Synchronous complex Chiari malformation and cleft palate—a case-based review
Autor: | Johnny Carlton, Jacques Lara-Reyna, Whitney E. Parker, Jeffrey P. Greenfield |
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Rok vydání: | 2018 |
Předmět: |
Male
medicine.medical_specialty Adolescent Craniofacial abnormality First year of life Occipitocervical fusion 03 medical and health sciences 0302 clinical medicine medicine Humans Abnormalities Multiple Chiari malformation business.industry Infant 030206 dentistry General Medicine medicine.disease Arnold-Chiari Malformation Surgery Cleft Palate Skull medicine.anatomical_structure Pediatrics Perinatology and Child Health Neurology (clinical) Neurosurgery Suboccipital decompression Presentation (obstetrics) business 030217 neurology & neurosurgery |
Zdroj: | Child's Nervous System. 34:2353-2359 |
ISSN: | 1433-0350 0256-7040 |
Popis: | The association between mid-facial clefts and Chiari malformation in the medical literature has been restricted to patients with syndromic craniofacial abnormalities. A common shared developmental pathway including causative factors for facial clefts and “complex” Chiari malformations, both midline skull base pathologies, seems logical but has not been reported. The coincident presentation of these findings in a single patient, and our subsequent discovery of other patients harboring these mutual findings prompted further investigation. We describe the case of a patient born with a cleft palate which was repaired during his first year of life, subsequently presenting as a teenager to our hospital with a severe and symptomatic complex Chiari malformation. We discuss his treatment strategy, suboccipital decompression with occipitocervical fusion and endoscopic anterior decompression surgeries, as well as his favorable radiological and clinical outcome, demonstrated at long-interval follow-up. Furthermore, we review his two pathologies, cleft palate and Chiari malformation, and posit a common embryological linkage. The embryologic interaction between the paraxial mesoderm and ectoderm may explain the co-occurrence of cleft palate and complex Chiari malformation in a single patient. Complete radiological, clinical, and genetic evaluation and counseling is advised in this situation and raises the question of whether the presence of a cleft palate independently increases the risk for other skull base developmental abnormalities. |
Databáze: | OpenAIRE |
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