Biomedical subjects
Publications and source records attributed to D B Kearns.
Congenital anomalies of the tracheobronchial tree must be included in the differential diagnosis of children with symptoms of respiratory distress. Tracheal agenesis is a rare major congenital anomaly that has been uniformly fatal. Diagnosis requires a high index of suspicion utilizing roentgenographic and endoscopic examinations.
Cancer is the second leading cause of death in children and, among cancers, lymphoma is the third most frequently diagnosed type. The majority of these are non-Hodgkin's lymphomas (NHL). Although NHL have been grouped in a variety of confusing ways, recent research in cytogenetics and immunology, coupled with a better understanding of the normal pathways of lymphoid differentiation, has greatly lessened this confusion. Burkitt's lymphoma (BL) is a type of B-cell NHL which is sub-classified as either endemic (African) or non-endemic (American). Kinetic studies have demonstrated a doubling time of only 24 h. As a result of this extremely rapid growth, symptomatic complaints occur early in the disease course. Structures contiguous to the primary become compressed and metabolic derangements occur. Signs of chronic debilitation such as cachexia, diarrhea, generalized lymphadenopathy, and weight loss usually are not found. Thirteen cases of American BL have been treated since 1980 at the Texas Children's Hospital and form the basis of this review. Ten patients had obstructive abdominal symptoms including constipation and pain, and 3 had masses in the neck, maxilla, or tonsil. Systemic manifestations included ascites, jaundice, fever, and hepatosplenomegaly. Two patients ultimately developed central facial nerve paralysis.
Tuberculous osteomyelitis of the temporal bone is a rare and dangerous entity that should be included in the differential diagnoses of infectious processes of the base of skull. A 21-year-old man presented with petrous apicitis, extradural and retromandibular abscesses, and paresis of the facial nerve. Immediate middle fossa craniotomy and drainage of the extradural abscess, in combination with a mastoidectomy, incision and drainage of the facial abscess, and antimicrobial therapy for gram-positive cocci, failed to check the destructive nature of the infection. The patient subsequently developed labyrinthitis, sensorineural hearing loss, and meningitis. Intraoperative biopsy specimens confirmed the presence of tuberculoid granulomas, and the infectious process responded to triple-drug therapy. Tuberculosis of the temporal bone should not always be considered an indolent infection. The management of tuberculous infection of the temporal bone is outlined.
Juvenile xanthogranulomas (JXGs) are rare, benign, fibrohistiocytic lesions. They usually appear as one or more cutaneous papules on the head, neck, or trunk in infants. Twelve cases of oral JXGs have been reported, four of which involved the tongue. We present a 6-year-old girl with a large tongue mass diagnosed as JXG after an excisional biopsy. Histological and immunohistochemical staining results are presented. This is the first reported case of a giant oral JXG. A review of the literature on these unusual lesions is presented, along with discussion of their differential diagnosis and key aspects of the patient's evaluation, management, and pathological diagnosis.
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