Unusual CNS infection from a subtorcular dermal sinus.

UNLABELLED: A 14-month-old toddler with a previously unrecognized dermal sinus tract presented with hydrocephalus and raised intracranial pressure. CT and MRI revealed a large posterior fossa dermoid mass. Cultures obtained from CSF and posterior fossa subdural empyema were positive for both Staphylococcus aureus and Escherichia coli. To the authors's knowledge, this is the first such report. CONCLUSION: This case suggests that children who present with CNS infections with unusual pathogens should be carefully evaluated for the presence of anatomic abnormalities including dermal sinus tracts.

Rapid infusion system for neurosurgical treatment of massive intraoperative hemorrhage.

Using an illustrative case of severe closed head injury that resulted in a posterior fossa epidural hematoma (EDH) and supratentorial epidural/subdural hematomas (SDH), the massive blood losses associated with operative repair of the torn sigmoid sinus and the significant fluid losses associated with refractory diabetes insipidus were treated by the intraoperative use of the Rapid Infusion System (RIS, Haemonetics). The RIS can rapidly infuse warm blood, crystalloid, or colloid at rates up to 1.5 L/min, thereby limiting the commonly associated hypotension, hypothermia, and coagulopathies. During the suboccipital craniectomy for evacuation of the EDH and repair of the sigmoid sinus, the patient required 18 units of blood replacement secondary to a large tear in the sigmoid sinus. During a separate craniotomy for evacuation of the SDH, the patient also developed diabetes insipidus, which increased the operative fluid replacement to 39 L. Despite these massive blood and fluid losses, the RIS limited the hypotension to less than 2 min and prevented hypothermia and the frequently associated coagulopathies. When used in a neurosurgical setting associated with massive blood and/or fluid losses, the RIS accomplishes three important objectives: (1) rapid infusion of intravenous fluids for maintaining perfusion pressure, (2) rapid warming of fluids despite high intravenous infusion rates of cold crystalloids, thereby preventing intraoperative hypothermia, and (3) continuous monitoring of infusion rates and totals.

Ultrasound-tailored functional hemispherectomy for surgical control of seizures in children.

Functional hemispherectomy techniques have been designed to minimize the long-term complications of anatomical resection without reducing the effectiveness of seizure control. The authors have used an ultrasound-guided approach tailored to combine temporal lobectomy with frontal and occipital disconnections with a central topectomy of the lateral, insular, and interhemispheric cortex. This technique achieves a comprehensive functional disconnection and minimizes entrance and manipulation within the body of the lateral ventricle. Eight patients ranging in age from 10 months to 23 years with congenital paresis and medically intractable seizures underwent functional hemispherectomy via this technique. The average surgical time was 4.5 hours, and blood loss ranged from 90 to 400 ml. All but one patient was discharged after 5 days. Postoperative fever syndromes, aseptic meningitis, and infection were avoided. On long-term follow-up evaluation (range 18-60 months, mean 38 months), seven of eight patients remain seizure free and were not on a course of anticonvulsant agents. Advantages of this technique include avoiding entrance into the ventricle, a more predictable postoperative period, and reduced postoperative complications.

Growth hormone deficiency following radiation therapy of primary brain tumors in children.

The medical records of 123 patients treated for brain tumors at Children's Hospital and Medical Center, Seattle, Washington, between 1985 and 1987 were reviewed. The endocrinological complications of radiation therapy and the effectiveness of growth hormone (GH) replacement therapy were assessed. These were the first 2 years after synthetic GH became available. The disease pathology was confirmed at craniotomy or biopsy in 108 patients. Ninety-five children completed radiation therapy and 65 of these were alive at the time of review; these 65 children represent the study population. The most common tumor types were medulloblastoma, craniopharyngioma, and ependymoma. Endocrine evaluation was initiated with changes in the patients' growth velocity. Patient workup included skeletal x-ray films for determination of bone and analysis of thyroxin, thyroid-stimulating hormone, and somatomedin-C levels. Following 1-dopa and clonidine stimulation, provocative studies of GH levels were performed. Growth hormone failure and short stature were observed in 26 children, most commonly in the 2nd year after tumor treatment. Eight patients with GH failure were also hypothyroid. Hormone replacement therapy was initiated with recombinant GH, 0.05 mg/kg/day, and all children so treated showed an increase in height, with eight patients experiencing catch-up growth. There were no complications of therapy or tumor recurrence. Studies of baseline bone age and somatomedin-C levels on completion of radiation therapy are recommended. Comprehensive endocrine studies should follow changes in the patients' growth velocity. With early GH replacement, catch-up growth is possible and normal adult heights may be achieved.

The risks of metastases from shunting in children with primary central nervous system tumors.

The authors reviewed the hospital charts of 415 pediatric patients treated for benign or malignant primary brain tumors over the past 20 years at the Children's Hospital Medical Center, Seattle. Patients' ages ranged from the neonatal period to 18 years. A shunt was placed in 152 patients (37%), 45 before and 94 after surgery. Confirmation of extraneural metastases was based on clinical and diagnostic examination. Factors analyzed as possibly influencing the occurrence of extraneural metastases were: 1) the shunt: type, valve, location, filter, and revisions; 2) extent of resection; 3) pathology; and 4) treatment regimen. Eight of the 415 patients developed extraneural metastases during life. All eight patients had a medulloblastoma (cerebellar primitive neuroectodermal tumor). These eight patients were separated into Group A (without a shunt) and Group B (with a shunt). In Group A (five patients), the mean interval from primary diagnosis to metastasis was 15 months. Two children had gross total resection of the tumor. The predominant location of metastases in Group A was: bone (two cases); cervical lymph nodes (one); lung/bone (one); and retroperitoneal pelvic mass (one). Three Group A patients had a simultaneous central nervous system (CNS) recurrence. Of the three Group B patients, two had a ventriculoperitoneal (VP) shunt and one a ventriculoatrial (VA) shunt; all were placed postoperatively. One Group B patient had a simultaneous CNS recurrence. No shunt revisions were performed in these three patients. The mean time from primary diagnosis to metastasis was 25 months. One patient had a total tumor resection. The predominant location of metastases was bone (one case), retroperitoneal pelvic mass (one), and abdominal cavity with ascites (one case). Only one patient in the entire series had a filter placed; this resulted in shunt obstruction and was removed 1 month following placement. It is concluded that cerebrospinal fluid shunts, regardless of type, location, revision rate, or filter insertion, do not predispose pediatric patients with brain tumors to develop extraneural metastases. A diagnosis of shunt-related metastases should be based on the development of intra-abdominal (VP shunt) or pulmonary (VA shunt) dissemination primarily with or without additional sites. The diagnosis of medulloblastoma is an important factor related to metastasis occurrence while the extent of resection and postoperative therapy are not influential.

Management and long-term follow-up review of children with lipomyelomeningocele, 1952-1987.

The medical records of patients at Children's Hospital and Medical Center treated for lipomyelomeningocele repair were reviewed to assess the long-term outcome of surgery. In total, 108 children were cared for between 1952 and 1987, and long-term data are available for 80 of 96 children who underwent surgery. Cutaneous manifestations and associated malformations were common. Surgical repair was performed at ages 1 week to 17 years; there was no operative mortality. Thirty-five of 38 children with normal preoperative examination were without deficits on long-term follow-up review. Bowel and bladder paralysis was present in 42 children and did not recover after release of cord tethering. Eleven children had return of symptoms and were reexplored 3 to 8 years after initial surgery. Surgical repair with release of cord tethering at the time of diagnosis is advocated, regardless of patient age.

Dermoids and dermal sinus tracts of the spine.

The evaluation of a child with a midline dimple or pit is one of the most common referrals to a pediatric neurosurgeon. Regardless of their depth, those below the top of the intergluteal crease end blindly and never extend intraspinally. Families can be reassured there is no infection or mechanical risk to the developing nervous system. A high index of suspicion must be maintained for all dimples above the intergluteal fold despite a normal examination or neuroradiologic studies. The midline must be carefully inspected when a child of any age suffers meningitis, especially when an unusual organism is cultured. Conservative management of dermal sinuses is not justified, and these lesions should be electively resected at the time of diagnosis. Dermal sinuses and inclusion tumors may lead to spinal cord tethering and progressive neurologic deterioration. Surgery in advance of deficits maintains normal neurologic function, and children can develop unencumbered by infection, motor, or bladder paralysis.

The treatment of hydrocephalus.

Hydrocephalus is one of the most commonly encountered clinical problems in neurosurgery and accompanies most cases of myelomeningocele. Spinal fluid shunting to the peritoneal cavity is the most common surgery performed for hydrocephalus, and there is a vast array of valve systems and hardware available. The authors present their techniques and refinements for placement of shunt systems and strategies to avoid complications. The medical management of hydrocephalus and shunt infections also is examined.

Surgical correction of sagittal craniosynostosis: complications of the pi procedure.

We have completed a retrospective analysis of our experience with the pi procedure to determine the complications of the technique. Forty-five children underwent correction of scaphocephaly during the last 4 years. The average patient age was 9.2 months. The modified prone position with beanbag head support was used in 40 of 45 patients. Accompanied by barrel-stave and radial osteotomies, the reverse procedure was performed in 18 patients and frontal pulling in 27 patients. The average anteroposterior skull reduction was 1.44 cm, and mean blood loss was 96 ml, 11 ml/kg, replaced in 36 patients. Complications included three dural injuries, and air embolism was not detected by Doppler or end-tidal monitoring. A single child had a postoperative seizure. We conclude that complications of the pi procedure are limited and compare favorably with other repair techniques. We consider the pi procedure the technique of choice for correction of sagittal craniosynostosis in patients older than 3 months.

Reflections on the natural history of lipomyelomeningocele.

A retrospective analysis of patients with lipomyelomeningocele cared for at two referral centers was completed to derive relationships between neurological function and patient age. Thirty-seven percent of 177 patients had intact neurological function on initial examination. Neurological deficits were progressive and linked with a logarithmic relationship to increasing patient age. Each child with intact examination retained normal bladder function following lipomyelomeningocele repair and release of cord tethering; complications of surgery were limited. Our analysis suggests that surgery on patients with intact function offers greater long-term protection of critical function than is offered by conservative management and expectant care. We recommend repair of lipomyelomeningocele at the time of diagnosis regardless of patient age or neurological function.

Cytogenetic analysis of a choroid plexus papilloma.

We report the cytogenetic analysis of a choroid plexus papilloma, a benign tumor, with a modal number of 56 chromosomes. In our review of the few reported karyotypes of choroid plexus tumors, we found no predictive relationship between the karyotype and the pathologic diagnosis or outcome.

Pediatric intracranial pressure monitoring in hypoxic and nonhypoxic brain injury.

We reviewed the results of all pediatric patients undergoing intracranial pressure (ICP) monitoring in a 2-year period at our institution. The outcome of patients suffering hypoxia or ischemic injuries (HII) is compared to those suffering non-hypoxic or non-ischemic injuries (NHII). Thirty-four patients had ICP monitors placed during the study period. Incomplete patient information led to the exclusion of 5 patients. An additional 5 patients were excluded because no measures to control ICP were taken after the monitor was placed. Twenty-four patients required treatment for raised ICP (hyperventilation, 24; mannitol, 19; barbiturate coma, 6). Admission Glasgow Coma Score in patients suffering HII (median score 5) and NHII (median score 6) were not significantly different (Mann-Whitney U Test). Only 2 of 8 patients with HII were near-drowning victims. The remaining 6 had HII from other causes (5 survivors of various forms of asphyxia and 1 of cardiac arrest). All 8 patients had poor outcomes (1 severely disabled; 7 died). The 16 patients with NHII had a variety of diagnoses (6 trauma, 5 encephalitis, 4 bacterial meningitis, 1 diabetic ketoacidosis). Among these, 6 had good outcomes and 10 poor outcomes (2 severely disabled, 2 vegetative, and 6 died). The difference in outcome between patients with NHII and HII is significant at P = 0.059 (Fischer Exact test). Patients with NHII may benefit from ICP monitoring. Patients with HII from near-drowning and other causes did not appear to benefit from ICP monitoring and interventions directed at controlling ICP.

Cerebral syrinx with Chiari I malformation.

A 13-year-old female presented with acute left gaze paralysis. MRI revealed hydrosyringomyelia (HSM) with syringobulbia in the left pons extending cephalad into the centrum semiovale. A suboccipital craniotomy was performed and the foramen of Magendie was imperforate. Ocular movements normalized in 2 months and postoperative MRI confirmed resolution of the cerebral syrinx and syringobulbia and diminished HSM. This case represents an extreme example of the altered spinal fluid dynamics with Chiari I malformation. Theories on HSM are reviewed.