Masseter muscle rigidity and malignant hyperthermia susceptibility in pediatric patients. An update on management and diagnosis.

Background: Controversy exists regarding the definition of masseter muscle rigidity (MMR) and anesthetic management after MMR. This study reports current anesthetic management after MMR, estimates the incidence of clinical malignant hyperthermia (MH) in patients with MMR, and is the first to evaluate the coincidence of MMR with malignant hyperthermia susceptibility (MHS) according to the 1987 North American Malignant Hyperthermia Group protocol.

Methods: Practicing anesthesiologists referred pediatric patients for biopsy between 1986 and 1991 based on evidence of MMR after succinylcholine (1975-1991).

Evidence for genetic heterogeneity in malignant hyperthermia susceptibility.

Malignant hyperthermia susceptibility (MHS) is a clinically heterogeneous pharmacogenetic disorder characterized by accelerated metabolism, hyperthermia, and frequently muscle rigidity. MHS is elicited by all commonly used potent inhalation anesthetics and depolarizing neuromuscular blockers and remains an important cause of death due to anesthesia. Recent linkage studies suggest a single genetic locus for this disorder on chromosome 19q13.

Cleft palate lateral synechia syndrome. Review of the literature and case report.

Cleft palate lateral synechiae syndrome is a rare syndrome consisting of cord-like interalveolar adhesions and concomitant hypoplasia of the mandible. We have presented an infant with CPLS syndrome and coincident hydrocephalus. Surgical division of the adhesions is necessary to allow normal feeding, avoid upper airway obstruction, and allow normal mandibular function and growth.