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Crouzon Syndrome

Crouzon Syndrome

Case History: A 3-month-old child brought by parents with complaints of abnormal growth of head size. As the patient’s appearance and head size was not normal, a detailed family and medical history was taken. Review of medical history was unremarkable. The mother reported normal labor and delivery. There were no anomalies in any siblings or near relatives. On general examination, the patient was had a wide nasal bridge, hypertelorism, and exophthalmos. Intra oral examination revealed high arch palate, hypo plastic maxilla causing prognathism of the mandible. The patient was subjected to radiographic investigations.

Lateral skull view demonstrated a scaphocephaly skull, retruded maxilla with a relatively large mandible and copper beaten appearance (Figure 1). Radiographic examination of metacarpal bones and fingers were unremarkable. Further, on non contrast CT, brain and face was performed, which revealed increased antero-posterior diameter of the skull with obliterated sagittal suture (scaphocephaly skull), small paranasal sinuses (Figure 2). Three-dimensional (3D) computed tomographic images of the skull revealed increased circumference of the skull in antero-posterior diameter with hypo plastic maxilla (Figure 3). On non contrast MRI and CT, brain did not reveal any abnormality in brain parenchyma. No evidence of hydrocephalous was noted (Figure 4).

Figure 1. Lateral skull projection reveals abnormally enlarged skull (more antero-posterior diameter) due to premature closure of sagittal suture (scaphocephaly) mandibular prognathism (white arrow), maxillary hypoplasia (arrow), copper beaten appearance and enlarged hypophyseal cavity.

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