Parry-Romberg Syndrome

Progressive facial hemiatrophy characterized by unilateral atrophy of the skin including the subcutaneous tissue and underlying bone or cartilage. In contrast to “sclérodermie en coup de sabre,” this form more often affects the lower half of the face (but is not limited to it) with cutaneous sclerosis and possible involvement of tongue, developing teeth, lips, and salivary glands. Seizures and trigeminal-like pain have been reported.

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Facial Hemiatrophy; Progressive Hemifacial Atrophy; Romberg Syndrome.

Very rare.

It was described in the last century (1825) by Parry and Henoch and subsequently in 1846 by Romberg.

Most cases are sporadic; mendelian inheritance with autosomal dominance has been evocated.

Unknown; autoimmunity is evocated.

Characterized by progressive atrophy of some or all tissues on one side of the face, occasionally extending to other parts of the body.

Onset occurs most often before puberty or during adolescence. There is no sexual predominance. The process may be bilateral in 5 to 10% of cases, with preferential left-sided involvement. In most affected individuals, hemifacial atrophy typically progresses over approximately 3 to 5 years and then ceases. All tissues of the face can be involved, including skin (circumscribed cicatricial alopecia, poliosis, increased patchy skin pigmentation), tongue, lip, gingiva, soft palate, cartilage of the nose, ear, subcutaneous fat, larynx, muscle (facial muscle atrophy), eye (enophthalmos, lagophthalmos, ptosis, blepharoptosis, blepharophimosis, loss of periorbital fat, mixed coloring of iris), and bone (basilar kyphosis short body and ramus of mandible). Delayed teeth eruption on ipsilateral side, which leads to malocclusion is frequent. Neurological signs are associated: Horner syndrome, trigeminal neuralgia, ataxia, migraine, and seizures (contralateral Jacksonian epilepsy).

Evaluate neurological function (clinical, history, CT scan, EEG); vision (clinical); airway (clinical, radiographs).

Direct laryngoscopy and tracheal intubation may be difficult because of tongue atrophy and mandibular anomalies. Tracheal intubation may also be difficult because of laryngeal muscles atrophy. Extubation should probably be overseen for the same reasons. Ventilation with facial mask can be difficult because of hemifacial atrophy. Spontaneous respiration must be maintained until the trachea has been secured and lung ventilation confirmed. A laryngeal mask airway should be immediately available. Ocular protection is imperative.

Consider interaction between antiepileptic agent and anesthetic drugs.

CREST Syndrome: An autoimmune connective tissue disorder associated with anticentromere antibodies. A form of scleroderma associated with esophageal dysmotility.