Myxoma of bone involving the orbit.
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Biomedical subjects
Publications and source records attributed to B S Carson.
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Pulse oximetry is a valuable, noninvasive technique for assessing oxygen saturation that has gained wide clinical acceptance despite little available information concerning normal values in the newborn, especially at an altitude different than sea level. We performed serial pulse oximetry studies on 150 term, appropriate-weight-for-gestational-age, clinically healthy infants at an altitude of 1610 m (5280 ft) at 24 to 48 hours, 1 month, and 3 months of age to define a reference range for oxygen saturation as a guideline in clinical care. We found that mean oxygen saturation at 24 to 48 hours of age is 92% to 93% and varies little with infant activity. With increasing postnatal age, there is a tendency for increased oxygen saturation during the awake states to 93% to 94%, while oxygen saturation during sleep stays the same or even decreases slightly. The lower end of the reference range (2 SDs below the mean) is as low as 85% during feeding at 24 to 48 hours of age, and as low as 86% during quiet sleep at 1 and 3 months of age, with 88% to 89% the lower limit in other activities at all ages.
Neuraxis radiation therapy (RT) for primary intracranial tumors is associated with major late effects if administered to very young children. To control residual tumor and to delay RT, we treated eight young children (median age 6.5 months) with primary central nervous system (CNS) tumors using combination chemotherapy: cisplatin, 20 mg/M2/day plus VP-16, 75 mg/M2/day i.v. for 5 days, given q. 3-6 weeks for 8 cycles. The tumors were medulloblastoma (one), malignant ependymoma (two), primitive neuroectodermal tumor PNET (two), malignant glioma (two), astrocytoma (one). Six had measurable disease; three had positive cerebrospinal fluid (CSF) cytopathology. All patients with measurable tumor had initial objective responses (three) complete response [CR], one partial response [PR], two minor response [MR], including cytopathology (three CR of three) and metastatic deposits (two CR of two). One patient relapsed during chemotherapy. Median time to disease progression was 17.5 months; median survival was 34 months. Three patients, none of whom received RT, have prolonged progression-free intervals of 47-67 months to date. Neurodevelopmental progress continued during and after chemotherapy. Chemotherapy toxicity was mild. Median neutrophil nadir was 312/mm3, platelets 72,000. Fever during neutropenia occurred in six of 61 courses. Moderate high-frequency auditory losses were detected in three patients, and mild renal injury (GFR less than 70 ml/min) was detected in two of seven evaluable children. This pilot study demonstrates the apparent efficacy and mild toxicity of 5 day courses of cisplatin plus VP-16, with delayed RT, in young children with CNS neoplasms. A POG treatment protocol that incorporates cisplatin plus VP-16 is evaluating primary chemotherapy with delayed radiotherapy in larger numbers of pediatric brain tumor patients.
Sixteen patients with recurrent childhood brain tumors were treated with intravenous cisplatin, cytosine arabinoside and etoposide (PAE), daily for three days every three to four weeks. Objective responses were observed in 6 of 15 evaluable patients and an additional six patients had stable disease for greater than 6 months. The tumor-specific response rate for astrocytoma/glioma was 3 of 7 and for medulloblastoma was 2 of 4. The mean progression-free interval was 11.0 months and the hazard rate for progression was 0.085 per patient-month of observation. The most common toxicities were neutropenia and thrombocytopenia. Clinically significant ototoxicity was identified in 7 patients. The activity of PAE chemotherapy for recurrent childhood brain tumors warrants further investigation.
The sublabial transseptal transsphenoidal approach provides excellent exposure for pituitary surgery in adults. However, sellar lesions in children are more difficult to approach because of the smaller size of the pyramidal aperture and because of the limited pneumatization of the sella. We resurrected the transpalatal transsphenoidal approach for the treatment of sellar lesions in three children (one adenoma and two cystic craniopharyngiomas). The advantages of this approach were a short working distance and a wide exposure that permitted extensive drilling of the poorly pneumatized sphenoid bone under direct vision. The technique successfully provided exposure for management of the sellar pathology in all three cases and resulted in no nasal or palatal defects.
Many aspects of pediatric neuro-oncology including epidemiology, diagnosis and treatment are covered by this review. Important advances in the treatment of hydrocephalus and craniosynostosis are also reviewed. A number of congenital conditions involving the cranium and spinal cord are analyzed with respect to optimal treatment and new experimental data are highlighted. Advances in the treatment of pediatric intracranial and extracranial vascular anomalies are reviewed and advances in the understanding of head trauma are emphasized. Finally, a couple of the technical advances in pediatric neurosurgery are highlighted.
Topical application of dexamethasone to brain tumour bed retains its biological effectiveness in controlling peritumoural brain oedema, possibly through the oncolytic mechanism (cytostatic effect). The question rises, whether the systemic administration of dexamethasone can be duplicated with dexamethasone topically to the brain tumour bed.
Eighty-five medically intractable trigeminal neuralgia patients treated by percutaneous retrogasserian glycerol rhizotomy (PRGR) were followed for 6 to 54 months. The median time to recurrence of symptoms refractory to medical therapy and requiring further intervention was 3 years (by Kaplan-Meier survival analysis). The median time to recurrence of symptoms requiring some form of medical treatment was 2 years. Following repeat PRGR for recurrent symptoms, the median time to recurrence was 1 year. Univariate log rank statistics and multivariate Cox proportional hazards modeling revealed significant associations between favorable outcome and female sex, absence of atypical features or associated cluster headache symptoms, success of prior carbamazepine therapy, duration of symptoms, and cerebrospinal fluid return during the procedure. A scoring system using prognostic factors has been developed based upon these findings. Assessment of published studies of PRGR and of other treatments for trigeminal neuralgia is made difficult by the variety of outcome measures employed and variable follow-up intervals. The present study attempts to address these issues by definition of endpoints, statistical analysis of the data, and identification of important prognostic factors in a manner useful to the clinician.
The simultaneous presentation of clinically symptomatic anal anomalies and roentgenographically demonstrated sacral dysgenesis should alert the pediatric surgeon to investigate for the presence of a presacral malformation. We report on such a case to illustrate a new radiographic technique that facilitates diagnosis and management of complex congenital malformations. A 1-day-old white boy presented with anal stenosis, a scimitar-shaped sacrum, and large anterior and posterior meningoceles. In addition, a distinct presacral tumor--a teratoma--was identified. These malformations were identified utilizing metrizamide myelography and three-dimensional reconstruction computed tomography (CT) scanning. The meningoceles and a tethered cord were successfully corrected utilizing a posterior approach. A diverting colostomy was performed and subsequently taken down. Two years postoperatively, the patient continues to do well. This case demonstrates that this triad of anomalies (presacral mass, sacral dysgenesis, and anorectal malformation), once considered, can be safely detected with modern radiologic techniques and can be expeditiously corrected during infancy before further deterioration occurs.
Occipitally joined craniopagus Siamese twins were separated with the use of cardiopulmonary bypass and hypothermic circulatory arrest. The 7-month-old infants shared a large sagittal venous sinus that precluded conventional neurosurgical approach because of risk of exsanguination and air embolism. After craniotomy and preliminary exposure of the sinus, each twin underwent sternotomy and total cardiopulmonary bypass with deep hypothermia. Hypothermic circulatory arrest allowed safe division and subsequent reconstruction of the sinus remnants. Several unusual problems were encountered, including transfusion of a large blood volume from one extracorporeal circuit to the other through the common venous sinus, deleterious warming of the exposed brain during circulatory arrest, and thrombosis of both pump oxygenators. Both infants survived, although recovery was complicated in each by neurologic injury, cranial wound infection, and hydrocephalus. This case demonstrates the valuable supportive role of cardiopulmonary bypass and hypothermic circulatory arrest in the management of complex surgical problems of otherwise inoperable patients.
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Seventy-two years after Dandy and Blackfan's initial description of the Dandy-Walker syndrome, controversies still exist regarding the precise methods of treatment for this disorder. One potential complication of simple lateral ventricular shunting is upward herniation of the posterior fossa cyst, which at times can result in precipitous clinical deterioration. We present three examples of posterior fossa cysts with upward herniation and review the pertinent literature. We advocate contrast studies to determine whether aqueductal communication exists, before shunting is performed, as well as close follow-up of the shunted child.
A thermoplastic helmet was designed to allow the use of the Leksell stereotactic frame in infants.
A 5-year-old child typical clinical features of neurofibromatosis presented with a history of suspected basilar meningitis and CT findings of enlarged optic nerves and an expanding left cavernous sinus mass. CSF cytologies and meningeal biopsy were unremarkable. At craniotomy, a mass confluent with the left trigeminal nerve was resected which had histologic characteristics of a nerve sheath tumor but was GFAP (glial fibrillary acidic protein) stain positive. Postmortem examination, 1 month following surgical resection, demonstrated a clinically unsuspected primary thoracic spinal cord astrocytoma with dissemination throughout the subarachnoid space, invasion of the trigeminal nerve and encasement of other cranio-spinal nerves. This unusual case emphasizes the occurrence of leptomeningeal spread in a clinically silent spinal cord glioma and the diagnostic value of immunohistochemistry.
Lipomas rarely occur intracranially. Moreover, the cerebellopontine angle is one of the more unusual sites of such hamartomas. Of the 11 reported cases, all but three caused symptoms related to compression of the cranial nerves in the cerebellopontine angle. Only three separate cases have been studied by computed tomography, and in one the fat density was not recognized. This report deals with the clinical presentations, surgical management, and radiologic assessment of these lesions.
Respiratory morbidity in term neonates is an important complication of elective cesarean delivery. The effect of preceding labor on the incidence and severity of respiratory morbidity in two comparable groups of neonates, 107 with and 80 without labor and with no predisposing factors to respiratory morbidity, was evaluated. Transient tachypnea of the newborn accounted for the majority of cases in term neonates. Respiratory morbidity occurred less frequently in neonates delivered after the onset of labor compared with those delivered before labor (11.2 versus 30%, P less than .002). The risk of respiratory morbidity decreased 1.5 times for each week of advancing gestational age. The presence of labor significantly reduced the risk of respiratory morbidity, independently of gestational age (P less than .03), and disease was less severe in neonates born during labor. Awaiting the onset of labor appears to be beneficial in preventing respiratory morbidity in term neonates delivered by elective cesarean section.