A disorder characterized by dystrophic skin
(deep circumferential creases), mental retardation,
hypertrichosis, and, occasionally, congenital heart defects. Other features
include cleft palate and neuroblastoma.
Michelin Tire Baby Syndrome; Multiple Benign Ring-Shaped
Skin Creases of Limbs.
Approximately 25 cases have been reported in the literature.
Presumed autosomal dominant inheritance with
variable penetrance and expression.
Unknown. Explanations for deep circumferential
skin creases include diffuse underlying lipomatous nevus, underlying smooth
muscle hamartoma, and a disorder of “elastic fiber” formation.
Primarily clinical. Biopsy to show the presence of a
smooth muscle hamartoma has been suggested. Histologic examination to
determine the presence of elastic fiber abnormalities has been proposed,
whereas electronic microscopy may show decreased elastin deposition.
Primary features include deep circumferential
skin creases on the forearms and sometimes neck and lower limbs. Gyrus-like
creases may be seen on the back. The skin is loose and may seem thick. The creases
resolve spontaneously during childhood, although remnants may be visible in
adults. Numerous other congenital abnormalities have occurred in association
with this disorder: cleft palate, congenital heart disease, localized
neuroblastoma, craniofacial anomalies (micrognathia), inguinal and umbilical
hernias, and psychomotor retardation.
The infant or young child presenting
with deep skin creases should be investigated for associated anomalies. A full
history and physical examination must be done to assess for cleft palate,
congenital heart disease, and craniofacial abnormalities. Developmental
delay may be present. Investigations should include hemoglobin level,
electrocardiogram, chest radiograph, and echocardiogram if necessary. Workup
for neuroblastoma would include urine and plasma catecholamine levels and
imaging studies. Uncommonly, hypertension is present, which should be well
controlled prior to anesthesia.
Potential for difficult airway
management must be anticipated because of micrognathia and/or cleft palate.
Difficult placement of intravenous, intraarterial, and central venous
catheters is most probable because of deep folds of loose, thick skin.
Considerations for congenital heart disease include knowledge of the anatomy
and pathophysiology of the lesion and strict avoidance of air embolism.
Fluctuations in blood pressure caused by release of catecholamines and/or
blood loss can occur during manipulation of a neuroblastoma tumor. Careful
padding for intraoperative positioning is required.
No specific pharmacological
implications. In the presence of congenital heart defect, anesthetic drug
implications are dictated by the cardiovascular pathophysiology. Subacute bacterial
prophylaxis must be considered in presence of a congenital heart defect.
Kunze J, Riehm H: A new genetic disorder: Autosomal-dominant multiple
benign ring-shaped skin creases. Eur J Pediatr
Sardana K, Mendiratta V, Kakar N, et al: Spontaneously improving Michelin tire baby
syndrome. Pediatr Dermatol
Sato M, Ishikawa O, Miyachi Y, et al: Michelin tyre syndrome: A congenital
disorder of elastic fibre formation? Br J Dermatol