Iniencephaly and long-term survival: a rare case report |
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Authors: | Murat Hamit Aytar Fikret Do?ulu Berker Cemil Ertan Ergün Gökhan Kurt Kemali Baykaner |
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Institution: | (1) Faculty of Medicine, Department of Neurosurgery, Gazi University, Ankara, Turkey;(2) Faculty of Medicine, Department of Neurosurgery, Division of Pediatric Neurosurgery, Gazi University, Ankara, Turkey;(3) Gazi üniversitesi Tıp Fakültesi, Beyin ve Sinir Cerrahisi Anabilim Dalı, Ankara, Turkey |
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Abstract: | Introduction Iniencephaly, a neural tube defect involving occiput and inion and combined with rachischisis of the cervical, thoracic spine,
and retroflexion position of the head is a very rare congenital abnormality of the fetus–newborn with a 0.1–10 of 10,000 prevalence.
This abnormality’s prognosis is thought to be dismal. This abnormality can be associated with other abnormalities such as
anencephaly, encephalocele, hydrocephalus, cyclopia, absence of the mandible, cleft lip and palate, cardiovascular disorders,
diaphragmatic hernia, renal abnormalities, overgrowth of the arms compared to the legs, and club food and gastrointestinal
atresia.
Discussion Most of the patients are dead born, and the others die in a few hours. There are only six previously documented long-term
survivors. In our case, our patient with iniencephalic signs and findings is still living. She is 2 years old now. We think
that this patient presents a mild form of iniencephaly. |
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Keywords: | Iniencephaly Congenital malformations Neural tube defect Survival Rachischisis Hydrocephaly |
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