STEW001. Stewart, Charles R., Stephen G. Kahler, and James M. Gilchrist. “Congenital myopathy with cleft palate and increased susceptibility to malignant hyperthermia: King Syndrome?” Pediatric neurology 4.6 (November-December 1988): 371-374.

5 photographs, 1 table

Publication type: Journal article

The authors present case reports on six Lumbee children, ages 8 months to 13 years (three of them related), five of normal intelligence and one mildly retarded. All had features of King Syndrome, which is defined as “multiple congenital facial and skeletal deformities along with a slowly progressive myopathy and susceptibility to malignant hyperthermia” (p. 371). These patients, in particular, had “congenital ptosis, generalized weakness, hypotonia at birth, cleft palate, short stature, kyphoscoliosis, and normal baseline serum creatine kinase (CK) levels” (p. 373). All six were born with cleft palate, which rarely occurs as part of King Syndrome. The authors conclude that “to our knowledge, this syndrome has not been described [before] in persons of one ethnic group living in the same region” (p. 374). They caution that “clinicians should be aware of the MH [malignant hyperthermia] risk in this group of patients because of the likelihood of surgery with general anesthesia for facial or skeletal deformities” (p. 374). Malignant hyperthermia is frequently triggered when susceptible patients are exposed to halothane or succinylcholine.

Additional Subjects: King Syndrome | Malignant hyperthermia

This annotation was written on: May 27, 2003; edited on June 21, 2003.

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