Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + At a Glance Download Section PDF Listen ++ Inherited polymalformative syndrome characterized by craniofacial anomalies with gingival fibromatosis, dystrophic fingers and fingernails. + Synonym Download Section PDF Listen ++ Zimmermann-Laband Syndrome. + Incidence Download Section PDF Listen ++ Unknown; 29 cases reported in the literature up until 2004. + Genetic Inheritance Download Section PDF Listen ++ Autosomal dominant. + Pathophysiology Download Section PDF Listen ++ Findings are suggestive of a storage disorder, but no biochemical defect has been identified. + Diagnosis Download Section PDF Listen ++ Clinical features first observed in infancy, with evolution during childhood. Radiologic evidence of hypoplastic terminal phalanges of the toes, with the most postaxial digits most 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 Download Section PDF Listen ++ Coarse facial appearance, gingival fibromatosis. Variable intellectual deficit, hepatosplenomegaly, hirsutism, small joint hyperextensibility, and “dystrophic” fingernails and toenails. Association with proximal aortic dilatation and cardiomyopathy. + Precautions before Anesthesia Download Section PDF Listen ++ Echocardiography may be required to assess cardiac function. Laboratory investigation should include coagulation profile, liver function, and renal excretion. + Anesthetic Considerations Download Section PDF Listen ++ As determined by cardiac function. Gingival fibromatosis may make tracheal intubation or insertion of a laryngeal mask difficult. + Pharmacological Implications Download Section PDF Listen ++ No known pharmacological implications. + Other Condition to Be Considered Download Section PDF Listen ++ Ramon Syndrome: Gingival fibromatosis associated with maxillary fibrous dysplasia, seizures, mental retardation, rheumatoid arthritis, and hypertrichosis. An autosomal recessive transmission has been suggested. + References Download Section PDF Listen ++Katz J, Guelmann M, Barak S: Hereditary gingival fibromatosis with distinct dental, skeletal and developmental abnormalities. Pediatr Dent 24:253, 2002. [PubMed: 12064501] ++Robertson SP, Lipp H, Bankier A: Zimmermann-Laband syndrome in an adult. Long-term follow-up of a patient with vascular and cardiac complications. Am J Med Genet 78:160, 1988.