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.
This adolescent girl with Parry-Romberg syndrome presents with a severe
form of left-sided hemifacial atrophy involving the skin, subcutaneous
tissue, and the osseous structures. This results in left-sided enophthalmos
(not shown) and profound atrophy of the skin and the left side of the
Facial Hemiatrophy; Progressive Hemifacial Atrophy;
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
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
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.
Lewkonia RM, Lowry RB: Progressive hemifacial atrophy (Parry-Romberg
syndrome): Report with review of genetics and nosology. Am ...