Luis Schut

Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients.

Treatment options for the craniosynostoses vary from conservative observation until completion of growth to radical remodeling in infancy. To further define the timing and type of treatment necessary in these complex disorders, we have retrospectively analyzed all patients operated on for this deformity during the past 12 years. One-hundred and sixty-four patients with craniosynostosis were analyzed and subgrouped into asymmetrical (predominantly unilateral) and symmetrical (bilateral) deformities, in addition to segregation by age and type of procedure performed. This was done recognizing that no deformity, like no normal human face, is truly symmetrical. Results of treatment were categorized on the basis of the need for additional surgery and varied from no refinements necessary (category I) to major reduplication of the initial procedure (category IV). Analysis of the data led us to conclude that excellent results can be expected in the asymmetrical deformities group treated in infancy by a unilateral approach. Similarly, for the mild symmetrical deformities, treatment at this time by bilateral orbital advancement gives satisfactory results in the majority of patients. By contrast, the more severe symmetrical groups treated in childhood have a high incidence of requiring secondary major reconstructions, and consideration should be given to delaying craniofacial surgery until age 7 or older, although earlier cranial surgery may be advisable.

Pathophysiology, treatment and outcome following severe head injury in children.

The pathophysiology and outcome following severe head injury in 85 children are presented. The commonest initial CT diagnosis was of acute brain swelling. This swelling was associated with an increased white matter density on the CT scan which decreased to normal concomitant with recovery and increased ventricular size. CBF measurements in 6 of these patients revealed an increased blood flow despite a decreased CMRO2 and clinical coma. This CT pattern of diffuse swelling is believed to be due to acute cerebrovascular congestion and hyperemia and not to edema. Because of this, all children were treated with endotracheal intubation and controlled hyperventilation as part of the initial management. Mass lesions were uncommon, 20%. ICP was monitored in 40 children. The ICP rose above 20 Torr despite therapy in 80% of children with decerebrate or flaccid coma and in only 20% of children with spontaneous motor function. The ICP was at its highest between the second and fifth day. Aggressive therapy to control the ICP, with barbiturates if necessary, was successful in 80% of the patients. The overall results were useful recovery in 87.5% of the children, 3.5% were left vegetative or severely disabled and 9% died.

Pediatric brain stem gliomas.

Sixty‐two pediatric patients with brain stem glioma diagnosed between 1964 and 1978 have been reviewed. Posterior fossa exploration was performed on 53% of the patients. Pathology was obtained by biopsy in 58% of those explored. Fifty‐four patients had definitive radiation therapy with a median dose of 5000 rads. The actuarial five‐year survival for the entire group is 30%.

Intracranial ependymomas in children

Between 1970 and 1988, 51 children with intracranial ependymal tumors (33-infratentorial, 18-supratentorial received initial treatment at the University of Pennsylvania. Therapy consisted of total or near total tumor resection in 15 patients and partial resection or biopsy in 36. Postoperative irradiation alone was given to 18, chemotherapy to 4, and a combination of these two modalities to 26. Patients have been followed for a median period of 7.75 years. The 5-year actuarial survival and progression-free survival (PFS) rates are 46% and 30%, respectively. Of the 30 patients who have progressed, 29 did so locally and one died before the site of failure could be determined. Six patients also had disease outside the primary site at relapse; three of them had received craniospinal irradiation. Local control was significantly better for patients whose tumor dose exceeded 4500 cGy (32% vs. 0%, p = .01) and for Caucasian patients (34% vs. 15%, p =.05). Survival was better for patients who were over 4 years of age at diagnosis (55% vs. 30%, p = .04), for patients who received local radiation doses above 4500 cGy (51% vs. 18%, p = .01), and for Caucasian patients (43% vs. 14%, p = .01). Extent of resection, histology, location, the use of cranial or craniospinal irradiation, and the use of chemotherapy did not significantly impact on survival. We conclude that the inability to control local disease remains the single most important factor leading to treatment failure. Older age, higher local radiation dose, and Caucasian race appear to be the only favorable prognostic factors.

Computed Tomography of Craniocerebral Injury in the Abused Child

Computed tomography (CT) was performed in 26 infants and children with craniocerebral trauma related to abuse during a 41-month period. The pattern of brain injury differed from that seen in the nonabused traumatized infant or child. Parieto-occipital acute interhemispheric subdural hematoma (AIHSH) with associated parenchymal injury was the most frequent finding (58%). Follow-up by CT in patients with AIHSH demonstrated infarction in half and cerebral atrophy in all.

Choroid plexus carcinoma of childhood

The presentation, growth patterns, and response to therapy of 11 consecutive children with choroid plexus carcinomas were analyzed, and the results were compared with the outcome reported in other series. Patients were a median of 26 months of age at diagnosis. Two patients had thalamic tumors, one had a posterior fossa primary, and the rest had ventricular lesions. Five of 11 (45%) children remain in continuous progression‐free remission a median of 48 months from diagnosis. Four of the five in continuous remission had a “gross total” surgical resection, and only one received radiation therapy. Five of six patients with subtotal resections relapsed despite postoperative treatment with radiation therapy (three) and chemotherapy (one). The response to treatment with radiation therapy or chemotherapy at relapse was disappointing, with only one child (treated with etoposide) responding. In combination with other series, 11 of 14 children had prolonged progression‐free survival after gross total resection (only two of whom received adjuvant therapy) compared with two of 20 after less than total resections, independent of the type of adjuvant therapy given. Adjuvant therapy for children with choroid plexus carcinomas is of unproven benefit, and this must be considered when analyzing innovative treatment trials for such children, especially for those with totally resected tumors. Patients with partially resected lesions fare poorly with present forms of treatment.

THE ROLE OF RADIATION THERAPY IN THE MANAGEMENT OF CHILDHOOD CRANIOPHARYNGIOMA

Between 1965 and 1986, 31 children were treated for craniopharyngioma at the Childrens Hospital of Philadelphia. Total removal was attempted in all patients. Some patients received radiation therapy following subtotal removal. Of the patients whose first resection was subtotal, five received radiation and seven did not. Four of the 5 patients who were radiated (80%) are stable (median 89 months, range 42-155 months) and one recurred at 42 months, failed salvage with total removal, and subsequently died of disease. Of the seven who were not irradiated, all had recurrences (median 12 months, range 3-192 months) and one died of disease. Nineteen patients initially had total removal and none received adjuvant radiation. One patient died postoperatively. Of the 18 remaining patients, 6 had recurrences (median 24 months, range 7-100 months) and 12 (66%) are stable (median 42 months, range 9-133 months). One of these stable patients died of endocrine complications 24 months after initial surgery. Fourteen of the 31 patients recurred. Two died with recurrence and one required no further treatment. Eleven had second resections following initial surgical removal. Seven of these 11 went on to receive radiation and four did not. All seven who were radiated are stable (median 33 months, range 1-228 months); whereas 1 of the 4 who were not radiated recurred again at 18 months. This patient had a third resection followed by radiation therapy and is now stable at 20 months. After initial surgery (and before radiation, when given) 26 of 31 patients had panhypopituitarism, 4 had partial deficits, and 1 was normal. Severe diencephalic syndrome, loss of visual acuity, and intellectual deficits were no more frequent in patients treated with total removal, subtotal removal, and in patients who received radiation. We conclude that radiation has an important role following subtotal removal and for salvage treatment after initial surgery. Aggressive attempt at total removal does result in prolonged progression-free survival in some patients. Extensive resections may result in significant mortality and endocrine morbidity. This review suggests that subtotal removal and radiation results in outcomes at least as favorable as treatment with total removal alone.

Leptomeningeal dissemination of low‐grade gliomas in childhood

Although leptomeningeal spread (LMS) of primary CNS tumors in children has been well documented in the literature, it has rarely been reported in children with low-grade gliomas. Between 1975 and 1985, 6 of 162 children (3.7%) with low-grade gliomas treated at Childrens Hospital of Philadelphia had LMS. LMS was present at diagnosis of the original tumor in one patient, was the first sign of relapse in one patient, occurred simultaneously with local relapse in two patients, and after local relapse in two patients. Pathology of the original tumor was low-grade astrocytoma in five and low-grade oligodendroglioma in one. Primary tumor site was cervical cord in three, chiasm in one, frontal lobe in one, and cerebellum in one. All of the children with LMS had undergone surgical treatment at the time of diagnosis of the primary tumor; four had total resections at some point in their course. Three of the six patients died; three are still alive after treatment with radiation therapy and/or chemotherapy. The longest survival to date has been 31/2 years after diagnosis of LMS. We compared clinical characteristics of these six patients with 131 children with low-grade tumors without dissemination treated at our institution during the same time period. LMS, although relatively infrequent, does occur in children with low-grade gliomas, especially spinal cord tumors. LMS may occur at any time during illness and diagnosis may be difficult unless LMS is suspected. Treatment, at times, results in clinical improvement and considerable disease control.

Spinal Lipomas in Infancy and Childhood

The clinical course of 42 children with lipomatous lesions of the lower spine is reviewed. Good neurological exam, IVP and myelography are considered essential components of the preoperative studies. The surgical aim is to free all intra- to extradural adhesions with dural grafting, if necessary. The results suggest that early corrective surgery within the first few weeks to months of life is the most satisfactory approach to preserving normal neurological and urological function in these children. 88% of children not operated on early developed progressive loss of neurological function and while delayed surgery frequently stopped progression of the disease it rarely returned the children to a normal state.

Recurrent intracranial ependymomas in children survival, patterns of failure, and prognostic factors

Thirty‐six pediatric patients (ages 0.8–16.8 years) with recurrent intracranial ependymoma were treated for a total of 52 separate cases of relapse from 1970 to 1989. Therapy consisted of surgery in 33 cases and chemotherapy in 38 cases. Twelve patients received radiation at the time of first relapse, and five of these 12 who had initially been treated with surgery and chemotherapy alone were irradiated to full dose. The 2‐year actuarial survival and progression‐free survival (PFS) rates are 29% and 23%, respectively. Two‐year survival after treatment of first relapse is 39%. Of the 52 cases, there have been 44 subsequent relapses (and one septic death), three of which have occurred in the five patients treated with definitive radiation. Twenty‐seven relapses have occurred exclusively with local disease. Eight patients failed with disease outside as well as in the primary site. Survival was better for patients who had histologically benign lesions at relapse (53% versus 9%, P < 0.02), and for patients in first versus subsequent relapse (p < 0.005). Cisplatin and etoposide (VP‐16) appeared to be the most active chemotherapeutic agents. The authors conclude that some patients with histologically benign ependymoma at first relapse may benefit from aggressive therapy, with occasional long‐term, progression‐free survival possible. Patients with malignant lesions, or patients who relapse a second time, are less likely to benefit from conventional therapy for a significant period of time.