Tandem pediatric neurosurgery: treatment of synostosis and intractable epilepsy. Illustrative case.

BACKGROUND: Hemispherectomy is a surgical procedure reserved for hemispheric intractable epilepsy. Sagittal craniosynostosis is a congenital disorder treated with open or endoscope-assisted approaches for synostosis correction. These procedures are not commonly performed in the same setting. OBSERVATIONS: In this report, the authors present a 6-month-old female with sagittal craniosynostosis, hemimegalencephaly, and intractable epilepsy who underwent a left hemispherotomy with open sagittal synostosis correction followed by cranial molding orthosis therapy. LESSONS: The report highlights the technical nuances of the procedure, but also discusses the possible genetic disorder responsible for both conditions, megalencephaly-capillary malformation syndrome.

Historical Perspectives on the Management of Craniosynostosis.

The history of craniofacial surgery is one of many fundamental advances by monumental figures. Although craniosynostosis has been known to exist for multiple centuries, modern management has evolved over roughly the last century. An overview of early history, early scientific exploration, the advancement of surgical treatment of craniofacial deformities and the current state of craniosynostosis management is discussed. To fully appreciate the evolution of craniosynostosis surgery, one must understand the gradual advancements that have brought the specialty to this modern era.

Modern Therapy for Spinal and Paraspinal Ewing Sarcoma: An Update of the University of Florida Experience.

PURPOSE: In 2010, we published a comprehensive review of our institutional outcomes about treating children with spinal and paraspinal Ewing sarcoma using photon therapy. Multimodality therapy was associated with fair disease control but also with serious toxicity, including a 37% rate of grade 3 or greater toxicity. We therefore sought to assess our more recent experience about treating children with more modern technology and treatment regimens. METHODS AND MATERIALS: Between 2010 and 2021, 32 pediatric patients with nonmetastatic spinal and paraspinal Ewing sarcoma were treated at University of Florida and enrolled in a retrospective outcome study. Median age at diagnosis was 9.8 years (range, 2.1-21.8 years). Within the cervical, thoracic, and lumbar spine regions, 3, 22, and 7 tumors arose, respectively. Median maximum tumor diameter was 5 cm (range, 3-19 cm). At diagnosis, 28 of 32 patients had motor, bowel, or bladder deficits. Chemotherapy was delivered according to contemporary North American and European interval-compressed regimens. Before radiation therapy, 14 patients underwent gross total resection, whereas 18 underwent a biopsy or subtotal resection with cord decompression. All patients were treated with proton therapy; 6 with hardware stabilization also received a component of intensity modulated photon therapy. Median prescription dose was 50.4 gray relative biological effectiveness (GyRBE; range, 45-55.8 GyRBE). Median maximum dose to the spinal cord was 50.2 GyRBE (range, 0-54.9 GyRBE). RESULTS: With a median follow-up of 4.1 years (range, 0.7-9.4 years), the 5-year local control, progression-free survival, and overall survival rates were 92%, 79%, and 85%, respectively. Ten of 30 living patients have residual motor, bowel, or bladder deficits. Overall, 22% of patients experienced Common Terminology Criteria for Adverse Events grade 3 late toxicity related to multimodality treatment: kyphosis (n = 4), esophagitis (n = 2), and chronic kidney disease (n = 1). No patients developed grade 4 or greater toxicity, new neurologic deficits, or second malignancy. CONCLUSIONS: Modern treatment advances may offer an improved therapeutic ratio for pediatric patients with spinal and paraspinal Ewing sarcoma. With appropriate management, most patients can be cured with recovery of long-term neurologic function and modest side effects.

Prioritizing Pediatricians' Neurosurgical Education: Results From a National Survey of Primary Care Pediatricians.

Introduction. We surveyed nonretired American Academy of Pediatrics-member US pediatricians regarding common neurosurgical conditions, identifying specific areas of focus in education. Methods. Data were acquired via self-administered electronic questionnaire. Results. Of 505 total respondents, 56% reported neurology was not a required residency rotation, and 86% had diagnosed craniosynostosis, plagiocephaly, or macrocephaly. Craniosynostosis can mostly be diagnosed by physical examination alone, but almost 50% reported relying on skull X-rays. Fifty-four percent reported diagnosing ocular surface disease (OSD; with 15% to 40% not screening an infant despite well-established cutaneous markers). Seventy-four screened OSD in a patient with sacral dimple. Ninety-seven percent reported treating concussion, but nearly 25% did not manage these patients alone. Two out of 3 patients indicated head injury as most important for continuing education. Conclusion. Improved education for craniosynostosis, OSD, head injury, and concussion management are important for earlier diagnosis, management, and referral of some disorders, while decreasing resource utilization in others. These results should be used when considering pediatrician educational programs.

Outcomes Following Proton Therapy for Pediatric Low-Grade Glioma.

PURPOSE: Dosimetric studies show that proton therapy can reduce the low/intermediate radiation dose to uninvolved tissue in children with low-grade glioma (LGG). For this reason, LGG is the fourth most common pediatric tumor treated with proton therapy, yet clinical outcome data on efficacy and toxicity are limited. METHODS AND MATERIALS: We reviewed the medical records of 174 children (≤21 years old) with nonmetastatic LGG enrolled on a prospective protocol and treated with proton therapy between 2007 and 2017. We assessed clinical outcomes and toxicity and analyzed patient, tumor, and treatment-related variables. RESULTS: The median age was 10.2 years (range, 2-21). Fifty-eight percent of tumors were World Health Organization grade 1 and 30% were grade 2; 12% were diagnosed on imaging characteristics alone. The most common histology was pilocytic astrocytoma (47%). The most common tumor subsites were diencephalon/optic pathway (52%), caudal brainstem (16%), and cerebellum (13%). Forty-two percent received chemotherapy before radiation therapy. The median follow-up was 4.4 years. The 5-year actuarial rates of local control, progression-free survival, and overall survival were 85% (95% confidence interval [CI], 78%-90%), 84% (95% CI, 77%-89%), and 92% (95% CI, 85%-95%), respectively. On univariate analysis, brainstem/spinal cord tumor location (62% vs 90% elsewhere) and dose <54 GyRBE (67% vs 91% for 54 GyRBE) were associated with inferior local control (P < .01 for both). Twenty-two patients (12.6%) experienced acute nausea or vomiting requiring ondansetron; 2 patients (1.1%) required corticosteroids. Serious toxicities (4% of patients) included brainstem necrosis requiring corticosteroids (n = 2), symptomatic vasculopathy (n = 2), radiation retinopathy (n = 1), epilepsy (n = 1), and death from radiation-induced high-grade glioma (n = 1). Thirty-nine patients (22%) developed new-onset central hormone deficiency. Pseudoprogression was observed in 32.1%. CONCLUSIONS: Compared with modern photon series, proton therapy reduces the radiation dose to developing brain tissue, diminishing acute toxicities without compromising disease control.

Outcomes following proton therapy for pediatric ependymoma.

BACKGROUND: Proton therapy can reduce the low and intermediate radiation dose to uninvolved brain tissue in children with intracranial ependymomas, which may improve functional outcomes and reduce second malignancies in survivors. Accordingly, ependymoma has become the most common pediatric tumor treated with proton therapy, yet data on efficacy and toxicity are limited. MATERIAL AND METHODS: Between June 2007 and February 2017, 179 children (≤21 years old) with nonmetastatic grade II/III intracranial ependymoma received proton therapy at our institution. Median age, 3.5 years (range, 0.7-21); 58% were male. Most (66%) tumors were in the posterior fossa and classified as WHO grade III (67%). 27% underwent multiple operations to maximize the extent of resection; ultimately 85% had a gross total or near total tumor resection before radiotherapy. 33% received preradiation chemotherapy. Median radiation dose in children ≤3 years old, 54 Gy(RBE). Most (>90%) children over 3 years old received 59.4 Gy(RBE). Patient and treatment variables were assessed for correlation with disease control. RESULTS: Median follow-up, 3.2 years. 3-year local control, progression-free survival, and overall survival rates were 85%, 76%, and 90%, respectively. First site of progression was local, metastatic, or simultaneous in 14, 17 and 6 patients, respectively. On multivariate analysis, subtotal resection was associated with inferior local control (67% vs. 88%; p ≤ .01) and progression-free survival (59% vs. 79%; p < .05). Male sex was associated with inferior progression-free (67% vs. 87%; p< .05) and overall survival (84% vs. 99%; p < .01). The 3-year CTCAE grade 2 + brainstem toxicity rate was 5.5% (95% CI: 2.9-10.2), including 1 grade 5 toxicity. CONCLUSIONS: This series of proton therapy for pediatric intracranial ependymoma demonstrates disease control comparable to photon series without unexpected toxicity. Subtotal resection and male sex were associated with inferior disease control. Additional follow-up to quantify the expected reductions in late toxicity with proton therapy is ongoing.

Baclofen pump catheter leakage after migration of the abdominal catheter in a pediatric patient with spasticity.

The authors report an unusual case of intrathecal baclofen withdrawal due to the perforation and subsequent leakage of a baclofen pump catheter in a patient with spastic cerebral palsy. A 15-year-old boy underwent an uncomplicated placement of an intrathecal baclofen pump for the treatment of spasticity due to cerebral palsy. After excellent control of symptoms for 3 years, the patient presented to the emergency department with increasing tremors following a refill of his baclofen pump. Initial evaluation consisted of radiographs of the pump and catheter, which appeared normal, and a successful aspiration of CSF from the pump's side port. A CT dye study revealed a portion of the catheter directly overlying the refill port and extravasation of radiopaque dye into the subfascial pocket anterior to the pump. During subsequent revision surgery, a small puncture hole in the catheter was seen to be leaking the drug. The likely cause of the puncture was an inadvertent perforation of the catheter by a needle during the refilling of the pump. This case report highlights a unique complication in a patient with an intrathecal baclofen pump. Physicians caring for these patients should be aware of this rare yet potential complication in patients presenting with baclofen withdrawal symptoms.

Delayed synostoses of uninvolved sutures after surgical treatment of nonsyndromic craniosynostosis.

BACKGROUND: Craniosynostosis causes significant cranial deformity in the pediatric population. Open and endoscopic-assisted surgeries have led to increasingly successful management of this condition. Following surgical reconstruction, subsequent development of postnatal synostosis of previously patent sutures have been described and noted to be most frequently associated with multisuture synostosis patients with syndromic diagnoses. Very rarely, postsurgical new sutural fusion has been identified in nonsyndromic patients who initially present with isolated single-suture synostosis. The purpose of this study was to evaluate the incidence of new synostosis among patients who had undergone craniosynostosis reconstruction with either the open or endoscopic technique. METHODS: Patients undergoing open and endoscopic surgery for nonsyndromic craniosynostosis were reviewed. Preoperative and postoperative imaging and clinical information were reviewed, and cases showing progressive cranial deformity were identified. RESULTS: Three (2.1%) of 145 patients undergoing open craniosynostosis surgery and 2 (1.7%) of 121 patients undergoing endoscopic surgery developed delayed fusion of an additional suture during follow-up. This was identified at a median of 16.4 months after initial surgery in the open group and 15.25 months after surgery in the endoscopic group. In patients undergoing open surgery, each patient developed new sagittal synostosis after initial presentation of coronal synostosis in 1 patient and metopic synostosis in 2 patients. In patients undergoing endoscopic surgery, each patient developed new coronal synostosis after sagittal repair. CONCLUSIONS: Management of craniosynostosis has evolved over time with increasing availability of effective and safe treatments. During long-term follow-up, a small number of patients may develop premature closure of a different suture that did not undergo surgical manipulation. In our case, series, we identified 3 patients undergoing open surgery and 2 patients undergoing endoscopic surgery for nonsyndromic, single-suture craniosynostosis. This finding supports the necessity of long-term clinical follow-up and the utility of delayed imaging when clinical suspicion indicates.

Direct cardiac ventriculoatrial shunt: technical note.

There are a number of choices for placement of the distal catheter during ventricular shunting for hydrocephalic patients. In very rare instances, patients with multiple revisions can no longer have their shunt placed in the routine locations. We describe the placement of the distal catheter into the atrium through direct cardiac access, a technique described decades ago but rarely needed in clinical practice. This can be a useful location in the limited number of patients who have exhausted other more routine locations.

Hemangiomas of the brachial plexus: a case series.

OBJECTIVE: Hemangiomas of the brachial plexus are very rare, and there has not been a collection of multiple cases published in the literature to date. Extraneural brachial plexus hemangiomas typically present with similar signs and symptoms as nerve sheath tumors, including pain, paresthesia, and occasionally weakness, in addition to nonspecific imaging findings, making their diagnosis difficult. Exploratory surgery can lead to significant bleeding and nerve injury when a hemangioma or an associated aneurysm is encountered intraoperatively. We present 5 cases of extraneural hemangiomas causing brachial plexopathy, including pre-, intra-, and postoperative decision making, with an emphasis on diagnostic and management issues as well as outcomes. METHODS: A retrospective review was performed of 5 patients who underwent surgery at a university teaching hospital between 1995 and 2007 for exploration of brachial plexus lesions that were confirmed to be hemangiomas at pathological examination. RESULTS: All 5 patients presented with findings on history, physical examination, imaging, and electromyography suggesting a diagnosis of nerve sheath tumor. Two patients had biopsies (1 needle, 1 open), both of which were nondiagnostic. Three patients underwent digital subtraction angiography with successful preoperative embolization. Each patient had a complete or a radical subtotal tumor resection, and all were intact neurologically after surgical resection. Pathological evaluation identified 3 venous hemangiomas, 1 hemangioma with arteriovenous malformation features, and 1 Masson hemangioma associated with a large aneurysm. CONCLUSION: Extraneural hemangiomas of the brachial plexus are very rare, but a high index of suspicion and appropriate preoperative evaluation, including angiography with the option for embolization, can result in decreased intraoperative hemorrhage and better patient outcomes.

Thalamic deep brain stimulation for midbrain tremor secondary to cystic degeneration of the brainstem.

OBJECTIVE: Tremor resulting from damage to midbrain structures is poorly understood and often difficult to treat. The authors report a case of cystic degeneration of the brainstem with resultant Holmes-like tremor which was successfully treated using a stimulating electrode placed in the contralateral ventralis intermedius nucleus (VIM) of the thalamus. CLINICAL PRESENTATION: A 31-year-old man presented with a multilobulated, multiseptated lesion of the upper brainstem diagnosed after subacute onset of headaches. The patient subsequently developed an incapacitating left-upper-extremity tremor refractory to medical treatment. INTERVENTION: The patient underwent implantation of a deep brain stimulator in the VIM with symptomatic and functional improvement. CONCLUSIONS: Deep brain stimulation is an effective and safe intervention for tremor of unusual etiology. Electrode placement should be based on an understanding of the structure-function relationships underlying the various and distinct types of tremor.

A novel neuroprosthetic interface with the peripheral nervous system using artificially engineered axonal tracts.

OBJECTIVE: We have previously described a technique developed in our laboratory to create transplantable living axon tracts of several centimeters in length. In this paper, we describe how these engineered neural tissue constructs can be used to create a novel neuroelectrical interface with the regenerating peripheral nervous system, to potentially enable afferent and efferent communications with prosthetic devices. METHODS: Using continuous mechanical tension, we have generated axon tracts of up to 10 cm in length, spanning two populations of neurons in vitro. We have now adapted this stretch-growth paradigm to include a mechanically compliant multi-electrode array that is attached to one of the neuron populations. Once the desired axon length has been reached, the neuroelectrode construct is completely embedded in a supportive hydrogel matrix and affixed to the transected sciatic nerve. RESULTS: Building upon our previous work with peripheral nerve repair, we have designed our neural interface to ensure transplant stability and firm attachment to the electrode array substrate. DISCUSSION: Our preliminary findings indicate that the interface not only maintains its orientation, but also is conducive to host nerve ingrowth. Our ongoing analysis seeks to characterize transplanted neuronal survival, synaptic integration, and functional connectivity. This research provides an opportunity to evaluate an entirely new approach in restoring motor and sensory functions of patients with peripheral nerve damage.

Clinical problem-solving: brachial plexus closed injury and reconstruction.

OBJECTIVE: Current management of severe brachial plexus injury has undergone recent modifications, and surgical options have expanded. METHODS: The case of a man with a severe closed brachial plexus injury resulting from a motorcycle accident is presented. The patient is found to have upper root avulsions that deprive him of function in the proximal arm. RESULTS: Pre-, intra-, and postoperative decision making is reviewed by an expert in peripheral nerve surgery. Attention is paid to both diagnosis and management. A brief review of the literature pertaining to these points follows. CONCLUSION: The recent expansion of surgical options for the management of severe brachial plexus injury has introduced significant controversy into this field.