may predict limb amputation, guiding in utero treatment decisions for this congenital condition.
Area of Science:
- Medical Genetics
- Developmental Biology
- Prenatal Diagnosis
Background:
- Amniotic band syndrome (ABS) is a spectrum of congenital malformations affecting fetal development.
- Proposed etiologies include early amnion rupture (exogenous) or fetal vascular compromise (endogenous).
Observation:
- ABS manifestations range from limb defects to craniofacial and visceral abnormalities.
- The severity dictates pregnancy management, with termination for severe cases and postnatal surgery for minor limb defects.
- In utero lysis of amniotic bands is considered for isolated limb constriction to prevent amputation.
Findings:
- The precise etiology of ABS remains unknown, contributing to its unpredictable natural course.
- Prenatal surgical interventions for ABS are controversial due to the unpredictable nature of the condition.
- Doppler studies of constricted limbs may offer predictive value for in utero amputation.
Implications:
- Accurate prenatal diagnosis and risk stratification are crucial for managing ABS.
- Doppler ultrasound may aid in decision-making for potential in utero interventions.
- Further research into ABS etiology is needed to refine prenatal management strategies.
Abstract:
Amniotic band syndrome is a collection of fetal congenital malformations, affecting mainly the limbs, but also the craniofacial area and internal organs. Two mains pathogenic mechanisms are proposed: early amnion rupture (exogenous theory) leading to fibrous bands, which entrap the fetal body; the endogenous theory privileges vascular compromise, mesoblastic strings not being a causal agent. The outcome of the disease depends on the gravity of the malformation. Termination of the pregnancy is usually proposed at the time of the diagnostic of severe craniofacial and visceral abnormalities, whereas minor limb defects can be repaired with postnatal surgery. In case of an isolated amniotic band with a constricted limb, in utero lysis of the band can be considered to avoid a natural amputation. However, as the exact etiology of amniotic band syndrome remains unknown and its natural course unpredictable, prenatal surgery continues to be controversial. Doppler studies of the constricted limb could be of useful predictive value of in utero amputation, and therefore could be helpful to determine when in utero treatment should be considered.
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