Iniencephaly and long-term survival: a rare case report.
BACKGROUND
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.


CONCLUSIONS
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.
Aytar
Murat Hamit
MH
Faculty of Medicine, Department of Neurosurgery, Gazi University, Ankara, Turkey. mhaytar@gazi.edu.tr
Doğulu
Fikret
F
Cemil
Berker
B
Ergün
Ertan
E
Kurt
Gökhan
G
Baykaner
Kemali
K
D000015:Abnormalities, Multiple / Q000175:diagnosis / Q000401:mortality; D002657:Child Development; D002675:Child, Preschool; D004677:Encephalocele / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D009436:Neural Tube Defects / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D011296:Prenatal Diagnosis; D012886:Skull / Q000002:abnormalities*; D017741:Survivors; D014463:Ultrasonography