Parry Romberg Syndrome: Literature Review and Report of Three Cases

Abstract

Parry Romberg Syndrome or Progressive Hemifacial Atrophy is a rare disease usually affecting one side of face with loss of soft and hard tissues. The disease appears suddenly and is usually self-limiting in 2-10 years time. The loss of soft and hard tissue leads to aesthetic and functional deficits which are compounded by the presence of associated symptoms like neuralgia, migraine, epilepsy and ocular involvement. The degree of deformity depends on the age at which the disease manifests first; the younger the age, the more severe the deformity. These patients undergo severe psychological trauma and social problems. The exact etiology is not known, and treatment is largely cosmetic. A report of three cases and a literature review is presented.

Keywords: Dermal fat graft; Parry Romberg Syndrome; Porous polyethylene implant; Progressive Hemifacial Atrophy; Temporalis myofascial flap.

Fig. 1

A case of Parry Romberg Syndrome showing mild loss of tissues in lower half of face on the left side

Fig. 2

Harvesting of dermal graft a marking of the defect, b harvesting the dermal fat graft, c insetting the graft

Fig. 3

Postoperative photograph 2 years post-op

Fig. 4

Preoperative photograph of case 2 showing extensive involvement of lower and middle third of left side of face

Fig. 5

Intraoral photograph showing hemiatrophy of tongue

Fig. 6

Restoration of contour of angle of mandible with temporalis myofascial flap and placement of porous polyethylene implants in the temporal hollow

Fig. 7

Augmentation of lower lip and cheek with dermal fat

Fig. 8

a Porous polyethylene implant to augment malar area, b collagen filler for upper lip and cheek

Fig. 9

Augmentation of mandible with porous polyethylene implants

Fig. 10

Postoperative photograph of case 2 showing the progressive changes. a Preoperative, b after angle augmentation with temporalis flap, c after lower lip augmentation with dermal fat, d after upper lip augmentation with collagen and porous polyethylene implant, e before fat injection upper lip, f after porous polyethylene implant on mandible

Fig. 11

Preoperative photograph of case 3 showing the severe involvement of hard and soft tissues of left side of face

Fig. 12

Photograph showing the sudden loss of tissues of left side of face in 1 year