Sternocleidomastoid muscle fibrosis has been recognized for centuries, but its pathogenesis and treatment remains controversial. Pseudotumor of infancy is a firm fibrous mass in the sternocleidomastoid muscle appearing at 2 to 3 weeks of age. Congenital muscular torticollis is less common and appears later in life. Pseudotumor and congenital muscular torticollis probably represent different manifestations of sternocleidomastoid muscle fibrosis. Pseudotumor will usually resolve with conservative therapy; however, some patients will subsequently develop torticollis. Congenital muscular torticollis usually requires surgical release of the sternocleidomastoid muscle to achieve a good cosmetic result and to prevent plagiocephaly, facial asymmetry, and scoliosis. This report provides guidelines for the management of congenital muscular torticollis and pseudotumor of infancy based on the authors' experience and review of the medical literature. Representative case histories from the neonate through the adult are presented, and the pathogenesis, diagnosis, treatment, and prognosis are discussed.

A retrospective review of 45 children with mucopolysaccharidoses was performed to determine the frequency of complications related to the head and neck. In this series, every patient had at least one complication involving the head and neck region, and in over half, operative intervention by the otolaryngologist was required. Upper airway obstruction occurred in 17 (38%) and necessitated a tracheostomy in 7 (16%). Cervical spine instability occurred in 8 (18%), making airway management difficult. Recurrent respiratory infections occurred in 17 (38%), and chronic recurrent middle ear effusions were noted in 33 (73%). This review demonstrates that children afflicted with the mucopolysaccharidoses frequently have otolaryngologic-related complications that are common throughout their life span and often the primary management issue in their continuing care. The otolaryngologic management of these patients is outlined based on the results of this study and review of the relevant literature.

Acute torticollis is commonly seen in the pediatric emergency department. It often results from an inflammatory process that irritates the cervical muscles, nerves, or vertebrae. Posturing of the head occurs with unilateral spasm of the sternocleidomastoid muscle such that the child will position the head with the occiput rotated to the affected side and the chin rotated to the contralateral side. We recently treated 26 children who presented to the emergency department with acute nontraumatic torticollis. The most common causes were upper respiratory infection, sinusitis, otomastoiditis, cervical adenitis, and retropharyngeal abscess or cellulitis. Four patients had subluxation of the atlantoaxial joint as a result of the inflammatory process. Children with acute torticollis need careful evaluation for either overt or occult otolaryngologic infections. Computed tomography and magnetic resonance imaging are helpful in determining the cause of the acute torticollis and in ruling out rotatory subluxation of the atlantoaxial joint.

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Plummer-Vinson syndrome (PVS) is characterized by iron deficiency anemia, upper esophageal stricture, cervical dysphagia, and glossitis. The precise role of iron deficiency in PVS has yet to be defined and remains a subject of much debate. A 29-year-old woman with PVS is presented. The patient had a 4-year history of severe iron deficiency anemia, a 2-year history of progressive dysphagia and weight loss, and a greater than 90% benign upper esophageal stricture. Iron therapy alone resolved her dysphagia and anemia, and a follow-up esophagram 1 year later showed a residual stenosis of less than 30%. The development of severe iron deficiency anemia in this patient 2 years before the onset of dysphagia, as well as the response of the stricture to iron repletion, supports the theory that iron deficiency can cause dysphagia and upper esophageal strictures. The occurrence of glossitis, gastritis, and esophagitis in iron deficiency demonstrates the adverse effects of iron depletion on the rapidly proliferating cells of the upper alimentary tract.