Three-dimensional imaging of progressive facial hemiatrophy (Parry-Romberg syndrome) with unusual conjunctival findings

Abstract

Progressive hemifacial atrophy, also known as Parry-Romberg syndrome, is an uncommon degenerative condition which is poorly defined. It is characterized by a slow and progressive atrophy affecting one side of the face. The onset usually occurs during the first two decades of life. Characteristically, the atrophy progresses slowly for several years, and then it becomes stable. Ophthalmic involvement is common, with progressive enophthalmos which is a frequent finding. Cutaneous pigmentation is common in such conditions, however its extension to the conjunctiva is rarely reported. We report a case of Parry Romberg syndrome with characteristic clinical and radiographic presentation accompanied with rare ocular findings. The clinical features, radiological findings, and differential diagnoses to be considered, and the available treatment options are discussed in this report.

Keywords: Conjunctiva; Facial Hemiatrophy; Parry-Romberg Syndrome; Pigmentation.

Fig. 1

Clinical photograph of the patient shows marked hypoplasia of the left malar area with diffuse blackish pigmentation of the same region.

Fig. 2

Photographs compare the normal and abnormal sides.

Fig. 3

Photograph shows enophthalmos and flattening of zygomatic complex.

Fig. 4

Photograph shows the pigmentation of the left palpebral conjunctiva.

Fig. 5

Panoramic radiograph shows shorter crowns and roots of teeth on the left side compared with the right side.

Fig. 6

Axial and coronal CT images reveal the hypoplastic left zygomatic complex and maxillary sinus.

Fig. 7

Axial CT image shows the left eye ball at a lower level when compared to right side. Also observe the hypoplastic lateral orbital wall. Also coronal CT image reveals the shrunken left eyeball, hypoplastic maxillary sinus and lateral orbital wall.

Fig. 8

Coronal and axial CT images show the minimal condylar changes on the right side compared with the left side.

Fig. 9

3D CT images show the normal anatomical features on the right side (A), deviated nasal septum and tilt in the occlusal table in the frontal view (B), hypoplastic zygomatic complex and orbital wall on left side (C).