Fluoroscopic and CT fistulography of the first branchial cleft.

We present an unusual case of a complete first branchial cleft fistula communicating between the external auditory canal and the skin near the angle of the mandible. CT and fluoroscopic fistulography were used to establish the presence and course of the tract and to assist in surgical planning. The embryology and classification of first branchial cleft anomalies are discussed, with emphasis on the impact of imaging.

Unbalanced 4;6 translocation and progressive renal disease.

Two sibs are described with an unbalanced 4;6 translocation resulting in partial trisomy 6p and monosomy for distal 4p. Growth retardation, psychomotor retardation, and characteristic facial appearance are present. The facial anomalies include high prominent forehead, blepharoptosis, blepharophimosis, high nasal bridge, bulbous nose, long philtrum, small mouth with thin lips, and low-set ears. Both children have small kidneys and have had proteinuria since early childhood. The older boy developed progressive renal disease including hypertension and renal failure necessitating renal transplantation at age 18 years. Renal biopsy of the younger girl also indicates significant renal involvement. Progressive renal disease is likely an important part of the trisomy 6p phenotype.

Hydroxyapatite cement to repair skull base defects: radiologic appearance.

PURPOSE: To describe the radiologic appearance of hydroxyapatite cement (HAC), which, when mixed with liquid, forms a paste that can be contoured to osseous defects and, over time, becomes "osseointegrated" (native bone grows into the pores of the HAC and forms a strong chemical bond with the substance). METHODS: Between March 1992 and June 1993, 24 adults (16 men, eight women) underwent skull base surgery that included reconstruction or closure with HAC. Fourteen patients had HAC placed in the paranasal sinuses or facial bones, and 10 had HAC placed in the mastoid cavity. RESULTS: HAC is homogeneously radiopaque on CT scans and plain radiographs. Large amounts (ablating a frontal sinus or mastoid air cells) are readily seen; small amounts are inconspicuous. On MR images, HAC is a signal void. Infected HAC in one patient was surrounded be enhancing soft tissue on MR images, separated from native bone by an irregular radiolucent cleft on CT scans. CONCLUSIONS: HAC is a valuable addition to the surgical armamentarium for the repair of skull base defects. More experience will determine the time course for normal osseointegration, as well as the typical appearance of infection.

Imaging of ossicular prostheses.

The prostheses available for reconstruction of the ossicular chain have expanded to include a variety of synthetic materials. Hydroxyapatite contains the inorganic constituents found in human living bone and is currently being incorporated into many new prostheses. This study demonstrates the computed tomography and magnetic resonance image characteristics of eight middle ear prostheses, a block of dense hydroxyapatite, and a human incus. Imaging of ossicular prostheses is more informative with computed tomography.

Odontoma of the middle ear cleft.

Odontoma of the middle ear cleft is an unusual cause of conductive hearing loss. We report two cases and discuss the developmental mechanism by which this anomaly may occur. The differential diagnosis of calcified masses in the middle ear is also discussed.

Hydroxyapatite cement: a new method for achieving watertight closure in transtemporal surgery.

Transtemporal approaches to the petrous apex and CP angle are standard procedures in the armamentarium of the neurotologist. In the majority of these cases, it is not possible to achieve a watertight suture closure of the dura following the procedure. Subsequently, cerebrospinal fluid leakage and potential meningitis are among the most troublesome complications for both patient and surgeon. Recent use of calcium phosphate cement (hydroxyapatite [HA]) has proved efficacious in animal studies and is now being used to close cranial defects in several medical centers, as part of an FDA-IDE study in human subjects. The use of this material is described in 11 neurotologic procedures. It is believed that hydroxyapatite cement (HAC) will become a standard tool in the management of cranial base and temporal bone defects following surgery.

Dehiscent temporomandibular joint.

The appearance of an acute effusion in a well-pneumatized temporal bone directs attention to the nasopharynx and skull base. Two patients are described in whom dehiscence of the temporomandibular joint allowed herniation of the contents of the joint posteromedically, where they obstructed the middle ear entrance of the eustachian tube, the protympanum. This is, to the authors' knowledge, a previously unreported cause of an acute middle ear and mastoid effusion.

Pacientes con limitación crónica al flujo aéreo: efectos del entrenamiento muscular respiratorio con válvula de carga umbral construída con tecnología apropiada, asociado a apoyo nutricional / Patients with chronic airflow limitation: effects of muscular respiratory training with threshold load valve constructed according to appropriate technology, associated to nutritional support

To assess prospectively the effects of a controlled program of inspiratory muscle program and nutritional support in patients with chronic obstructive lung disease (COPD). 23 patients with COPD were randomly assigned into 4 groups. Group I received a 1000 kcal/day nutritional supplement, given as a casein based enteral nutritional formula; group III was subjected to inspiratory muscle training, using an inexpensive pressure threshold load valve constructed according to appropriate technology principles of the WHO, adjusted at 30 percent of Maximal Inspiratory Mouth Pressure and received also the nutritional supplement; group IV was trained but did not receive the nutritional supplement and group II was not trained nor supplemented. Patients were studied during 3 months and monthly, inspiratory muscle function, exercise capacity and antropometry were measured. A significant improvement in exercise capacity, maximal inspiratory pressure and inspiratory muscle endurance was observed in the 4 groups throughout the study. Trained subjects had greater improvement in their inspiratory muscle endurance, compared to untrained subjects. Nutritional support had no effect in inspiratory muscle function or exercise capacity. No changes in antropometric measures were observed. The pressure threshold load valve used in this study, improved inspiratory muscle endurance and nutritional support had no effect in patients with COPD