DOI: http://dx.doi.org/10.18203/2320-1770.ijrcog20181370

Prenatal diagnosis of a rare case of iniencephaly apertus

Mahy Mohsen, Ahmed El Sheikhah, Lamees Mohamed, Sief El-Eslam Ali, Armia Michael, Ahmad A. Radwan, Shymaa S. Ali, Ahmed M. Abbas

Abstract


Iniencephaly is a rare neural tube defect that was firstly reported by Saint-Hilaire in 1836. Its incidence ranges from 0.1 to 10 in 10,000 deliveries with higher incidence in females. The most common features present iniencephaly are bifida at the cervical region, defect in the occipital bone and retroflexion of the head on the cervical spine. Here we report a case of a 24 years old second gravida with a history of consanguinity. She presented with a malformed fetus at 22 weeks gestation diagnosed by detailed ultrasonographic anatomy scan as iniencephaly apparatus. Termination of pregnancy was performed vaginally through medical induction by prostaglandins.


Keywords


Congenital malformations, Iniencephaly, Neural tube defect, Prenatal

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References


Hilaire IG. Iniencephalus. In: Bailliere J, ed. History of the Anomalies of the Organization, Paris, 1836;2:308-10.

Hemal U, Solanki RS, Varsheney A, Baliga S. Prenatal diagnosis of iniencephaly on ultrasound. Indian J Radiol Imaging. 2004;14(3):265-6.

Romero R, Pilu G, Jeanty P, Ghidini A, Hobbins J. Iniencephaly, in Prenatal Diagnosis of Congenital Anomalies, 3rd ed. Appleton & Lange, Norwalk, Conn, USA,1988.65

Erdinçler P, Kaynar MY, Canbaz B, Koçer N, Kuday C, Ciplak N. Iniencephaly: Neuroradiological and surgical features. Case report and review of the literature. J Neurosurg. 1998;89(2):317-20..

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.

Tugrul S, Uludoğan M, Pekin O, Uslu H, Celik C, Ersan F. Iniencephaly: Prenatal diagnosis with postmortem findings. J Obstet Gynaecol Res. 2007; 33(4):566-9.

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

Bhambhani V, George S. Association of Clomiphene with Iniencephaly. Indian Pediatr. 2004; 41(5):517.

Rasmussen SA, Chu SY, Kim SY, Schmid CH, Lau J. Maternal obesity and risk of neural tube defects: A metaanalysis. Am J Obstet Gynecol. 2008; 198(6):611-9.

Mórocz I, Szeifert GT, Molnár P, Tóth Z, Csécsei K, Papp Z. Prenatal diagnosis and pathoanatomy of iniencephaly. Clin Genet. 1986;30(2):81-6.

Nyberg DA, McGahan JP, Pretorius DH, Pilu G. Diagnostic imaging of fetal anomalies.1st ed. Lippincott Williams & Wilkins. Philadelphia: 2003:315-316

Woodward PJ,‎ Kennedy A,‎ Sohaey R,‎ Byrne J,‎ Oh K,‎ Puchalsk M. Diagnostic Imaging: Obstetrics. 1st ed.: AMIR SYS Inc, Salt Lake City 2005:10-4.

Sahid S, Sepulveda W, Dezerega V, Gutierrez J, Rodriguez L, Corral E. Iniencephaly: prenatal diagnosis and management. Prenat Diagn. 2000; 20(3):202-5.

Balci S, Aypar E, Altinok G, Boduroğlu K, Beksaç MS. Prenatal diagnosis in three cases of iniencephaly with unusual postmortem findings. Prenat Diagn. 2001;21(7):558-62.

Marton T, Tanko A, Mezei G, Papp Z. Diagnosis of an unusual form of iniencephaly in the first trimester of pregnancy. Ultrasound Obstet Gynecol. 2001; 18(5):549-51.

NINDS Iniencephaly Information Page. National Institute of Neurological Disorders and Stroke (NINDS). NINDS, 13 Feb. 2007.

Aytar MH, Doğulu F, Cemil B, Ergün E, Kurt G, Baykaner K. Iniencephaly and long-term survival: A rare case report. Childs Nerv Syst. 2007; 23(6):719-21.