Allogeneic cartilage used for skull base plasty in children with primary intranasal encephalomeningocele associated with cerebrospinal fluid rhinorrhea.

Three children with primary intranasal encephalomeningocele associated with cerebrospinal fluid rhinorrhea were operated on at the Department of Neurosurgery, Hradec Králové. In two children, aged 4 and 9.5 years, freeze-dried allogeneic costal cartilage was glued into the skull base defect. This plugging was covered up with deep frozen allogeneic fascia lata. In the third child, an only 1-year-old boy, after transection of the neck of the encephalomeningocele freeze-dried allogeneic dura mater was glued on extradurally and deep-frozen allogeneic fascia lata applied intradurally. The cerebrospinal fluid rhinorrhea ceased immediately after surgery. Spontaneous atrophy of the intranasal portion of the encephalomeningocele was demonstrated respectively 11, 1, and 7 years postoperatively on computed tomography. To evaluate cartilage healing histologically, the extracted allogeneic cartilage used for orbital roof plasty after 4 months was examined. The extent of spotty regressions represented about 7% of the tissue volume. It is stressed that, once diagnosed, intranasal encephalomeningocele associated with cerebrospinal fluid rhinorrhea should be operated on for prevention of meningitis as soon as possible.

Catheterobronchial fistula due to vena cava superior thrombosis as a late complication of ventriculoatrial shunt.

A case of a catheterobronchial fistula as a rare late complication of a ventriculoatrial shunt is reported. The ventriculoatrial shunt was implanted in a 4-month-old boy suffering from extreme postinfectious hydrocephalus. During the following years, twelfth nerve palsy on the right, vertebralgias, and salty taste sensations in the mouth associated with intermittent coughing and swelling of the neck and supraclavicular region on the right side developed. Valvography established a diagnosis of fistula 12 years after the implantation of a shunt. Ultrasonography of the neck and mediastinum and contrast-enhanced dynamic computed tomographic scanning demonstrated a catheterobronchial fistula to the subsegmental bronchus of the anterior segment of the right upper lung lobe, a thrombosis of the right internal jugular and both right and left brachiocephalic veins and the superior vena cava, and an extensive collateral venous system mainly draining into the azygos vein. Normalization of cerebrospinal fluid and blood flow and pressure allowed extraction of the "atrial" catheter without complications. One year after surgery the boys is in good health and without signs of shunt dependence.

Multiple sulphatase deficiency in homozygotic twins.

Multiple sulphatase deficiency was studied in 3 siblings--one pair of monozygotic twins and their sister. The children's psychomotor development was arrested at the age of 18 to 24 months, and the hypotonic syndrome combined with signs of spasticity appeared. There was marked hepatosplenomegaly, conspicuously dry scaly skin with the decortication syndrome developing and persisting in the presence of pronounced cachexia. Also present were numerous X-ray abnormalities, metachromatically staining granules in the urine, and Alder- Reilly 's bodies in the blood leukocytes and in specimens of bone marrow. Liver, skin and muscle biopsies performed simultaneously revealed accumulations of water-soluble mucopolysaccharides and deposits of sulphatides in the two twins. Enzyme assays demonstrated arylsulphatase A and B deficiency. The diagnosis was subsequently confirmed at all the three siblings' postmortem examinations.