PubMed · 7818717
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H Becker. 1994. The computer and truth.. https://pubmed.ncbi.nlm.nih.gov/7818717/
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OBJECTIVES: To inform otolaryngologists about upper airway obstruction requiring tracheotomy and other otolaryngological manifestations of malignant infantile osteopetrosis (MIOP) and to discuss pathophysiological features, management, and new treatment strategies in MIOP. DESIGN: Ongoing case series combined with a retrospective chart review. SETTING: International tertiary pediatric hospital. INTERVENTIONS: Patients with MIOP were initially referred for treatment and routine follow-up. Tracheotomy was performed to manage obstructive sleep apnea. Audiograms were also performed at regular intervals. RESULTS: The records of 9 patients were examined. The otolaryngological findings of hearing loss, obstructive sleep apnea (sometimes requiring tracheotomy), otitis media, and chronic osteomyelitis with facial fistulas were identified. CONCLUSIONS: Osteopetrosis is a rare condition caused by a failure of the osteoclast to resorb bone. This results in thickened dense, deformed, and easily fractured bone. As a result, growth failure, anemia, hypoplastic dentition, chronic infections, facial fistulas, blindness, hearing loss, nasal congestion, and upper airway obstruction may occur. The management of otolaryngological problems in a child with osteopetrosis is an important component in comprehensive care. To our knowledge, this study represents the largest case series of MIOP in the otolaryngology literature.
Craniofacial dyskinesias encompass a variety of abnormal spontaneous craniofacial movements that often appear similar in morphology but are, in fact, of varied cause and nature. Although hemifacial spasm and blepharospasm are the two most common abnormal craniofacial movements, the clinician should be cognizant of other dyskinesias, particularly craniofacial dystonias, tremor, tic, chorea, and stereotypies. Most craniofacial dyskinesias respond favorably to injections of botulinum toxin type A or oral medications. Surgical treatment may be beneficial for refractory cases.