John E. Kalsbeck

Ventriculography and Myelography with Absorbable Radiopaque Medium

Positive contrast radiographic studies of the central nervous system were first reported by Sicard and Forestier in 1922, using Lipiodol (8). Other substances investigated since that time include Abrodil (1), Thorotrast (5), Perabrodil or Diodrast (2), Pantopaque (4, 6, 9, 10, 11), Hypaque (3), and various chelate compounds (7). Failures with air, Pantopaque, and Lipiodol at stereotaxic surgery and in suspected midline tumors aroused our interest in absorbable positive contrast media. This paper reviews our investigations with Conray and other water-soluble positive contrast agents. Laboratory Investigations LD50 Study: A previous report indicated that Conray was less toxic than other similar compounds when injected cisternally in the rabbit. Toxicity studies were carried out in our laboratory with Conray, Hypaque, Skiodan, and a series of experimental compounds. These will be reported in detail later, but Conray was found to be considerably less toxic than either Hypaque or Skiodan. Isotope Studies: l131...

Origin of Organisms Infecting Ventricular Shunts

&NA; Results of skin cultures obtained before 413 of 505 operations for cerebrospinal fluid‐diverting ventricular shunt placement or revision in a pediatric population from April 1980 to May 1983 are analyzed and compared to results of cultures from 20 subsequent shunt infections. Sensitivities to 11 different antibiotics were determined for each isolate cultured. The total operative infection rate was 20 of 505 (4%). Gram‐negative bacilli alone accounted for 3 of 20 (15%) shunt infections. One gram‐negative bacillus/Staphylococcus aureus infection occurred. Factors predisposing for gramnegative bacillus shunt infection were found in all 4 cases. The majority of shunt infections were caused by typical resident skin organisms: Staphylococcus epidermidis alone, 9/20 (45%); Staphylococcus aureus alone, 4/20 (20%); Corynebacterium sp., 1/20 (5%); &agr;‐Streptococcus with S. epidermidis, 1/20 (5%); and Micrococcus with S. epidermidis, 1/20 (5%). Only 4 (20%) of the 20 shunt infections were due to organisms identical to those originally grown from the skin. Another 4 (20%) seemed to be infected with a strain of organism different from that initially recovered from the skin. The remaining skin organism shunt infections may or may not have come from the patients skin. The data suggest that not all skin organism shunt infections arise from contamination by resident skin bacteria at the incision sites at the time of operation. Alternate sources for the infecting organisms are discussed. The antibiotic sensitivity data on skin isolates and shunt isolates suggest that vancomycin is the antibiotic best suited for prophylaxis against shunt infection at our institution.

Optic Chiasmatic Glioma in Children

We reviewed the records of 29 patients with optic nerve or chiasm glioma, or both, seen from 1955 to 1966. Sixteen patients were girls and 13 were boys. At the time of diagnosis, 14 patients were less than 2 years old. Optic atrophy was the most frequently seen physical finding, present in 26 of 29 patients. Twelve patients had diencephalic syndrome (41%). Proptosis was seen in eight. Eleven patients (38%) had associated neurofibromatosis. Pneumoencaphalogram was done on 26 patients and was abnormal in 23. The diagnosis was confirmed at surgery in 27 patients. All tumors were astrocytomas. Eighteen patients underwent radiotherapy. Surgery and radiotherapy were used as treatment for optic-chiasmatic glioma with diencephalic syndrome.

Ventricular gallbladder shunts: An alternative procedure in hydrocephalus

Hydrocephalus is a frequently encountered problem in infancy and is most commonly treated by placement of ventriculoperionteal (VP) or ventriculoatrial (VA) shunts. Other sites for insertion of the distal shunt have included the stomach, ureter, and fallopian tube. This report describes an experience with ventricular gallbladder shunts (VGB) in 25 children performed from 1970 to 1985. There were 13 girls and 12 boys ranging in age from 6 months to 16 years. Diagnosis included meningomyelocoele (7), congenital hydrocephalus (7), postmeningitic complications (5), intracranial tumor (4), and intraventricular hemorrhage (2). Indications for operation included VP shunt infection (15), massive ascites following VP shunt (3), VA shunt infection (4), and distal shunt malfunction due to fibrinous adhesions or cysts (secondary to infection; (3). Three patients had early shunt failure due to proximal obstruction (2) and gallbladder atony (1). Shunt revision was required in two and the atony was successfully treated with cholecystokinin in one. Fourteen shunts remain in place, two patients have been lost to follow-up, and three children died from unrelated causes. Seventy percent of the 20 patients available for long-term follow-up have functional shunts in place. The VGB shunt procedure remains an attractive alternative for patients with hydrocephalus in whom intraperitoneal and intravascular shunts are no longer feasible.

CLOACAL EXSTROPHY: THE NEUROLOGICAL IMPLICATIONS

Congenital anomalies commonly accompany the abdominal wall defects of cloacal exstrophy with an incidence as high as 100% in some series. The urological literature cites an incidence of vertebral and/or spinal cord anomalies in 75% of cases. We reviewed the records of the 17 patients with cloacal exstrophy treated at our institution since 1978. Abnormalities of the vertebral column or spinal cord were identified in 16 patients, including 11 of 12 (92%) fully evaluated by myelography, computerized tomography or magnetic resonance imaging of the lumbosacral spinal cord. Nine patients had a lipomeningocele and 4 had a lipomyelocystocele. Spinal exploration, repair and cord untethering were done in 11 patients of whom 2 subsequently underwent reoperation for re-tethering the cord. A total of 15 patients is ambulatory although 5 require lower extremity braces. The incidence of spinal cord or vertebral anomalies in our patients with cloacal exstrophy approaches 100%, which is higher than commonly cited in the urological literature. We recommend magnetic resonance imaging of the spinal cord as part of the initial evaluation of newborns with cloacal exstrophy. Lifelong attention to the risk of spinal cord tethering can optimize neurological outcome in affected individuals.

Myelodysplasia and Developmental Scoliosis: A Manifestation of Syringomyelia

Fourteen of 15 myelodysplastic patients evaluated for developmental scoliosis were found to have communicating hydrosyringomyelia, a manifestation of spontaneously arrested hydrocephalus. Eight of these patients had progressive extremity paralysis in addition to the scoliosis. Treatment by ventricular decompression was associated with short term stabilization in 6 early cases of scoliosis. However, more advanced scoliosis in 2 patients continued to progress. Neurologic deficits were improved even in advanced cases by ventricular shunting. The authors propose that hydromyelia and syringomyelia are the cause of developmental scoliosis in myelodysplasia and suggest methods of investigating and treating patients with these conditions.

A Study of Experimental Syringomyelia by Scanning Electron Microscopy

Experimental hydromyelia and syringomyelia, induced by kaolin in dogs, has been investigated by scanning electron microscopy. This study demonstrated that communication between the 4th ventricle and the spinal central canal is normally occluded by a plug of acellular material lying within the central canal. This plug is broken down following the onset of hydrocephalus and communication is rapidly established between the ventricles and the central canal. The canal distends and ruptures dorsally into the spinal gray matter with the development of extensive intramedullary cavities, lined by glial and neuronal elements. The morphological changes demonstrated are consistent with a physical destructive process of the cord resulting from CSF dissection.

Chiari III malformation treated with CSF diversion and delayed surgical closure

Chiari III malformations are extremely rare hindbrain malformations that are associated with a high early mortality rate, or severe neurologic deficits in the survivors. The preferred treatment is early operative closure and CSF shunting. We report a case of a newborn infant with a Chiari III malformation with displacement of the brainstem and cerebellum into the cervical encephalocele which precluded immediate operative closure of the defect. Instead, a ventriculoperitoneal shunt was placed and the patient was followed with serial imaging studies. The child survived. The shunt allowed the brainstem and cerebellum to regress into the cervical spinal canal as the dilated cerebral aqueduct and fourth ventricle decompressed. A delayed closure of the cervical encephalocele was performed at 30 months of age. Cerebrospinal fluid diversion with delayed closure may be an option for large lesions.

Developmental Outcome of Very Low Birth Weight Infants Requiring a Ventriculo-Peritoneal Shunt

All infants of less than 1,500 g birth weight who required a ventriculo-peritoneal shunt were followed for developmental problems over the 1st year of life. There were 19 infants; 2 died within the 1st postoperative week. At 1 year of age corrected for prematurity the mean Bayley Mental Developmental Index was 62 +/- 23.0, and the mean Psychomotor Developmental Index was 61 +/- 19. Only 2 infants attained scores greater than 85 on both scales, while an additional 2 infants attained a score of greater than 85 on one scale. It is concluded that ventriculo-peritoneal shunting procedures performed after progressive ventricular enlargement has taken place are not likely to result in normal development of the infant less than 1,500 g birth weight.

A histochemical investigation of catecholamines in spinal cord injury.

The catecholamine hypothesis of progressive spinal cord necrosis following mechanical trauma was investigated with the histofluorometric method. Forty-four adult mongrel dog were examined as control, L1 crush-injured, and crush-injured with prior T1 total transection groups. In crush injured dogs, catecholamines were present in a 1 cm length of white matter at the crush site, with the greatest accumulation in the deep lateral and ventral funiculi. Gray matter fluorescence was not enhanced. Prior transection did not abolish the intense accumulation of catecholamines at the site of the cord injury. We propose that the catecholamines accumulating at the cord injured site are not central in origin, but represent an uptake mechanism into white matter as a reflection of cord microperfusion.