Ocular involvement is common, and the most frequent manifestation is enophthalmy, due to fat loss around the orbit. The extension of the atrophy is frequently limited to one side of the face, and the ipsilateral involvement of body is rare. Although, it is uncommon and generally unilateral, 5% to 10% of cases were described as being bilateral.
There is involvement of the skin and subcutaneous fat, and on rare occasions also of the muscles and bones. Alterations concerning involvement, duration and deformity can stabilize in any stage of growth and development. The disease manifests in the first or second decade of life with a slow progression over many years showing atrophy and then becomes stable. The term progressive hemi facial atrophy (PHA) is more widely accepted. Eulenberg coined the term 'progressive facial hemi atrophy' in 1871.
Unilateral progressive atrophy of the face was first described by Parry in 1825 and Romberg in 1846. Patient is under regular follow-up for 1 year and has not shown any recurrence. At the end of treatment, there were no symptoms of neuralgia, though facial hemiatrophy persisted. This treatment was continued for 1 month and then gradually tapered and stopped. After just 2 days he showed remarkable improvement. Carbamezipine 200 mg three times a day was initiated. Interestingly, he had minimal response to analgesics, steroids, and propranolol, but showed immediate response to carbamazepine. Supportive and conservative management showed satisfactory response. In view of the persistent headache, a neurologist's opinion was sought and concluded that the subdural hygroma, which was very minimal, was not the cause for the headache. Nerve conduction study showed features suggestive of trigeminal neuralgia of left side. Computerized tomogram brain (CT) showed Left minimal sub dural hygroma with no midline shift, and no evidence of cerebral edema or cerebral atrophy (Figure 4). X-ray chest revealed no abnormalities and X-ray skull showed both sides equal (Figure 3).
Diagnostic radiological imaging were done in which ultrasound abdomen (USG abdomen) showed no organomegaly or free fluid. Routine blood and urine investigations showed within normal limits (Table 2). The measurements showed hemi facial atrophy of the left side. Local examination, measurement were taken from the nasion to the tragus, nasion to angle of mandible, and mid chin to tragus of both right and left side (Table. All other systems were found to be normal. Systemic examination of the central nervous system (CNS) was normal. 5 years ago the same patient appeared normal with no obvious facial abnormality (Figure 2). Further, there was no other evidence of Horner's syndrome, facial palsy, or hemi facial spasm. Examination of the face revealed no sensory or motor deficits on both sides. Left side of the face showed signs of hemi atrophy with minimal drooping of left eyelid (Figure 1). On physical examination, he was conscious, oriented, afebrile, general condition was fair and vitals stable. Patient was moderately built and nourished with a body mass index (BMI) of 21 kg/m2. Patient denies history of smoking or alcohol intake. Patient gave past history of head injury 10 years ago to the left skull for which he took no treatment and no imaging studies were done. He had no history of fever, convulsions, loss of consciousness or ear, nose, throat bleed. Hence this patient indeed had Parry Romberg syndrome: Hemi facial atrophy with trigeminal neuralgia.Ī 30-year-old South Indian man presented with complaints of left sided headache and left sided facial pain, which was intermittent, sharp stabbing quality for past 3 months, severe for past 10 days.
MRI of the skull base was also normal and showed no evidence of trigeminal nerve compression. Nerve conduction study showed features suggestive of trigeminal neuralgia. Computerized tomogram of the brain showed left minimal sub dural hygroma with no midline shift, and no evidence of cerebral edema or cerebral atrophy.
X-ray chest revealed no abnormalities and x-ray skull showed both sides equal. Routine blood and urine investigations results were within normal limits. Left side of the face showed signs of hemi atrophy with minimal drooping of left eyelid. On physical examination, right side of the face appeared normal. We present a 30-year-old South Indian man who presented with complaints of left sided headache and facial pain for past 3 months, severe for past 10 days.