Facial cleft with intracranial lipoma

Case contributed by Dr Shailaja Muniraj

Presentation

Seizures. Delayed speech and milestones. Low intelligence quotient.

Patient Data

Age: 4 years
Gender: Female

There is an ill defined bony defect seen in the bifrontal region. Prortusion of meninges seen through this defect, suggestive of meningocele. Adjacent skin tenting noted.

There is a well defined linear hypodense lesion noted in the frontal region, parasaggitaly. This lesion shows a thick eccentric calcific focus. Features suggest lipoma

Few fatty droplets seen in the mid/posterior falx, likely to suggest fatty falx.

No features of chemical meningitis.

The posterior aspect of corpus callosum is not distinctly visualized, suspicious for dysgenesis.

CSF containing space seen between the frontal horns of lateral ventricle, suggestive of cavum septum pelucidum.

There is soft tissue defect noted in the bilateral outer curve of the nostril / ala. No underlying bony defects.

The bilateral frontal bony defects with skin tenting is much appreciated here.

Teisser facial cleft classification

Diagram

Tessier classification system of cranio-facial clefts is currently the most widely accepted classification.

The numbering system helps in identifying the anatomic pathways of soft tissue and skeletal / bony clefts.

This is a self concept line diagram for the basic understanding of anatomic routes, based on tessier classification.

The individual detailed description is beyond the scope this case. 

Special thanks: Dr H K Anand.

Case Discussion

This case demonstrates the following:

1. Bilateral frontal bone defect with meningocele.

2. Intracranial lipoma.

3. Suspicious callosal dysgenesis.

4. Bilateral nostril soft tissue clefts - Teisser facial cleft #2.

 

 

 

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