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Iniencephaly: Case Report.

Alvis-Miranda HR, Bula-Anichiarico DA, Calderón-Miranda WG, Moscote-Salazar LR - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: The iniencephaly involves a variable defect in the occipital bone, resulting in a large foramen magnum, partial or total absence of the cervical and thoracic vertebrae, accompanied by incomplete closure of arcs and/or vertebral bodies, significant shortening of the spinal column and hyperextension of the malformed cervicothoracic spine; the individual's face is deviated upward, the mandibular skin is directly continuous with anterior thorax due to the absence of neck.Its incidence is about 1:1000-1:2000 births, so this is a pretty rare neural tube defect.We present a case of iniencephaly in association with cardiovascular, spinal cord, and intracranial malformations that ended demonstrating the low survival of patients affected with this condition.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, University of Cartagena, Colombia.

ABSTRACT
The iniencephaly involves a variable defect in the occipital bone, resulting in a large foramen magnum, partial or total absence of the cervical and thoracic vertebrae, accompanied by incomplete closure of arcs and/or vertebral bodies, significant shortening of the spinal column and hyperextension of the malformed cervicothoracic spine; the individual's face is deviated upward, the mandibular skin is directly continuous with anterior thorax due to the absence of neck. Its incidence is about 1:1000-1:2000 births, so this is a pretty rare neural tube defect. We present a case of iniencephaly in association with cardiovascular, spinal cord, and intracranial malformations that ended demonstrating the low survival of patients affected with this condition.

No MeSH data available.


Related in: MedlinePlus

Portable X-ray image showing: Cervical vertebras in block, thoracic vertebras with irregular fusion resulting in severe scoliosis with hyperextension of the malformed cervicothoracic spine. Cardiothymic silhouette is visible with dextrocardia
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Figure 2: Portable X-ray image showing: Cervical vertebras in block, thoracic vertebras with irregular fusion resulting in severe scoliosis with hyperextension of the malformed cervicothoracic spine. Cardiothymic silhouette is visible with dextrocardia

Mentions: Portable X-ray evidenced the physical deformity of the costal arch and spine, with severe scoliosis, lung volume of eight intercostal spaces; few reticulonodular infiltrates with pulmonary acceptable transparency. Cardio-thymic silhouette is visible with dextrocardia. The abdomen shows gas in the gastric camera and intestinal loops without edema or pneumatosis [Figure 2].


Iniencephaly: Case Report.

Alvis-Miranda HR, Bula-Anichiarico DA, Calderón-Miranda WG, Moscote-Salazar LR - J Pediatr Neurosci (2015 Apr-Jun)

Portable X-ray image showing: Cervical vertebras in block, thoracic vertebras with irregular fusion resulting in severe scoliosis with hyperextension of the malformed cervicothoracic spine. Cardiothymic silhouette is visible with dextrocardia
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489071&req=5

Figure 2: Portable X-ray image showing: Cervical vertebras in block, thoracic vertebras with irregular fusion resulting in severe scoliosis with hyperextension of the malformed cervicothoracic spine. Cardiothymic silhouette is visible with dextrocardia
Mentions: Portable X-ray evidenced the physical deformity of the costal arch and spine, with severe scoliosis, lung volume of eight intercostal spaces; few reticulonodular infiltrates with pulmonary acceptable transparency. Cardio-thymic silhouette is visible with dextrocardia. The abdomen shows gas in the gastric camera and intestinal loops without edema or pneumatosis [Figure 2].

Bottom Line: The iniencephaly involves a variable defect in the occipital bone, resulting in a large foramen magnum, partial or total absence of the cervical and thoracic vertebrae, accompanied by incomplete closure of arcs and/or vertebral bodies, significant shortening of the spinal column and hyperextension of the malformed cervicothoracic spine; the individual's face is deviated upward, the mandibular skin is directly continuous with anterior thorax due to the absence of neck.Its incidence is about 1:1000-1:2000 births, so this is a pretty rare neural tube defect.We present a case of iniencephaly in association with cardiovascular, spinal cord, and intracranial malformations that ended demonstrating the low survival of patients affected with this condition.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurosurgery, University of Cartagena, Colombia.

ABSTRACT
The iniencephaly involves a variable defect in the occipital bone, resulting in a large foramen magnum, partial or total absence of the cervical and thoracic vertebrae, accompanied by incomplete closure of arcs and/or vertebral bodies, significant shortening of the spinal column and hyperextension of the malformed cervicothoracic spine; the individual's face is deviated upward, the mandibular skin is directly continuous with anterior thorax due to the absence of neck. Its incidence is about 1:1000-1:2000 births, so this is a pretty rare neural tube defect. We present a case of iniencephaly in association with cardiovascular, spinal cord, and intracranial malformations that ended demonstrating the low survival of patients affected with this condition.

No MeSH data available.


Related in: MedlinePlus