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Biomedical subjects

J K Bredenkamp

Publications and source records attributed to J K Bredenkamp.

9 recordsLinked to original sources

Otolaryngologic manifestations of the mucopolysaccharidoses.

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.

Adolescent↗

Congenital muscular torticollis. A spectrum of disease.

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.

Adult↗

Inflammatory torticollis in children.

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.

Atlanto-Axial Joint↗

Importance of iron repletion in the management of Plummer-Vinson syndrome.

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.

Adult↗

Maxillary ameloblastoma. A potentially lethal neoplasm.

Ameloblastomas are benign tumors derived from the odontogenic apparatus. Of these tumors, 80% originate in the mandible, while 20% originate in the maxilla. Six cases of maxillary ameloblastoma treated at the UCLA hospitals are presented; four of these cases showed extensive and destructive tumor growth involving vital structures, including the orbit, base of skull, and parasellar structures. Two of four patients with extensive disease died of their tumors, one, with extensive involvement of the base of the skull, became unavailable for follow-up, and, one year after diagnosis, one is alive with middle cranial fossa disease. A review of the medical literature provides further evidence of the locally aggressive behavior and potentially lethal nature of this tumor. No effective treatment has evolved for extensive ameloblastomas of the maxilla that have invaded surrounding vital structures. When tumor-free surgical margins are not possible, radiation therapy may offer palliation of disease.

Adolescent↗

Two-stage temporalis flap reconstruction for facial paralysis.

Treatment of the paralyzed face remains a challenging surgical problem. When facial nerve damage is irreparable or facial nerve grafting has failed, static and dynamic techniques must be considered. A two-staged modification of the dynamic muscle transfer using ipsilateral temporalis muscle is described. Initially, a free temporalis fascia graft, harvested from the contralateral scalp, is placed around the oral commissure of the paralyzed side of the face through an incision in the nasolabial crease. Several weeks later, an ipsilateral temporalis muscle and fascia transfer is made to the anterior face and attached to the previously placed fascia graft. Oral commissure grafting, as a first step, provides for a secure anchoring point for the temporalis flap, and achieves a more satisfactory correction of the oral commissure.

Facial Paralysis↗

Lymphokine-activated killing of autologous and allogeneic short-term cultured head and neck squamous carcinomas.

Interleukin-2-(IL-2)-activated lymphocytes have been shown to kill a variety of continuously cultured allogeneic (nonself), natural killer cell-sensitive and resistant cell lines, and some autologous (self) tumor cells. Lymphokine-activated killer (LAK) cytotoxicity of autologous head and neck squamous cell carcinoma (HNSCC) cells has not been previously demonstrated, and efforts to demonstrate this have been hampered by the lack of a reliable and reproducible method of obtaining satisfactory tumor targets. In this study, fresh tumor cells were enzymatically dissociated, enriched by adherence to plastic, and used in a 3-hour chromium-51 cytotoxicity assay. Peripheral blood lymphocytes (PBLs) were incubated for 3 days with or without added IL-2. IL-2-activated PBLs showed significant cytotoxicity against autologous and allogeneic targets, while only low levels of tumor lysis occurred with unstimulated PBLs. These findings suggest the possible use of IL-2-activated lymphocytes in the adoptive immunotherapy of HNSCC patients.

Carcinoma, Squamous Cell↗

Tonsillectomy under local anesthesia: a safe and effective alternative.

Tonsillectomy using local anesthesia (local tonsillectomy) is a safe and effective alternative to general anesthesia in the healthy cooperative teenage or adult patient. This retrospective analysis involved 64 local tonsillectomies performed over the past 7 years in a minor operating room using only local anesthesia with intravenous sedation. Operations were performed by residents in training as well as by experienced head and neck surgeons. Blood loss, morbidity, complications, and patient satisfaction were reviewed and compared with tonsillectomies done under general anesthesia. The average blood loss was 42 mL in the local tonsillectomy group with no cases of postoperative hemorrhage, compared with 198 mL in the general anesthesia group with two cases of postoperative hemorrhage. There was one major complication related to postoperative antibiotic use in the local anesthesia group, and follow-up interviews revealed that patients were satisfied with the procedure and would recommend and choose local anesthesia again. We conclude that local tonsillectomies have high patient acceptance and are associated with minimal morbidity and complications. Furthermore, they are cost-effective.

Adolescent↗