Melanotic medulloblastoma. Report of a case with ultrastructural findings.

A pigmented neoplasm of the cerebellar vermis in a four year old child was typical of differentiating medulloblastoma with islands of epithelial-like cells containing melanin pigment. There have been several previous reports of such melanotic cerebellar neoplasms. Reported cases have had a clinically malignant behavior with dissemination in the central nervous system. They appear to be variants of medulloblastoma and not pigmented neuroectodermal tumors of infancy (melanotic prognomas or retinal anlage tumors). Ultrastructurally the neoplasm was compatible with medulloblastoma with focal poorly differentiated cells which contained melanin pigment. The pigment resembled neural crest (cutaneous or ocular) melanin rather than neuromelanin.

Dumbbell neuroblastomas in children.

A "dumbbell" neuroblastoma is a malignant neoplasm usually primary in the mediastinum or retroperitoneum with posterior extension through an intervertebral foramen to additional tumor within the spinal canal. Neurologic deficits are found in almost all cases. Nineteen patients with such tumors were reviewed with respect to diagnosis, management, and results. Treatment consisted of prompt laminectomy with total or subtotal excision of the extradural tumor. The primary tumor in the mediastinum or retroperitoneal area was removed at a second stage. All patients received postoperative radiation therapy. Eleven of the 17 patients observed more than two years are free of disease, a cure rate much higher than the overall experience with neuroblastoma. Substantial recovery of neurologic function occurred in most, but kyphoscoliotic deformities were a disturbingly frequent late complication.

Congenital scalp defects: aplasia cutis congenita.

The authors discuss the rare lesion of congenital scalp defect, both partial and full thickness. The majority occur in children who are otherwise normal, but a few are seen in children who have many concomitant anomalies. A high percentage of the multiple anomaly cases were found to have trisomy 13-15. Etiology of these lesions is not clear, but there seems to be an inherited component. Treatment of otherwise normal children is conservative for the smaller lesions, with excision and primary closure when possible for the larger ones.

Sagittal synostectomy. Technical note.

A procedure for correction of scaphocephaly is described. It differs from strip craniectomy in that it provides early correction of the deformity by removing the protuberances at the bregma and at the occiput. The morbidity and mortality of this procedure is no greater than with the more standard linear craniectomy.

The dilemma of childhood optic gliomas.

A retrospective series reviewing 28 cases of optic glioma in childhood is reported from the Columbus Children's Hospital. The literature is reviewed from a clinical and pathological standpoint; in the pathological literature there is support for consideration of these lesions as a neoplastic entity, but none for designation of these gliomas as "hamartomas." An approach to the management of optic gliomas is presented. Treatment should be tailored to the individual patient; no clear therapeutic protocol appears to be supported by available data.

Primitive neuroectodermal tumors of the central nervous system in children.

A series of 18 primitive neuroectodermal tumors in children (15 cerebral and three spinal) is reported. These are highly malignant neoplasms, both histologically and clinically. They are rapidly growing tumors, with a brief duration of symptoms and a rapidly progressive course. Forty percent of the patients were alive at 6 months, only 10% at 1 year, and all patients had died within 2 years following diagnosis.

Shunt complications.

To some extent our greatest problem is the philosophic answer to the question, "who shall be treated, how intensely and to what expense?" The cost of care is almost always greatest in the child who is borderline in selection and least likely to survive. Over half of the children shunted after application of searching criteria appear to this author to be compensated (Table 28.13).

Application of blood cadmium determination to industry using a punched disc technique.

A paper disc flameless atomic absorption spectroscopy (AAS) method is described for the determination of cadmium (Cd) in blood, enabling difficulties in sample preparation to be minimized. By control of the ashing step the matrix can be removed without loss of cadmium. Problems with the fast signal response during atomization can be met by spectral band width and temperature control. At the 106 pg level (471 nmol Cd/1 blood; 5-3 mug/100 ml) the relative standard deviation (RSD) was 0-06. Results in four industrial situations are reported. This description of the method should facilitate further investigation of its application to industry using capillary or venous blood.

Percutaneous third ventriculostomy: experience and technique.

A series of stereotaxic percutaneous third ventriculostomies has been carried out on 46 shunt-dependent hydrocephalic children. The technique and results are presented. The advantages of this procedure over the open craniotomy type are discussed. There has been low morbidity and one mortality. The necessity of preoperative shunting to provide a 'slack brain' is emphasized.

Familial inheritance of coronal craniosynostosis.

Five members of three families with premature fusion of the coronal sutures are briefly reported. The pedigrees of these patients are presented. In each case the mode of inheritance was autosomal dominant with reduced penetrance.

The Kleeblattschädel anomaly.

Two cases with the characteristic features of the Kleeblattschädel anomaly are presented. The precise etiology of this rare syndrome is unknown and no genetic or environmental factors have been identified to date. Within the spectrum of disorders characterized by craniostenosis, the Kleeblattschädel anomaly appears to represent the most severe degree of expression. An early aggressive neurosurgical approach to correct the cranial malformation did not significantly alter the poor prognosis in these two patients.

Experiences with the technique and complications of meglumine iothalamate (Conray) ventriculography.

The development of contrast ventriculography is outlined and an experience with 40 meglumine iothalamate ventriculograms in 37 patients is described. The details of the procedure, hazards, value, and indications are discussed.

Subtotal neonatal calvariectomy for severe craniosynostosis.

This report sets forth an example of a new mode of management of severe craniosynostosis in the neonate: subtotal calvariectomy. An infant with synostosis of sagittal, coronal, and lambdoidal sutures who had signs of increased intracranial pressure with impending neurologic complications was operated on at 13 days of age. Wide excision of the bony calvarium from the underlying dura was accomplished from the supraorbital ridge to near the foramen magnum; the entire area of the coronal sutures was included. Regeneration of the calvarium occurred over two to 12 weeks and was accompanied by the presence of "sutures" in a usual location. These procedures have allowed for normal brain growth without reoperation up to the current age of two years. Neurologic and intellectual performance has been satisfactory, and cosmetic results appear to be preferable to those obtained by more limited surgery. The observations suggest that this approach may permit better craniofacila growth by minimizing secondary deformities in growth of the cranial base.

Central neurogenic pulmonary edema: with a review of its pathogenesis and treatment.

One of the unusual signs of sudden increase in intracranial pressure is central neurogenic pulmonary edema. This pulmonary edema produces a capillary-aveolar block resulting in hypoxia complicating the underlying neurologic condition. Good success in treating this problem has been obtained with controlled ventilation and positive end expiratory pressure respiration.

The management of the Kleeblattschadel syndrome.

This paper describes the classic presentation of the Kleeblattschadel syndrome and the involvement of the ophthalmologist in preventing the loss of sight from exposure. It also described newer neurosurgical approaches for these patients and improvement in the outcome of this syndrome.