DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20180926

Iniencephaly: a rare birth defect

Alpana Singh, Vasudha Gupta, Amrita .

Abstract


Iniencephaly is form of neural tube defect which includes occipital bone defect at foramen magnum along with fixed retroflexion of fetal head and absence of fetal neck. Incidence of iniencephaly is 0.1-10 in 10,000 pregnancies. There is a known female predilection for this condition. Iniencephaly has a poor prognosis. It can be diagnosed antenatally by raised maternal serum alpha-fetoprotein and typical ultrasound features. Termination should be advised to patients who present before 20 weeks. Herein we are describing a case of iniencephaly who presented at 27 weeks of gestation with a brief review of literature.


Keywords


Anencephaly, Iniencephaly, Retroflexion

Full Text:

PDF

References


Alvis-Miranda HR, Bula-Anichiarico DA, Calderón-Miranda WG, Moscote-Salazar LR. Iniencephaly: Case Report J Pediatr Neurosci. 2015;10(2):181-4.

Sainit-Hilaire IG. Iniencephalus In: Bailliere JB, ed. History of the Anomalies of the Organization Paris; 1836:308-10.

Pungavkar SA, Sainani NI, Karnik AS, Mohanty PH, Lawande MA, Patkar DP, et al. Antenatal diagnosis of iniencephaly: sonographic and MR correlation: a case report. Korean J Radiol. 2007;8(4):351-5.

Joó JG, Beke A, Papp C, Szigeti Z, Csaba A, Papp Z. Major diagnostic and pathological features of iniencephaly based on twenty-four cases. Fetal Diagn Ther. 2008;24(1):1-6.

Kulkarni PR, Rao RV, Alur MB, Joshi SK. Iniencephaly clausus: a case report with review of literature. J Pediatr Neurosci. 2011;6(2):121-3.

Halder A, Pahi J, Pradhan M, Pandey A, Gujral R, Agarwal SS. Iniencephaly: a report of 19 cases. Indian Pediatr. 1998;35(9):891-6.

Lewis HL. Iniencephalus. Am J Obstet. 1897;35:11-53.

Hawkins J, Lawrie R. Iniencephalus. J Obstet Gynecol Br Emp. 1939;46:26-31.

Singh A, Mundhra R, Manchandra S, Radhakrishnan G, Meena P. Early diagnosis of iniencephaly: a case report with review of literature. Int J Reprod Contracept Obstet Gynecol. 2015;4:2047-9.

Eken MK, Ilhan G, Ertekin AA, Kaygusuz EI, Sahinoglu Z, Herkiloglu D, et al. Iniencephaly in early gestation and accompanying ultrasonographic findings. J Cases Obstet Gynecol. 2016;3(2):72-6.

Romero R, Jeanty P, Pilu G, Ghidini A, Hobbins JC. Iniencephaly, In: Prenatal diagnosis of congenital anomalies. Appleton and Lange, Norwalk, Conn, USA; 1988:65.