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Open access

Approaches to ventriculoperitoneal shunt scalp erosion: countersinking into the calvarium. Illustrative case

Denise Brunozzi, Melissa A LoPresti, Jennifer L McGrath, and Tord D Alden

BACKGROUND

Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors.

OBSERVATIONS

The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing.

LESSONS

Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 22 (Nov 2023)

Open access

Cranial vault suspension for basilar invagination in patients with open cranial sutures: technique and long-term follow-up. Illustrative case

Christopher B Cutler, Daphne Li, and John R Ruge

BACKGROUND

Hajdu-Cheney syndrome (HCS) is an extremely rare genetic disorder characterized by severe osteoporosis, scoliosis, and persistent open cranial sutures (POCSs). Neurological complications include hydrocephalus, Chiari I malformations, and basilar invagination (BI). Surgical intervention in HCS is challenging due to severe osteoporosis, ligamentous laxity, POCSs, and extreme skeletal deformities. Herein, the authors present a case of BI repair in a patient with HCS and POCSs, requiring a novel technique of cranial vault suspension, with long-term follow-up.

OBSERVATIONS

A 20-year-old female with HCS and progressive symptomatic BI, initially managed with posterior fossa decompression and occipital to cervical fusion, subsequently required cranial vault expansion due to symptomatic shifting of her cranium secondary to POCS. This custom construct provided long-term stabilization and neurological improvement over a follow-up duration of 9.5 years. A literature review performed revealed three other cases of surgical intervention for BI in patients with HCS and clinicopathological characteristics of each case was compared to the present illustrative case.

LESSONS

POCSs in patients with BI complicate traditional surgical approaches, necessitating more invasive techniques to secure all mobile cranial parts for optimal outcomes. Using this cranial vault suspension and fusion technique results in lasting neurological improvement and construct stability.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 22 (Nov 2023)

Open access

Myasthenia gravis in a pediatric patient with Lennox-Gastaut syndrome following responsive neurostimulation device implantation: illustrative case

David A Zuckerman, Cameron P Beaudreault, Carrie R Muh, Patricia E McGoldrick, and Steven M Wolf

BACKGROUND

Myasthenia gravis (MG) is an autoimmune disorder in which the postsynaptic acetylcholine receptor of the neuromuscular junction is destroyed by autoantibodies. The authors report a case of MG in a pediatric patient who also suffered from Lennox-Gastaut syndrome (LGS) and is one of a limited number of pediatric patients who have undergone placement of a responsive neurostimulation (RNS) device (NeuroPace).

OBSERVATIONS

A 17-year-old female underwent placement of an RNS device for drug-resistant epilepsy in the setting of LGS. Five months after device placement, the patient began experiencing intermittent slurred speech, fatigue, and muscle weakness. Initially, the symptoms were attributed to increased seizure activity and/or medication side effects. However, despite changing medications and RNS settings, no improvements occurred. Her antiacetylcholine receptor antibodies measured 62.50 nmol/L, consistent with a diagnosis of MG. The patient was then prescribed pyridostigmine and underwent a thymectomy, which alleviated most of her symptoms.

LESSONS

The authors share the cautionary tale of a case of MG in a pediatric patient who was treated with RNS for intractable epilepsy associated with LGS. Although slurred speech, fatigue, muscle weakness, and other symptoms might stem from increased seizure activity and/or medication side effects, they could also be due to MG development.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 22 (Nov 2023)

Open access

Cervical corpectomy in a pediatric patient with chondrodysplasia punctata and C5 dysplasia with spinal cord compression: illustrative case

Nirali P Patel, Mark W Youngblood, Melissa A LoPresti, and Tord D Alden

BACKGROUND

Chondrodysplasia punctata (CDP) describes skeletal dysplasia secondary to a variety of genetic underpinnings characterized by cartilaginous stippling from abnormal calcium deposition during endochondral bone formation. Approximately 20%–38% of patients with CDP have cervical spine abnormalities, resulting in stenosis and cord compression. However, approaches to management differ among patients.

OBSERVATIONS

The authors present an 18-year-old male with a known history of CDP and cervical kyphosis with worsening paresthesias and increased spasticity. Imaging confirmed dysplastic C4 and C5 vertebra with focal kyphosis, bony retropulsion, spinal cord compression, and myelomalacia. To treat the stenosis and deformity, the patient underwent C4 and C5 vertebrectomies with C3 to C6 anterior fusion with resolution of symptoms.

LESSONS

Despite many CDP patients having cervical deformities with spinal cord compression and associated neurological symptoms, there is a paucity of data on surgical management and outcomes. There are only scattered reports, and most authors recommend initial conservative management because of the high risk of operative morbidity and mortality secondary to comorbidities. When surgery is performed, long-term follow-up is recommended because of the high rates of progression of deformity, requiring subsequent operations. The authors hope that their experience adds to the literature describing the surgical management of cervical deformities in these patients.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 21 (Nov 2023)

Open access

Symptomatic spinal arachnoid cyst with spastic diplegia secondary to cerebral palsy: illustrative case

Andrew Guillotte, Abdul-Rahman Alkiswani, Kathryn A Keeler, and Michael D Partington

BACKGROUND

Selective dorsal rhizotomy (SDR) can improve the spastic gait of carefully selected patients with cerebral palsy. Spinal arachnoid cysts are a rare pathology that can also cause spastic gait secondary to spinal cord compression.

OBSERVATIONS

The authors present an interesting case of a child with cerebral palsy and spastic diplegia. He was evaluated by a multidisciplinary team and determined to be a good candidate for SDR. Preoperative evaluation included magnetic resonance imaging (MRI) of the spine, which identified an arachnoid cyst causing spinal cord compression. The cyst was surgically fenestrated, which provided some gait improvement. After recovering from cyst fenestration surgery, the patient underwent SDR providing further gait improvement.

LESSONS

SDR can be beneficial for some patients with spastic diplegia. Most guidelines do not include spinal MRI in the preoperative evaluation for SDR. However, spinal MRI can be beneficial for surgical planning by localizing the level of the conus. It may also identify additional spinal pathology that is contributing to the patient’s spasticity. In rare cases, such as this one, patients may benefit from staged surgery to address structural causes of spastic gait prior to proceeding with SDR.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 20 (Nov 2023)

Open access

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

Ogechukwu Ariwodo, Douglas R Nordli III, Nathan J Ranalli, and Alexandra D Beier

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.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 20 (Nov 2023)

Open access

Endoscopic placement of a triventricular stent for complex hydrocephalus and isolated fourth ventricle: illustrative case

V. Jane Horak, Beste Gulsuna, Melissa A LoPresti, and Michael DeCuypere

BACKGROUND

Hydrocephalus is commonly encountered in pediatric neurosurgery. The etiology is diverse, and complexity in management increases in patients with loculated or trapped ventricles. The authors sought to examine a treatment option of endoscopic placement of a triventricular stent in a pediatric patient with complex hydrocephalus and a trapped fourth ventricle.

OBSERVATIONS

In this case, the authors present the treatment of complex hydrocephalus with a trapped fourth ventricle in a pediatric patient using endoscopic placement of a triventricular aqueductal stent. The patient had a complex neurosurgical history, which included over 15 surgeries for shunted hydrocephalus. This case highlights the unique approach used, and the authors discuss surgical nuances of the technique, as well as learning points.

LESSONS

Complex hydrocephalus can be difficult to manage because patients often have multiple catheters, loculated or trapped ventricles, and extensive surgical histories. Endoscopic placement of a triventricular stent can decrease shunt system complexity, restore normal cerebrospinal fluid pathway circulation across the cerebral aqueduct, and promote communication between the ventricles. The authors’ treatment modality resulted in the successful resolution of the trapped fourth ventricle and symptomatic improvement in hydrocephalus.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 19 (Nov 2023)

Open access

Middle meningeal artery embolization for refractory chronic subdural hematoma in a pediatric victim of nonaccidental trauma: illustrative case

Zsombor T Gal and Alfred P See

BACKGROUND

Chronic subdural hematoma (cSDH) in children is associated with several etiologies, one of which is nonaccidental trauma (NAT). Performed alone or as an adjunct to surgical intervention, middle meningeal artery (MMA) embolization may be effective in resolving and preventing the recurrence of cSDH in adult patients. However, MMA embolization for pediatric cSDH has not been adequately evaluated, and there exists no consensus on the appropriate selection of pediatric patients for this minimally invasive procedure. Appreciating the variable underlying pathophysiology of pediatric cSDH will enable the development of guidelines for patient selection.

OBSERVATIONS

A 14-month-old female patient presented with depressed consciousness, seizure-like activity, and apneic episodes. She was diagnosed with bilateral subacute on chronic SDH associated with NAT, and imaging revealed diffuse brain atrophy. Despite undergoing burr hole drainage, she developed refractory cSDH and was treated with bilateral MMA embolization. At her 10-month follow-up, magnetic resonance imaging revealed a decrease in the size of the subdural collections, and she was tolerating feeds, at her neurological baseline, and seizure-free.

LESSONS

Infants and young children with symptomatic cSDH secondary to NAT, whose tissue dynamics are reminiscent of cSDH in elderly patients with diffuse brain atrophy, may derive the most benefit from MMA embolization.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 17 (Oct 2023)

Open access

Clinical, imaging, and molecular features of radiation-induced glioblastomas developing more than 20 years after radiation therapy for intracranial germinomatous germ cell tumor: illustrative cases

Yoshihiro Tsukamoto, Manabu Natsumeda, Haruhiko Takahashi, Asuka Ueno, Kiichi Sakai, Kazuki Shida, Hiroki Seto, Taiki Saito, Satoshi Shibuma, Yoko Nakayama, Yuki Tanaka, Toshimichi Nakano, Atsushi Ohta, Katsuya Maruyama, Masayasu Okada, Takeyoshi Eda, Yasuhiro Seki, Yuichirou Yoneoka, Hiroshi Shimizu, Kouichirou Okamoto, Akiyoshi Kakita, and Makoto Oishi

BACKGROUND

Germinomatous germ cell tumor is highly sensitive to chemoradiotherapy; patients are expected to survive for decades. Many radiation-induced malignant gliomas (RIMGs) occur >10 years after radiotherapy. Standard therapy for RIMGs has not been established because of the lesion’s rarity, the patient’s shorter survival period, and the risk of radiation necrosis by repeat radiation.

OBSERVATIONS

Two patients, a 32-year-old man and a 50-year-old man, developed glioblastomas more than 20 years after radiation monotherapy for germinoma with or without mature teratoma. The first patient showed a tumor in the left frontotemporal region with disseminated lesions and died 2 months after partial resection of the tumor without responding to the chemotherapy with temozolomide and bevacizumab. Methylation classifier analysis classified the pathology as closest to diffuse pediatric-type high-grade glioma, Rtk1 subtype. The second patient showed a tumor mass in the brainstem and left cerebellar peduncle, which worsened progressively during chemotherapy with temozolomide and bevacizumab. The tumor transiently responded to stereotactic radiotherapy with the CyberKnife. However, the patient died of RIMG recurrence-related aspiration pneumonia 11 months after the biopsy. Methylation classifier analysis classified the pathology as closest to infratentorial pilocytic astrocytoma.

LESSONS

Chemoradiotherapy may improve the survival of patients with RIMGs. Furthermore, molecular features may influence the clinical, locoregional, and pathological features of RIMG.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 16 (Oct 2023)

Open access

Novel combination of GammaTile cesium-131 brachytherapy with 5-aminolevulinic acid fluorescence–guided resection in the re-irradiation of pediatric recurrent high-grade glioma: illustrative case

Julian A. Gordon, Melisa Pasli, Cathleen M. Cook, Rainor Connor, Philip J. Boyer, Andrew W. Ju, K. Stuart Lee, Kathleen E. Knudson, and M. Sean Peach

BACKGROUND

Herein, the authors describe the successful utilization of 5-aminolevulinic acid (5-ALA) and the first case of GammaTile cesium-131 therapy in a pediatric patient with recurrent high-grade glioma. 5-ALA was utilized to optimize gross-total resection prior to GammaTile implantation. After conversion to an equivalent dose in 2-Gy fractions (EQD2), a composite was made of the GammaTile dose with the initial external beam radiotherapy. Two hypothetical plans consisting of a standard hypofractionated strategy for glioma reirradiation and a CyberKnife plan using GammaTile’s planning target volume were developed and likewise underwent EQD2 conversion and composite plan generation with the initial radiotherapy.

OBSERVATIONS

5-ALA was useful in achieving gross-total resection with no acute toxicity from the surgery or GammaTile irradiation. When compared with the hypothetical composite doses, GammaTile’s composite, axium point dose (D0.03cc) to the brainstem was 32.9 Gy less than the hypofractionated and the CyberKnife composite plans at 38.7 Gy and 40.2 Gy, respectively. The right hippocampus demonstrated a substantially reduced composite plan dose with GammaTile with a D0.03cc of 62.4 Gy versus 71.7 and 80.7 Gy for the hypofractionated and CyberKnife composite plans, respectively.

LESSONS

Utilization of 5-ALA and GammaTile therapy yielded clinically superior tumor debulking and effective radiotherapy dose localization with sparing of organs at risk, respectively.

In Journal of Neurosurgery: Case Lessons Volume 6 (2023): Issue 16 (Oct 2023)