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At a glance

Inherited polymalformative syndrome characterized by craniofacial anomalies with gingival fibromatosis and dystrophic fingers and fingernails. The clinical characteristics are gingival fibromatosis that is present at birth or in the days following it, severe hypoplasia of the distal phalanges of the hands and feet, dysplasia of the nail, joint hypermobility, and hepatosplenomegaly. Other features include a nose and pinnae usually large and poorly developed. It is often associated with mental retardation.

Synonyms

Zimmermann-Laband Syndrome; Zimmermann-Laband Syndrome (ZLS); Laband Zimmermann Syndrome; Laband’s Syndrome; Gingival Fibromatosis Nail Dysplasia Abnormal Fingers Syndrome.

History

This medical condition was first described in 1928 by a German anatomist and histologist named Karl W. Zimmerman (1861-1935). It was subsequently revised by Dr Carl Jacob Witkop in 1971.

Incidence

The exact incident remains unknown. Thirty cases have been reported in the literature. The sexual distribution is the same among males and females. It most frequently affects individuals of Eastern Indian ancestry originally from India and the West Indies. However, this medical condition has also been reported in Europe.

Genetic inheritance

It is presumed inherited as an autosomal dominant pattern.

Pathophysiology

Findings are suggestive of a storage disorder but no biochemical defect has been identified.

Diagnosis

Clinical features first observed in infancy, with evolution during childhood. The radiologic evidence of hypoplastic terminal phalanges of the fingers and toes, with the most postaxial digits are markedly affected. The terminal phalanges of the hands may have narrow shafts and are hypoplastic. There may also be narrowing of the distal interphalangeal joint spaces in both hands and feet, with the narrowing being more pronounced in the feet.

Clinical aspects

Coarse facial appearance, gingival fibromatosis. The clinical presentation is variable with intellectual deficit, hepatosplenomegaly, hirsutism, small joint hyperextensibility, and “dystrophic” fingernails and toenails. A possible association with a proximal aortic dilatation and cardiomyopathy has been suggested.

Precautions before anesthesia

Echocardiography may be required to assess cardiac function. Laboratory investigation should include coagulation profile, liver function, and renal excretion.

Anesthetic considerations

As determined by cardiac function. The presence of enlarged gingival fibromatosis may have an impact on the airway management and laryngoscopy and may make tracheal intubation difficult. Furthermore, it is important to recognize that in case of airway emergency, the insertion of a laryngeal mask may also be render difficult. Therefore, it is highly recommended to maintain at all times during the airway management spontaneous ventilation until the tracheal has been secured and ventilation of the lungs is confirmed.

Pharmacological implications

No known pharmacological implications.

Other conditions to be considered

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