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Open access

Epidural lipomatosis with foci of hemorrhage and acute compression of the spinal cord in a child with CLOVES syndrome: illustrative case

Dmytro Ishchenko, Iryna Benzar, and Andrii Holoborodko

BACKGROUND

Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, spinal/skeletal anomalies, and/or scoliosis (CLOVES) syndrome is the most recently described combined vascular anomaly characterized by congenital excessive growth of adipose tissue, vascular malformations, epidermal nevi, and skeletal deformities. This condition exhibits a significant variability in clinical manifestations and a tendency for rapid progression and affects extensive anatomical regions. Information regarding the association of epidural lipomatosis with low-flow venous lymphatic malformations is rare, with few reports in the literature.

OBSERVATIONS

The authors present a case of a 6-year-old girl who was admitted to the emergency department complaining of rapidly progressing weakness in her lower extremities and partial loss of sensation in the inguinal area. Radiologically, an extradural mass was identified at the T2–6 level, causing acute spinal cord compression. Urgent decompression and partial resection of the mass were performed. Despite satisfactory intraoperative hemo- and lymphostasis, postoperative lymphorrhea/seroma leakage was encountered as a delayed complication and was managed conservatively.

LESSONS

CLOVES syndrome is characterized by the combination of various clinical symptoms, not all of which are included in the abbreviation, as well as a progressively deteriorating course, the emergence of new symptoms, and complications throughout the patient’s life. This necessitates ongoing monitoring of such patients.

Open access

Phantom limb pain, traumatic neuroma, or nerve sheath tumor? Illustrative case

Patrick J Halloran, E. Antonio Chiocca, and Andres Santos

BACKGROUND

Phantom limb pain and traumatic neuromas are not commonly seen in neurosurgical practice. These conditions can present with similar symptoms; however, management of traumatic neuroma is often surgical, whereas phantom limb pain is treated with conservative measures.

OBSERVATIONS

A 77-year-old female patient with a long-standing history of an above-the-knee amputation experienced severe pain in her right posterior buttocks area for several years’ duration, attributed to phantom limb pain, which radiated down the stump of her leg and was treated with a variety of conservative measures. A recent exacerbation of her pain led to a prolonged hospitalization with magnetic resonance imaging of her leg stump, revealing a mass in the sciatic notch, at a relative distance from the stump. The anatomical location of the mass on the sciatic nerve in the notch led to a presumed radiological diagnosis of nerve sheath tumor, for which she underwent excision. At surgery, a neuroma of the proximal portion of the transected sciatic nerve that had retracted from the amputated stump to the notch was diagnosed.

LESSONS

Traumatic neuromas of transected major nerves after limb amputation should be considered in the differential diagnosis of phantom limb pain.

Open access

Intraoperative application of indocyanine green and temporary venous occlusion test to assess collateral flow during microvascular decompression for venous-related trigeminal neuralgia: illustrative case

Kentaro Fujimoto, Yosuke Akamatsu, Yasumasa Nishikawa, and Kuniaki Ogasawara

BACKGROUND

In microvascular decompression (MVD) for vein-related trigeminal neuralgia (TN), determining whether transection of the offending vein is safe can be challenging. Here, the authors present a case of vein-related TN successfully treated by sacrificing the offending vein on the basis of findings from indocyanine green (ICG) video angiography and a temporary venous occlusion test to assess the collateral flow of the offending vessel.

OBSERVATIONS

A 43-year-old man presented with TN, which had failed to respond to previous medical therapy. Gadolinium-enhanced magnetic resonance imaging (MRI) revealed that the transverse or superior petrosal vein was the offending vein. The patient underwent MVD. Because the transposition of the offending vein was anatomically challenging, a temporary vein occlusion test was performed using ICG video angiography. During and after temporary occlusion, bidirectional flow in the offending vein was observed, suggesting collateral flow even after vein occlusion. On the basis of these findings, the offending vein was transected, resulting in relief from pain without any complications. Postoperative MRI revealed no new lesions in the brainstem or the cerebellar hemisphere. The patient has been free from neuralgia for 6 months.

LESSONS

The temporary vein occlusion test under ICG video angiography was useful for evaluating collateral flow in the offending vein in TN.

Open access

Neurocysticercosis of the third ventricle: illustrative case

John Choi, Grace Cullen, Dawn Darbonne, Dennis Adams, Christina Coyle, Joseph Cooper, and Harminder Singh

BACKGROUND

Neurocysticercosis is a parasitic infection of the central nervous system. Cysts located in the ventricles, intraventricular neurocysticercosis (IVNCC), can cause symptoms of increased intracranial pressure and, if untreated, can be fatal. Neuroendoscopic removal of IVNCC is recommended as the first-line treatment.

OBSERVATIONS

The authors present the case of a healthy 30-year-old male originally from Mexico who presented with headaches and vomiting. He was found to have a cyst in the third ventricle on imaging, consistent with IVNCC. The authors successfully performed neuroendoscopic surgery with removal of the cyst en bloc.

LESSONS

A multidisciplinary team of neurosurgery and infectious disease specialists is recommended for successful management of patients with IVNCC. These patients typically require neuroendoscopic surgical removal for definitive treatment. In this case, the authors show surgery resulted in an effective cure without the need for antiparasitic medication and excellent long-term outcomes.

Open access

Successful resection of bilateral parafalcine meningioma with unilateral interhemispheric and contralateral transfalcine approach under nonintubated spontaneous breathing conditions: illustrative case

Seiichiro Hirono, Keisuke Kawano, Masato Ito, Kana Saito, Taiichiro Hayashida, and Yoshinori Higuchi

BACKGROUND

The best surgical approach for resecting bilateral parafalcine meningioma, as well as the optimal anesthesia and airway management for craniotomy in patients with interstitial pneumonia (IP) for preventing postsurgical exacerbation, remains unclear.

OBSERVATIONS

A 66-year-old female with a history of multiple relapses of IP underwent craniotomy for resection of a 4.5-cm bilateral parafalcine meningioma located just beneath the inferior sagittal sinus. To avoid mechanical ventilation or high-concentration oxygenation, the entire procedure was performed under nonintubated spontaneous breathing conditions with a supraglottic airway/laryngeal mask airway (SGA/LMA) device. Half of the tumor was resected using the ipsilateral interhemispheric approach, while the remaining half was resected using the contralateral transfalcine approach (CTA). No brain retractors were required. Preoperative embolization contributed toward reducing blood loss and surgery duration. During most of the operation, additional oxygen administration was not required. The postoperative course was uneventful, without exacerbation of the IP.

LESSONS

This case demonstrated the utility and feasibility of a unilateral interhemispheric approach combined with CTA for resection of a bilateral parafalcine meningioma. Additionally, this case provides an alternative method of airway and anesthesia management with an SGA/LMA device and nonintubated spontaneous breathing for the prevention of postoperative acute exacerbation of IP.

Open access

Cervicothoracic ventral-dorsal rhizotomy for bilateral upper-extremity hypertonia in cerebral palsy: illustrative case

Ryan Kelly, Hanna R Kemeny, Sunny Abdelmageed, Robin Trierweiler, Tim Krater, Melissa A LoPresti, and Jeffrey S Raskin

BACKGROUND

Management of medically refractory limb-specific hypertonia is challenging. Neurosurgical options include deep brain stimulation, intrathecal baclofen, thalamotomy, pallidotomy, or rhizotomy. Cervical dorsal rhizotomy has been successful in the treatment of upper-extremity spasticity. Cervical ventral and cervical ventral-dorsal rhizotomy (VDR) has been used in the treatment or torticollis and traumatic hypertonia; however, the use of cervicothoracic VDR for the treatment of upper-extremity mixed hypertonia is not well described.

OBSERVATIONS

A 9-year-old girl with severe quadriplegic mixed hypertonia secondary to cerebral palsy (CP) underwent cervicothoracic VDR. Modified Ashworth Scale scores, provision of caregiving, and examination improved. Treatment was well tolerated.

LESSONS

Cervicothoracic VDR can afford symptomatic and quality of life improvement in patients with medically refractory limb hypertonia. Intraoperative positioning and nuances in surgical techniques are particularly important based on spinal cord position as modified by scoliosis. Here, the first successful use of cervicothoracic VDR for the treatment of medically refractory upper-limb hypertonia in a pediatric patient with CP is described.

Open access

Posterior-only 2-level vertebrectomy and fusion in a medically complex patient with lumbar metastasis: illustrative case

Ryan Johnson, Annabelle Shaffer, Ashley Tang, Kathryn Tsai, Gina Guglielmi, and Paul M Arnold

BACKGROUND

Spinal metastases are commonly seen in patients with cancer and often indicate a poor prognosis. Treatment can include curative or palliative surgery, chemotherapy, and radiation therapy. The surgical approach varies widely on the basis of the affected region of the spine, the location of the tumor (anterior versus posterior), the goal of surgery, the health of the patient, and surgeon preference.

OBSERVATIONS

The authors present a case of a 68-year-old male with intractable lower-back pain and substantially diminished ambulation. Diagnostic imaging revealed a lumbar metastasis from a cholangiocarcinoma primary at L2–3 (4.5 cm anteroposterior × 5.7 cm transverse × 7.0 cm craniocaudal). The patient underwent a 2-level vertebrectomy with expandable cage placement and T10 to S2 fusion via a posterior-only approach. The patient regained much of his mobility and quality of life after the surgery.

LESSONS

Although this was a high-risk surgery, the authors show that a posterior-only approach can be used for lumbar vertebrectomies and fusion when necessary. Palliative surgeries carrying a high risk, especially in the setting of a limited prognosis, should include multidisciplinary deliberations and a thorough discussion of the risks and outcome expectations with the patient.

Open access

Adhesive arachnoiditis, subarachnoid hemorrhage, and intradural extramedullary thoracic cavernoma: illustrative case

Agne Andriuskeviciute, Michel Gustavo Mondragón-Soto, Nicolas Penet, and Juan Barges-Coll

BACKGROUND

Spinal arachnoiditis can result from various factors, including spinal subarachnoid hemorrhage (sSAH). In this paper, the authors describe a case of intradural extramedullary cavernoma with an initial presentation of subarachnoid hemorrhage leading to multilevel spinal arachnoiditis to discuss the pathophysiology and optimal treatment strategy.

OBSERVATIONS

Spinal intradural extramedullary cavernoma manifesting with sSAH is a rare clinical presentation; therefore, there is no clear strategy for the management of sSAH. Spinal arachnoiditis is a result of chronic inflammation of the pia arachnoid layer due to hematomyelia. No effective treatment that interrupts this inflammatory cascade and would also prevent the development of spinal arachnoiditis has been described to date.

LESSONS

Lumbar drainage could aid in sSAH management, relieve spinal cord compression, and restore the normal spinal cerebrospinal fluid circulation gradient. It could help to clear the blood degradation products rapidly and prevent early inflammatory arachnoiditis development. Mini-invasive intrathecal endoscopic adhesiolysis appears to be a reasonable approach for reducing the risk of aggravating spinal arachnoiditis with a mechanical-surgical stimulus. Whether a conservative approach should be applied in these patients with mild myelopathy symptoms is still debatable.

Open access

Immunocompetent isolated cerebral mucormycosis presenting with obstructive hydrocephalus: illustrative case

Khoa N Nguyen, Lindsey M Freeman, Timothy H Ung, Steven Ojemann, and Fabio Grassia

BACKGROUND

Isolated cerebral mucormycosis is rare in immunocompetent adults and is only sparsely reported to be associated with obstructive hydrocephalus.

OBSERVATIONS

Here, the authors report a case of obstructive hydrocephalus secondary to central nervous system mucormycosis without other systems or rhino-orbital involvement and its technical surgical management. A 23-year-old, incarcerated, immunocompetent patient with history of intravenous (IV) drug use presented with syncope. Although clinical and radiographic findings failed to elucidate an infectious pathology, endoscopy revealed an obstructive mass lesion at the level of the third ventricle, which, on microbiological testing, was confirmed to be Rhizopus fungal ventriculitis. Perioperative cerebrospinal fluid diversion, endoscopic third ventriculostomy, endoscopic biopsy technique, patient outcomes, and the literature are reviewed here. The patient received intrathecal and IV amphotericin B followed by a course of oral antifungal treatment and currently remains in remission.

LESSONS

The patient’s unique presentation and diagnosis of isolated cerebral mucormycosis reveal this pathogen as a cause of ventriculitis and obstructive hydrocephalus in immunocompetent adult patients, even in the absence of infectious sequelae on neuroimaging.

Open access

Impact of intraoperative cortical indocyanine green extravasation on local vasogenic edema immediately after direct revascularization in an adult with moyamoya disease: illustrative case

Maeho Yamasaki, Masaki Ito, Haruto Uchino, Taku Sugiyama, and Miki Fujimura

BACKGROUND

Local vasogenic edema induced after direct revascularization in moyamoya disease (MMD) is associated with blood–brain barrier dysfunction, potentially leading to postoperative cerebral hyperperfusion (CHP) or delayed intracerebral hemorrhage. This phenomenon allows the leakage of fluids, proteins, and other substances from the blood vessels into the extracellular compartment. Typically, such edema is observed postoperatively rather than intraoperatively.

OBSERVATIONS

A 48-year-old female with ischemic-onset MMD underwent revascularization on her left hemisphere with Suzuki’s angiographic stage III. Direct bypass was successfully performed, as confirmed by intravenous indocyanine green (ICG) video angiography. Subsequently, ICG extravasation was observed near the anastomosis site, despite the absence of cortical injury or bleeding under white light microscopy. Postoperative radiological imaging showed reversible pure vasogenic edema in the corresponding area, with no evidence of CHP. The patient did not exhibit neurological deterioration and was discharged home on postoperative day 16.

LESSONS

ICG, characterized by low molecular weight, water solubility, and high affinity with plasma proteins, can extravasate, serving as a direct indication of local vasogenic edema induced by direct revascularization in MMD. To enhance comprehension of the vulnerability of the blood–brain barrier in MMD, it is advisable to gather cases with prolonged observations of ICG video angiography after direct revascularization.