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Septo-optic Dysplasia Complex with Omphalocele, Pre-maxillary Agenesis and Encephalocele

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Abstract

Septo-optic dysplasia complex (SODC) is a rare congenital anomaly, characterized with absence of septum pellucidum, optic nerve hypoplasia with or without pituitary dysfunction. Various midline defects are also associated with SODC in addition to its classical findings. We report, for the first time a rare presentation of SODC which is associated with pre-maxillary agenesis along with sphenoidal encephalocele and omphalocele. The case showed resulting median cleft lip and palate along with other multiple congenital defects including hydrocele, inguinal hernia, micropenis and a wide mediastinum.

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Authors and Affiliations

  1. Maxillofacial Surgery Department, KFMMC, Dhahran, KSA

    Abdul Majeed Kavarodi, Khalid Zharani, El-Sayed Ali & Hussain Sharahili

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  1. Abdul Majeed Kavarodi
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  2. Khalid Zharani
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  3. El-Sayed Ali
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  4. Hussain Sharahili
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Correspondence to Abdul Majeed Kavarodi.

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Kavarodi, A.M., Zharani, K., Ali, ES. et al. Septo-optic Dysplasia Complex with Omphalocele, Pre-maxillary Agenesis and Encephalocele. J. Maxillofac. Oral Surg. 14 (Suppl 1), 457–461 (2015). https://doi.org/10.1007/s12663-014-0675-z

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  • DOI: https://doi.org/10.1007/s12663-014-0675-z

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