|Year : 1973 | Volume
| Issue : 3 | Page : 136-139
Progressive facial hemiatrophy (a case report)
I.P.S Parmar, Prem Chandra, Jugindar Kumar, RC Nagpal
Department of Ophthalmology, Medical College, Rohtak, Haryana, India
Department of Ophthalmology, Medical College, Rohtak, Haryana
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Parmar I, Chandra P, Kumar J, Nagpal R C. Progressive facial hemiatrophy (a case report). Indian J Ophthalmol 1973;21:136-9
|How to cite this URL:|
Parmar I, Chandra P, Kumar J, Nagpal R C. Progressive facial hemiatrophy (a case report). Indian J Ophthalmol [serial online] 1973 [cited 2020 Aug 14];21:136-9. Available from: http://www.ijo.in/text.asp?1973/21/3/136/31393
| Introduction|| |
Hemiatrophy of the face is an uncommon deformity of unknown etiology. Two main types have been described; a congenital facial hemiatrophy and a progressive facial hemiatrophy (WALSH, 1949). The progressive facial hemiatrophy commences usually in the second decade and is seen more commonly in females than in males. The skin becomes thinned because of atrophy of the papillary layer and disappearance of the subcutaneous fat. Subsequently the underlying cartilages, muscles and bones are affected.  The maxillary, malar and palatine bones on the affected side are usually smaller and flatter. The mandible and the mastoid processes may be severely affected.  The involvement of external ear is quite frequent. PURVES-STEWART  reported that the tongue, when protruded, comes out straight unlike that of a patient with atrophy from hypoglossal palsy. DUKE-ELDERS stated that the patient may get speech difficulties as the tongue on the affected side may be underdeveloped. Atrophy of the tongue on the same side has also been reported (SHARP)  The hair on the affected side may fall out or may become white. ,
Sometimes the process involves other parts of the body. Shoulder girdle of the same side or even whole of one side of body may be affected. ,
VOLHARD  reported pigmentation of the opposite side of the body and kyphoscoliosis was reported by SHARP. 
Ocular involvement in these cases is not infrequent. Homer's syndrome is a relatively common accompaniment. Enophthalmos may sometimes form a striking feature. ARCHAMBAULTI and WLASH  observed an apparent upward displacement of the eye. Other ocular changes reported are: muscle palsies,  monocular nystagmus  infrequent blinking  downward displacement of outer canthus  enophthalmos, blephrophimosis and colobomata.  STREIF reported that pupil on the same side may be dilated and nonreactive to light and also reported narrowing of the pupil which failed to dilate with cocaine and adrenaline.
| Case Report|| |
A.S. 23 years male attended the Ophthalmic out-patient department in February, 1973 with the complaints of itching and watering in both eyes for about two months. He was aware of the presence of white patches over the skin of the right half of the face including forehead, both lids, cheek, lips, chin and side of neck, for the last 17 years. About 15 years ago he noticed that his hair on the right side of the head, right eye brow and cilia started becoming grey. For the last eight years the right half of the face started becoming flatter and the right half of the chin started receding. There was no history of such deformity in the family.
General Examination:- It revealed a marked asymmetry of the face, the right half being flatter as compared to the left. The malar prominence was less marked on the affected side. The chin on the right side had receded backwards and the mandible was hypoplastic [Figure - 1]. The skin of the right half of the face and neck showed areas of hyper pigmentation and depigmentation. This change stopped abruptly at the midline. The subcutaneous fat was much less in the affected area. The hair on the right side of the head were grey in large areas, while they were black on the left side [Figure - 2].
Examination of ear, nose and throat revealed that the right pinna was smaller especially the outer border was much thinner as compared to the left. The ears were otherwise normal. He had hypertrophic rhinitis and the hair in the right nostril were white while those in the left were black. The tongue on the right half was atrophic and furrows were increased and on protrusion it deviated to the right side. Dental examination showed that the space between upper central incisors was increased and the centric line of occlusion was shifted to the right.
Examination of the body showed that the right shoulder was sagging down and the muscle mass around the shoulder girdle was less prominent and less firm as compared to the opposite side. The right thenar eminence showed flattening. The muscle power of the upper limb was normal. There was no abnormality of lower limbs. Over an extensive area involving the left half of thorax there was a big naevus, the upper border of which coincided with a horizontal line drawn posteriorly from the nipple. It was more dense in the upper part and patchy in the lower and posterior portion [Figure - 3]. The hair in the area of naevus were more promnient than the corresponding area in the right side.
The right eye brow showed white hair in the medial 1.5 cm. The middle portion was black, while the hair in the lateral third were white. On the right upper lid there were two patches of vitiligo measuring 2 cm x 1.5 cm and 1.5 cm x 1.5 cm. The cilia in the upper as well as lower lid showed poliosis and madarosis. The lid margins showed squamous blepharitis. Slight enophthalmos was present. The anterior segment, fundus and vision were normal.
Apart from squamous blepharitis in both the lid margins, the left eye was normal.
| Investigations|| |
Routine blood and urine investigations were normal. The V.D.R.L. was negative. Radiological examination showed that the right mandible was smaller than the left. The X-ray of thoracic spine showed scoliosis and no abnormality was seen in the shoulder joints.
| Discussion|| |
PURVES-STEWART reported that there may be white hair on the scalp on the affected side. In the case presented by us the hair on the right side showed greying. The distribution of white hair to the right half was characteristically demonstrated by the presence of white hair in the right nostril and black in the left.
Pigmentation of the skin has been reported frequently. In our case there were areas of hyperpigmentation and depigmentation on the face and neck on the right side only. The absence of the subcutaneous fat in the affected area agrees well with the views Of PURVES-STEWART.  DUKE ELDER  stated that the involvement of the external ear is quite frequent. Our case also showed a smaller pinna on the right side. The tongue showed increased furrowing and atrophy on the right side. When protruded out, the tip showed deviation to the right side. This finding was contrary to the one reported by PURVES-STEWART  who observed that on protrusion, the tongue didn't show any deviation.
There was pronounced atrophy of right mandible with relatively less affected maxilla. This finding is in agreement with that of DUKE ELDER.
Shoulder on the right side was at a lower level than the left with the muscle mass less prominent and more flabby as compared to the left side. This has been reported by OPPENHEIM  and FINESILVER AND Rosow.  The atrophy was even involving the muscles of the hand as the thenar eminence on the right side was less prominent. All the same the muscle power of the right upper limb was not affected substantiating the views Of PURVES-STEWART.
VOLHARD reported the presence of pigmentation on the opposite side of the body. In the case reported here a large naevus was present on the left half of thorax.
The ocular examination revealed the presence of vitiligo, poliosis and madarosis. This is perhaps the first time that these ocular changes have been reported in association with progressive facial hemiatrophy.
| Summary|| |
A case of progressive facial hemiatrophy has been reported. It was associated with poliosis and vitiligo which have not been reported previously.
| References|| |
Archambault, L. and Fromm, N. K.: Arch. Neurol. and Psychiat., 27: 529, 1932.
Chasanow, M.: Ziet. f.d. ges. Neurol. U. Psychiat. 140: 473; 1932. (Quoted by Walsh, F. B.).
Duke-Eider, S.: The system of ophthalmology, London, 1964, Vol. III Part 2, p. 1026, Henry Kuupton.
Finesilver, B. and Rosow, H. M.: Jour. Amer. Med. Assoc. 110: 366, 1938.
Langelaam, J. W.: Rev. Neurol. 21, pt. 2: 520, 1913, (Quoted by Walsh, F. B.)
Oppenheim, H.: Lehrbuch der Nervenkrankheiten, 6. Aufl., Berlin, S. Karger, p. 1972, 1913, (Quoted by Walsh, F. B.)
Pick, W.: Arch. f. Dermat. V. Syph., 167: 543, 1933, (Quoted by Walsh, F. B.)
Purves-Stewart, J.: The diagnosis of nervous diseases. Ed. 5, New York, E. B. Trent & Co., 1920, p. 142.
Salomon: Neurol. zbl. 26: 846, 1907, (Quoted by Duke-Elder, S.)
Sharp, C.: Brit. J. Plastic Surgery, 24: 93, 1971.
Shumay, E. A.: Arch. Ophth. 13: 8, 1933.
Streif, S.: Zeit. f. d. ges. Neurol. U. Psychiat 147: 573, 1933, (Quoted by Walsh, F. B.)
Volhard: Quoted by Oppenheim. Cited by Walsh, F. B.
Walsh, F. B.: Amer. J. Ophth. 22: 1, 1939.
[Figure - 1], [Figure - 2], [Figure - 3]