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Progressive hemifacial atrophy (Parry-Romberg's disease), principally involving bone

Published online by Cambridge University Press:  29 June 2007

Yochanan Goldhammer ,
Jona Kronenberg ,
Rina Tadmor ,
Jackson Braham  and
George Leventon
Yochanan Goldhammer
Affiliation:
Department of Neurology, The Chaim Sheba Medical Center, Tel-Hashomer, and theTel-Aviv University Sackler School of Medicine, Israel.
Jona Kronenberg
Affiliation:
Department of Otolaryngology, The Chaim Sheba Medical Center, Tel-Hashomer, and theTel-Aniv University Sackler School of Medicine, Israel. ENT Department, The Chaim Sheba Medical Center, Tel-Hashomer, Israel.
Rina Tadmor
Affiliation:
Department of Radiology, The Chaim Sheba Medical Center, Tel-Hashomer, and theTel-Aniv University Sackler School of Medicine, Israel.
Jackson Braham
Affiliation:
Department of Neurology, The Chaim Sheba Medical Center, Tel-Hashomer, and theTel-Aviv University Sackler School of Medicine, Israel.
George Leventon
Affiliation:
Department of Otolaryngology, The Chaim Sheba Medical Center, Tel-Hashomer, and theTel-Aniv University Sackler School of Medicine, Israel.
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Abstract

A case of progressive hemifacial atrophy (PHA) with extensive atrophy of the walls of the maxillary sinus is reported. Prolapse of subcutaneous tissue and orbital fat through the bony defect simulated the presence of a tumour and led to surgical exploration. Such a severe degree of bony involvement with relatively mild soft tissue atrophy is exceptional. Radiological evaluation by hypocycloidal tomography contributed greatly to the diagnosis in this case.

Unilateral progressive atrophy of the face was first described by Parry (1825) and by Romberg (1846). Eulenberg (1871) coined the name ‘progressive facial hemiatrophy’, but ‘progressive hemifacial atrophy’ (PHA) is a more appropriate term (Gorlin and Pindborg, 1964; Kabir and Halprin, 1964). The disease commences usually in the first or second decade of life, and is more common in females. Over several years there is a slowly progressive atrophy of the face on one side, with involvement of the skin and subcutaneous fat, and on rare occasions also of the muscles and bones (Gorlin and Pindborg, 1964; Finesilver and Rosow, 1938; Bramley and Forbes, 1960; Singh and Bajpai, 1969; Crikelair et al., 1962). The etiology of the disease is unknown.

We report an unusual case in which the hemifacial atrophy affected the bone much more than the soft tissues and which presented as a mass in the maxillary sinus.

Type
Clinical records
Information
The Journal of Laryngology & Otology , Volume 95 , Issue 6 , June 1981 , pp. 643 - 647
Copyright
Copyright © JLO (1984) Limited 1981

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References

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