Congenital midline Abnormalities: About two cases
Soumeya N Fedala1
Mahdi A Haddam2
1 Service endocrinologie CHU Bab el oued;
2 Service endocrinologie EPH Bologhine Ibn Ziri;
3 Service biochimie Ain naadja hospital
International Journal of Clinical Case Reports, 2013, Vol. 3, No. 9 doi: 10.5376/ijccr.2013.03.0009
Received: 03 Aug., 2013 Accepted: 05 Aug., 2013 Published: 05 Aug., 2013
© 2013 BioPublisher Publishing Platform
This is an open access article published under the terms of the Creative Commons Attribution License
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Several observations have reported a high frequency (12à50%) of the association between pituitary insufficiency and midline anomalies. The latter are clinical markers that need to systematically search for endocrine disorders. They are hypothalamic origin and are accompanied by abnormalities of the pituitary ontogenesis in 30% of cases. Neuroendocrine disorders and abnormalities of the median line are attached to a embryological defect bud naso frontal primitive that allows the development of different regions of the brain and face. We report two children who had abnormal facial midline. Neuroendocrine and neuroradiological exploration are not made until the appearance of delay stature in one case. Hormonal assessment was in favor of GH deficiency in the first while in the other case the endocrinology consultation was motivated by the existence of a micropenis and cryptorchidism related to hypogonadotropic hypogonadism. There was in both cases a pituitary hypoplasia with other intracranial malformations in one of them. The evolution was marked by the appearance of other hormonal deficiencies.
Midline abnormalities; Delay stature-failure pituitary-MRI-prenatal ultrasound
International Journal of Clinical Case Reports
• Volume 3