Histiocytosis X of the head and neck in a pediatric population.

Despite an extensive literature, histiocytosis X is still a poorly understood disease and the origin remains unknown. To study the impact of recent therapeutic and diagnostic advances on this disease, 28 cases of histiocytosis X treated at Children's Hospital of Los Angeles, Calif, from 1976 through 1987 were reviewed. A classification system was developed that accurately described the clinical outcome in our patients. Type patients (n = 7) had monostotic disease, and type 2 patients (n = 15) had multiple tissue sites other than the viscera. The latter type was further divided into types 2A and 2B, depending on whether lesions were solely osseous (type 2A [n = 8]) or if soft-tissue sites were involved (type 2B [n = 7]). Type 3 patients (n = 6) had disseminated disease at the time of presentation, as defined by visceral involvement other than the liver. Type 1 patients were usually treated by surgical curettage alone and had an excellent prognosis. Type 2A patients required either surgery or radiation for symptomatic lesions. Type 2B patients were treated similarly. In addition, Type 2B patients received chemotherapy, a practice that is intensively debated in the literature. All the Type 2 patients survived with varying morbidity. Type 3 patients were all treated with chemotherapy but none of them survived. The majority of patients with histiocytosis X had otolaryngologic manifestations ranging from bony lesions to infectious complications. To conclude, the term histiocytosis X should be maintained to describe a spectrum of diseases. A simple classification system with three divisions gives reliable prognostic and therapeutic guidelines.

Bedside videolaryngoscopic assessment of intubation trauma.

Telescopic laryngoscopic examination was performed at the bedside after extubation in 29 consecutive surgical intensive care unit patients who required endotracheal intubation for more than 16 hours. The examinations were documented with a portable video recording system. The majority of patients exhibited evidence of acute endolaryngeal trauma. Vocal fold ulceration and vocal fold motion abnormalities were the most common lesions. Patients with abnormal larynges were followed up after discharge from the intensive care unit. Most of the identified injuries resolved without intervention. However, silent aspiration was identified frequently in patients with vocal fold paresis and was thought to be a significant factor in postoperative pulmonary complications. Early identification of significant laryngeal trauma and/or vocal fold paresis in critically ill patients is important for both postoperative pulmonary care and voice rehabilitation. This pilot study demonstrated that documentation of the laryngeal examination is feasible in critically ill patients in an intensive care unit setting.

Production of lymphokine-activated lymphocytes. Lysis of human head and neck squamous cell carcinoma cell lines.

Lymphokine-activated killer cells are thought to be important mediators of host tumor defense. In the present study, the cytotoxic potential of lymphokine-activated lymphocytes against different head and neck squamous cell carcinoma cell lines was investigated. Lymphokine-activated killer cells were derived from peripheral blood lymphocytes. Effector peripheral blood lymphocyte cell suspensions were incubated in the presence or absence of recombinant interleukin-2. Cytotoxicity of incubated cells or fresh peripheral blood lymphocytes was determined in a 3-hour chromium 51 release assay. Target cell lines included K562 (a natural killer-sensitive target) and the following head and neck squamous cell carcinoma cell lines: Cal 27, UMSCC-1, UMSCC-8, UMSCC-16, UMSCC-19, and UMSCC-22a. Fresh peripheral blood lymphocytes and peripheral blood lymphocytes cultured in the absence of added interleukin-2 demonstrated minimal cytotoxic effects against the squamous cell carcinoma targets. In contrast, these fresh and incubated lymphocytes showed significant cytotoxic effects against K562. Cells preincubated in the presence of interleukin-2 demonstrated a statistically significant increase in cytotoxic effects against K562 and all squamous cell carcinoma targets. These investigations support the possible role of lymphokine-activated killer cells in host defense against squamous cell carcinoma. In vitro natural killer cell activity against head and neck squamous cell carcinoma cell lines is low; however, significant lymphokine-activated killer cell cytotoxicity is present.

Parotitis and facial nerve dysfunction.

Paralysis of the facial nerve in association with suppurative parotitis is rare, with only ten previously reported cases. In some situations, inflammation surrounding a benign neoplasm accounted for the observed paralysis. In this article, three new cases of parotitis with associated facial nerve dysfunction are described, none of which was associated with a neoplasm. In one, an occult abscess was present and in another an aggressive necrotizing process was seen. The treatment of this disease should initially be conservative management with high doses of wide-spectrum antibiotics. In the majority of cases, resolution of the facial paralysis should follow. However, persistence of a parotid mass with continued facial palsy mandates surgical exploration to exclude the presence of an underlying neoplasm.

Granulocytic sarcomas of the head and neck.

Granulocytic sarcomas (chloromas) are rare tumors with a predilection for head and neck sites. In a review of six cases from two teaching institutions, chloromas were associated with the development of acute myelogenous leukemia or a relapse in five cases. Treatment modalities included chemotherapy and radiation therapy for problematic lesions. Our only survivor received combination therapy despite a negative bone marrow examination. Work-up should include a computed tomographic scan or magnetic resonance imaging to evaluate the extent of the tumor. Bone marrow examination and biopsy should be performed and specimens prepared for immunohistochemical studies or electron microscopy. Recommended treatment includes both aggressive chemotherapy and 2000 to 3000 cGy of external-beam radiotherapy to the tumor when possible.

An accurate method of Teflon injection using functional phonosurgery.

A technique for Teflon injection is described that allows the laryngologist to assess vocal fold vibration during general anesthesia. A tracheostomy tube fitted with a rostral air line allows translaryngeal airflow. During endoscopy with a bivalved laryngoscope, the cords are approximated manually. Vocal fold vibration is produced with the cords adducted. The precise site of defects in glottic closure is clearly seen and corrected with Teflon injection. In cases where standard Teflon injection has failed, utilization of this method has allowed substantial voice improvement. The ability to assess vibratory function of the vocal folds during direct suspension laryngoscopy enhances the precision of vocal fold augmentation techniques in difficult rehabilitation cases.

Osteomyelitis of the clavicle.

Osteomyelitis of the clavicle is a rare entity and can occur as a complication of head and neck surgery. Ten consecutive cases of the clavicular osteomyelitis were reviewed at the University of California Medical Center, Los Angeles, over the past seven years. Six cases were associated with prior surgical procedures, and five cases presented as chronic wound drainage. One case was related to a pharyngocutaneous fistula following a supraglottic laryngectomy. Four patients presented with acute symptoms resulting from hematogenous spread, and two of the four patients had Staphylococcus aureus on blood cultures. Long-term intravenous antibiotic therapy (six to eight weeks) was used to successfully treat cases of hematogenously spread osteomyelitis. Wide surgical débridement was the mainstay of treatment in the chronic conditions, with antibiotic therapy having a secondary role. Myocutaneous flaps were required in two patients who had had surgery and antecedent radiotherapy. To conclude, the surgeon should be aware that osteomyelitis of the clavicle can occur as a complication of head and neck procedures. In addition, the treatment of the chronic form of clavicular osteomyelitis is surgical débridement and possible flap reconstruction.

Nonsalivary sinonasal adenocarcinoma.

Thirteen cases of primary non-salivary gland adenocarcinoma of the nasal cavity and paranasal sinuses were studied at UCLA over 20 years. All pathologic specimens were reviewed and those tumors that were histologically distinct from the more common salivary gland-derived tumors were included in the study. Three classifications were identified: well, moderately, and poorly differentiated adenocarcinoma. A distinct variant of sinonasal adenocarcinoma was the intestinal type. The clinical behavior of the latter resembled the well or moderately differentiated types, with behavior mainly predicted by the extent of the disease. These groups have prognostic significance, with the poorly differentiated group having the most virulent course. Nine of 13 tumors occurred in the ethmoid sinuses and all were aggressive locally. Only one case had distant metastases (nodal neck disease in a terminal case). Of five long-term survivors (median five-year follow-up), all had extensive surgical resections and three had full-course radiotherapy. The single most important factor in the treatment of these lesions is adequacy of surgical margins. Four of six patients with confirmed negative margins were cured despite extensive tumors. Three survivors had the cribriform plate taken and one required a combined intracranial/extracranial approach for tumor resection. There were no survivors in four patients treated with primary irradiation.

Granular cell tumors of the head and neck.

Granular cell tumors have a predilection to occur in the head and neck. Thirty granular cell tumors have been diagnosed during the past 26 years at UCLA; 13 of which presented in the head and neck. Of these 13 cases, 10 were correctly diagnosed on the primary pathological specimen, which included one fine-needle aspirate and three frozen sections. Four cases required diagnostic confirmation with electron microscopy or histochemistry. The tongue accounted for more than one third of the cases. Three lesions exhibited locally aggressive behavior, but none of the tumors metastasized. Initial treatment was wide local excision in all but one case; recurrence was noted in two cases. Fine-needle aspiration can be used to provide preoperative diagnosis of granular cell tumors. Wide local excision with histologically confirmed clear margins provides definitive treatment.

A point mutation in the Nul gene of bacteriophage lambda facilitates phage growth in Escherichia coli with himA and gyrB mutations.

A mutant of lambda was isolated that grows in the Escherichia coli himA delta/gyrB-him320(Ts) double mutant at 42 degrees C; conditions which are non-permissive for wild-type lambda growth. The responsible mutation, ohm1, alters the 40th codon of the Nul reading frame. The Nul and A gene products comprise the terminase protein which cleaves concatameric DNA into unit-length phage genomes during DNA packaging. The Nul-ohm1 gene product acts in trans to support lambda growth in the double himA/gyrB mutant, and lambda cos154 growth in the single himA mutant. The observation that an alteration in Nul suppresses the inhibition of growth in the double himA/gyrB mutant implicates DNA gyrase, as well as integration host factor, in the DNA:protein interactions that occur at the initiation of packaging.

Separation of some enantiomeric di- and tripeptides on chiral stationary phases.

The chromatographic behavior of the N-3,5-dinitrobenzoyl derivatives of twelve dipeptide esters and two tripeptide esters was investigated on three different chiral stationary phases (CSPs). It is observed that the stereoisomers present in each sample may be cleanly separated on each chiral phase. A degree of regularity is noted in the elution order of the enantiomers and often of the diastereomers. Elution order of the enantiomers is related to a chiral recognition model for each CSP.

Aspiration and nasogastric intubation.

Routine use of nasogastric tubes in major surgery is associated with unwarranted risks of aspiration through at least three mechanisms: hypersalivation--allowing pooling of secretions in the hypopharynx, a depressed cough reflex, which is often associated with nasogastric intubation, and various laryngeal and pharyngeal abnormalities that are frequently caused by nasogastric tubes, leading to an inability to handle secretions and protect the airway. Using indirect cinelaryngoscopy, we can accurately document these mechanisms; three representative cases are presented here. Aspiration associated with nasogastric intubation is a newly described but potentially serious complication. Routine use of nasogastric intubation should be abandoned, except when the clinical situation warrants it.

Caprine beta-mannosidosis: clinical and pathological features.

Beta-mannosidosis, an inherited defect of glycoprotein catabolism associated with deficiency of tissue beta-mannosidase and accumulation of Man(beta 1-4)GlcNAc and Man(beta 1-4)GlcNAc(beta 1-4)GlcNAc, appeared in four of 13 offspring of a single pair of clinically normal, related Nubian goats. Neurological examinations revealed that all four affected goats were unable to rise or walk. All had facial dysmorphism, dome-shaped skulls, small palpebral fissures, carpal contractures, hyperextension of the pastern joints, proximal muscle atrophy, intermittent ocular oscillations resembling pendular nystagmus, marked intention tremor, and deafness. With intensive care, three affected kids were hand-reared and then killed at 1, 7, and 21 days of age. Macroscopically, there were paucity of myelin in the cerebral and cerebellar hemispheres and ventricular dilatation. Microscopically, the extent and distribution of cytoplasmic vacuolation, myelin paucity, axonal spheroids, and filamentous expansions were evaluated in the cerebrum, cerebellum, brainstem, spinal cord, and peripheral nerves of the four affected kids and two age-matched, clinically normal kids. Widespread cytoplasmic vacuolation correlated with the previously reported accumulation of oligosaccharides in the brain and kidney and the deficiency of tissue beta-mannosidase. beta-Mannosidosis, not yet identified in man or other species, is characterized by distinctive neonatal clinical, pathological and biochemical features which differentiate it from the alpha-mannosidosis and other inherited diseases of glycoprotein catabolism.