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Neonatal Fat Necrosis
Joseph Junewick, MD FACR
over 4 years ago
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Case History


5 month old with craniofacial dysmorphism.


Case Detail

Anatomy: Brain-Spine
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: CT
Activities:
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Case Images


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Reveal Findings

CT – 3D volume rendered reformats show right frontozygomatic suture synostosis with ipsilateral frontal flattening and orbital deformity.

Reveal Discussion

Premature closure of the frontozygomatic suture represents a rare type of frontoorbital dysostosis. Findings previously reported in the only known publication describing this anomaly by Currarino, which highlighted two case reports, depicted ipsilteral small orbit with a short and broad lateral wall without a frontozygomatic suture, a normal orientation of the sphenoid bone, and contralateral elliptic conformation of the orbit with mild elevation of the lesser wing of the sphenoid. Furthermore, there was marked flattening of the ipsilateral frontal bone. The cribiform plate was also noted to be slightly obliqued upward and to the right, mimicking a coronal synostosis on the opposite side.

Unilateral coronal synostosis results in ipsilateral frontal flattening, orbital enlargement in the coronal plane with upward slanting of the roof of the orbit and the lesser wing of the sphenoid bone, and decreased height of the lateral orbital wall. By contrast, premature closure of the frontozygomatic suture will have normal coronal sutures and the features described above.

The mechanism behind the anatomic changes is unclear but perhaps is the result of alteration of fibrous organization of the dura. This entity may also be related to coronal synostosis and caused by dysgenesis of the frontoorbital region.

Reveal Diagnosis

Right Frontozygomatic Synostosis

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