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Revista ADM Órgano Oficial de la Asociación Dental Mexicana

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Órgano Oficial de la Asociación Dental Mexicana
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2013, Number 2

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Rev ADM 2013; 70 (2)

Protrusion of sphenoethmoidal encephalocele through the cleft palate of a patient with median cleft face syndrome: a review of the literature and a case report

Takahashi ACT, Montoya CM, Solano GAC, Franco GLE, Hernández NM
Full text How to cite this article

Language: Spanish
References: 9
Page: 103-107
PDF size: 435.25 Kb.


Key words:

Encephalocele, median cleft face syndrome, median cleft, Tessier anomalies.

ABSTRACT

The incidence of median cleft face syndrome is low worldwide, occurring in only 1 in 1,000,000 births. Though the clinical picture varies, the condition appears as a 0–14 Tessier cleft, with incomplete closure of skeletal and/or facial soft tissue along the midline. Herniation of intracranial content through these fissures is common due to agenesis of the anterior arch, making protrusion and descent toward the oral cavity possible. As the condition is uncommon, it has received little consideration, therefore we have decided to draw attention to it and review the rather limited amount of literature dealing with it that exists. We report the case of a 9-day-old male patient in whom a pulsatile brain herniation—initially mistaken for a premaxilla—, was detected through the patient’s cleft palate. Diagnosis and examination were carried out by the Maxillofacial Surgery and Neurosurgery service at Ciudad Juárez Specialist Children’s Hospital.


REFERENCES

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  2. Celebiler O, Sönmez A, Erdim M, Ozek M, and Numanoğlu A. Atypical midline cleft with duplication of the metopic suture. J Craniomaxillofac Surg. 2007 Mar; 35 (2):81-83. doi:10.1016/j.jcms.200710.005

  3. Chen, C.-P. Syndromes, disorders and maternal risk factors associated with neural tube defects (VII). Taiwan J Obstet Gynecol 2008; 47 (3): 276-282. doi:10.1016/S1028-4559(08)60124-2

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  5. Ghareeb FM, Hanafy AM. Surgical planning and correction of median craniofacial cleft. Surgery 2003; 27(1): 143-152.

  6. Gupta H, Gupta P. Median cleft face syndrome. Indian Pediatrics 2004; 41 (1): 90.

  7. Hassani E, Karimi H, Hassani A. Inferior encephalocele: transpalatal repair using paired costal bone grafts with a 14-year follow-up. J Pediatr Surg 2011; 46 (10): 9-13. doi:10.1016/j.jpedsurg.2011.06.026

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Rev ADM. 2013;70