LAPORAN KASUS: Eksensefali sebagai Salah Satu Komplikasi Amniotic Band Syndrome

Dewi Maharsita Sri Prajanta Putri, Hadi Susiarno, Budi Handono

Abstract


Abstrak
Amniotic band syndrome(ABS) merupakan kelainan non genetik dengan spektrum luas dari  konstriksi dan amputasi sederhana pada jari ekstremitas hingga kelainan kraniofasial mayor hingga defek visera bahkan kematian. Beberapa laporan kasus menyebut anensefali merupakan contoh defek kraniofasial yang dihubungkan dengan ABS. Sonografi membantu diagnosis eksensefali sebagai kondisi peralihan menuju kondisi terminal anensefali sebagai kondisi yang lebih sering ditemukan yaitu kondisi otak janin sudah habis teresorbsi oleh cairan serebrospinal. Fetus dengan eksensefali tidak dapat bertahan hidup, sebagian besar berakhir dengan abortus, intrauterine fetal death dan stillbirth sehingga jika terdeteksi perlu dipertimbangkan untuk dilakukan terminasi kehamilan. Berdasar laporan kasus persalinan spontan Bracht di kamar bersalin RS Hasan Sadikin pada 1 Desember 2016, Ny RA 33 tahun, G3P1A1 gravida 30-31 minggu, letak sungsang, eksensefali, anemia.
Pada laporan kasus ini ditemukan membran amniotik berfusi dengan kranium bayi dan absennya tulang tengkorak pada tempat perlekatan membran amnion.  Eksensefali pada kasus ini diperkirakan diawali oleh residu selaput korion berupa jaringan fibrosa yang membentang cavum korionik dan menekan kranium sehingga migrasi membran neurokranium tergganggu dan berakhir dengan akalvaria. Ekstremitas yang berkembang sempurna namun cacat pada beberapa segmen terutama tempat perlekatan membran amnion dan kranium  sesuai letak kejadian cincin konstriksi mendukung teori Torpin.

Kata kunci: amniotic band syndrome; acrania

Exencephaly as One of Amniotic Band Syndrome Complication

Abstract

Amniotic Band Syndrome includes a spectrum of non-genetic anomalies, varying from simple digital band constriction to major craniofacial and visceral defects, and even fetal death. Anencephaly represents the most common neural tube defect. Sonographic as well as pathologic evidence points to a close link between exencephaly (also frequently referred to as “acrania”) and anencephaly. It has been proposed that the brain tissue of exencephalics may gradually degenerate due to the exposure to amniotic fluid in combination with mechanical trauma. ABS is an aetiological factor in exencephaly. Appropriate counselling for affected families needs to be given after prenatal diagnosis. Based on medical report Mrs RA 33 years old , G3P1A1 30-31 weeks of pregnancy, breech presentation, exencephaly, anemia that delivered her baby on Delivery Room Hasan Sadikin General Hospital at December 1,2016.
Amniotic membrane found  fused with fetal cranium and on its attachment found that the cranium was absent (acrania) In the case reported here, the amniotic membrane was well fused to the scalp, and skull bones were absent on the site of attachment of the amniotic membrane. This case supports Torpin’s hypothesis of early amnion rupture, with failure of the cranial bones to develop at the site of attachment of the amniotic band as “early amnion disruption sequence.”. The fibrous strands would entangle and entrapped the fetal head, resulting in faulty migration of the membranous neurocranium which leads to exencephaly on this case.

Keywords: Amniotic band syndrome; exencephaly


Keywords


amniotic band syndrome; acrania

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References


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DOI: http://dx.doi.org/10.24198/obgynia.v1n1.18

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