A case of amniotic band syndrome with extensive acrania

DOI
  • Suzuki Konosuke
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Toya Yukiko
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Matsumoto Atsushi
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Tsuchiya Shigekuni
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Takashimizu Nao
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Konishi Yu
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Sotodate Genichiro
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Tanifuji Sachiko
    Department of Pediatrics, School of Medicine, Iwate Medical University
  • Akasaka Manami
    Department of Pediatrics, School of Medicine, Iwate Medical University

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Other Title
  • 広範囲な無頭蓋症を伴う羊膜索症候群の長期生存例

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Abstract

<p> We report a case of a male infant born at 38 weeks and 0 days of gestation. The patient was referred to our hospital after suspicion of acrania by fetal ultrasonography at 25 weeks. The cranium could be identified only up to the frontal region, and the brain tissue was floating in the amniotic fluid, surrounded by membrane-like structures. The patient was born by cesarean section and was found to have acrania, encephalocele, phalangeal defect, and strangulated ring. Placental pathology showed extensive omental defects, and a diagnosis of anencephaly due to amniotic band syndrome was made. We administered medical treatment for convulsive seizures and infections associated with spinal fluid leakage from the head. The patient underwent encephalocele repair and ventriculoperitoneal shunting for encephalocele and ventricular enlargement, respectively, at the age of 1 year and 9 months. The patient is currently being managed at home. Prenatally diagnosed cases of widespread anencephaly often lead to pregnancy interruption and fetal death. The possibility of long-term survival is suggested for acrania without anencephaly and other major visceral abnormalities. Accumulation of further cases is warranted.</p>

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