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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

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

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.

Open access

Combination of neuroendoscopic hematoma evacuation and endovascular coil embolization for a ruptured anterior choroidal artery aneurysm in patients with moyamoya disease: illustrative cases

Kohei Uemasu, Hiroyuki Koizumi, Daisuke Yamamoto, Sumito Sato, Hideto Komai, Madoka Inukai, Takuichiro Hide, Yasushi Asari, and Toshihiro Kumabe

BACKGROUND

The treatment strategy for hemorrhagic moyamoya disease (MMD) due to a ruptured aneurysm at the distal portion of the anterior choroidal artery remains controversial. The authors successfully treated the ruptured aneurysm with neuroendoscopic hematoma evacuation, followed by endovascular coil embolization.

OBSERVATIONS

The authors encountered two patients with massive hemorrhagic MMD whose MMD had already been diagnosed and who had a periventricular anastomosis due to a ruptured aneurysm of the distal portion of the anterior choroidal artery involving the periventricular anastomosis. In both cases, neuroendoscopic hematoma evacuation was performed for hemorrhagic MMD in the acute phase, followed by endovascular coil embolization of the ruptured aneurysm in the chronic phase. In both endovascular treatments, the patient’s condition was stabilized by hematoma evacuation, allowing a detailed preoperative evaluation of the anatomical findings of the vessel and functional findings of intraoperative neurophysiological monitoring using continuous monitoring of motor evoked potentials to preserve motor function.

LESSONS

Combination therapy can be useful for hemorrhagic MMD in patients with diagnosed MMD with a periventricular anastomosis. Additionally, a preoperative understanding of the vascular construction and intraoperative neurophysiological monitoring will aid in the successful coil embolization of aneurysms at the distal portion of the anterior choroidal artery with hemorrhagic MMD.

Open access

Successful surgical management of a superior cerebellar artery aneurysm in a patient with Marfan syndrome: illustrative case

Fangjun Liu, Mengqing Hu, Daling Ruan, Xiaoling Ruan, Ting Lei, and Xiang’en Shi

BACKGROUND

Marfan syndrome, a connective tissue disorder, poses unique challenges in neurosurgery, given the fragility of vascular structures. Superior cerebellar artery (SCA) aneurysms in patients with the syndrome are rare and present distinct surgical difficulties, necessitating innovative approaches.

OBSERVATIONS

A 29-year-old male with Marfan syndrome presented with a subarachnoid hemorrhage from a ruptured SCA aneurysm. Given the lack of a defined aneurysm neck and the small diameter of the SCA, standard clipping and endovascular therapies were unsuitable. A microsurgical approach using microsutures was successfully employed, effectively managing the aneurysm while preserving the parent artery.

LESSONS

This case underscores the efficacy of the microsuture technique in complex neurosurgical scenarios, particularly in patients with connective tissue disorders such as Marfan syndrome. The adaptability of surgical strategies, as demonstrated in this case, is crucial for achieving successful outcomes in patients with unique anatomical challenges.

Open access

Spontaneous development and involution of a de novo pseudoaneurysm at the superficial temporal artery–middle cerebral artery bypass anastomotic site in a patient with moyamoya disease: illustrative case

Kristine Ravina, Biraj Patel, and Benjamin Yim

BACKGROUND

De novo pseudoaneurysm formation is a rare complication of extracranial-intracranial bypass surgery.

OBSERVATIONS

The authors report the case of a 28-year-old male who presented with new-onset right temporal and occipital ischemia who was found to have bilateral proximal internal carotid artery occlusion with collateral vasculature formation consistent with moyamoya disease. The patient underwent bilateral superficial temporal artery–middle cerebral artery bypasses. A de novo pseudoaneurysm was found at the left-sided bypass distal anastomotic site recipient vessel 1 month after the surgery. The pseudoaneurysm demonstrated a progressive reduction in size and eventual complete involution at 6 months after surgery.

LESSONS

Limited literature reports extracranial-intracranial bypass-associated aneurysms treated primarily with either clipping or resection and reanastomosis. The authors demonstrate, for the first time, a progressively benign natural history course of an extracranial-intracranial bypass distal anastomotic site pseudoaneurysm.

Open access

Sternocleidomastoid muscle-splitting method for high cervical carotid endarterectomy: illustrative cases

Atsushi Sato, Tetsuo Sasaki, Toshihiro Ogiwara, Kazuhiro Hongo, and Tetsuyoshi Horiuchi

BACKGROUND

The number of cervical carotid endarterectomies (CEAs) has decreased as carotid artery stenting (CAS) has increased. However, CEA and CAS both have advantages and disadvantages; therefore, appropriate procedures must be selected for individual patients. High-positioned carotid artery stenosis presents technical challenges for CEA and is occasionally managed by performing CAS. However, CAS is associated with a high risk of thrombosis in patients with soft plaques, suggesting a clinical need for a better procedure. Consequently, appropriate surgical treatment for patients requiring high-level CEAs is essential.

OBSERVATIONS

In this study, a novel and straightforward method was devised. The primary concept underlying this technique is separation of the sternocleidomastoid muscle (SCM) from other anatomical structures to ensure a wider surgical field. By anatomically separating the SCM into the sternal and clavicular head groups, the objective of the wider surgical field can be met. Herein, we report technical innovations in high-positioned carotid artery stenosis and evaluate their efficacy in two patients.

LESSONS

In conclusion, high CEA surgery using this new method is valuable and may eliminate barriers to more advanced approaches.

Open access

Disconnection of a jugular foramen dural arteriovenous fistula with cortical venous reflux via an intradural retrosigmoid approach: illustrative case

Richard Shaw, Johnny Wong, Hugo Andrade, and Ivan Radovanovic

BACKGROUND

Jugular foramen dural arteriovenous fistulas (DAVFs) are rare and challenging lesions. Described methods of treatment include embolization and microsurgical disconnection through a far lateral transcondylar approach. The authors present the case of a Borden type III jugular foramen DAVF, which was treated with a novel, less invasive retrosigmoid approach with intradural skeletonization and packing of the sigmoid sinus.

OBSERVATIONS

The patient presented with headache and visual field deficit. Neuroimaging demonstrated a right temporal intracerebral hematoma with mass effect. This was due to a Borden type III jugular foramen DAVF with cortical venous reflux into the vein of Labbe secondary to recanalization of a previously thrombosed sigmoid sinus. Microsurgical disconnection was performed via a retrosigmoid approach, in which the sigmoid sinus was identified intradurally at the jugular foramen. The sigmoid sinus was isolated by drilling at the pre- and retrosigmoid spaces to permit packing and clip ligation. Postoperative angiography revealed complete occlusion of the DAVF.

LESSONS

Jugular foramen DAVFs are rare entities, which have been traditionally treated through a far lateral transcondylar approach. An intradural retrosigmoid approach is a safe, less invasive alternative, which involves less soft tissue and bony dissection and does not have the associated morbidity of craniocervical instability and hypoglossal neuropathy.

Open access

High-flow bypass for giant dolichoectatic vertebrobasilar aneurysms: illustrative cases

Richard Shaw, Alistair Kenneth Jukes, and Rodney Stewart Allan

BACKGROUND

Giant fusiform dolichoectatic vertebrobasilar artery aneurysms are challenging lesions with a poor natural history. When there is progressive brainstem compression from these lesions, endovascular treatment can be insufficient, and bypass surgery remains a possible salvage option. High-flow bypass surgery with proximal occlusion can potentially arrest aneurysm growth, promote aneurysm thrombosis, and reduce rupture risk. The authors describe their experience in two patients with giant fusiform dolichoectatic vertebrobasilar artery aneurysms treated with high-flow bypass.

OBSERVATIONS

Both patients presented with enlarging giant dolichoectatic vertebrobasilar aneurysms causing symptomatic brainstem compression. The authors performed staged treatment involving high-flow bypass from the external carotid artery to the posterior cerebral artery using a saphenous vein graft, Hunterian proximal vertebrobasilar occlusion, and finally posterior fossa decompression with or without direct aneurysm thrombectomy and debulking. Postoperative angiography revealed successful flow reversal, aneurysm exclusion, and no brainstem stroke. Clinically, one patient had improvement in their modified Rankin Scale (mRS) score from 3 preoperatively to 1 at 12-month follow-up. The second patient had a deterioration in their mRS score from 4 to 5 at 12-month follow-up.

LESSONS

High-flow bypass strategies remain high risk but can be a viable last resort in patients with neurological deficits and enlarging giant fusiform dolichoectatic vertebrobasilar artery aneurysms.

Open access

Surgically treated intracranial arteriovenous fistulas with hemorrhage, resulting in complete obliteration: illustrative cases

Ako Matsuhashi, Kei Yanai, Satoshi Koizumi, and Gakushi Yoshikawa

BACKGROUND

Intracranial arteriovenous fistula (AVF) is a rare disease, defined as anastomoses between cerebral or meningeal arteries and dural venous sinuses or cortical veins. With the development of new agents and devices, endovascular embolization has been considered safe and effective in a majority of cases. However, cases that require direct surgery do exist. Herein, the authors present 3 cases of intracranial AVFs that presented with hemorrhage and were treated with direct surgery, achieving complete obliteration and favorable outcomes.

OBSERVATIONS

Intracranial AVFs that present with hemorrhage require immediate and complete obliteration. When AVFs involve the dural sinus, transvenous embolization is usually the first choice of treatment. AVFs with single cortical venous drainage are best treated with interruption of the draining vein close to the fistula. Transarterial embolization can be a curative treatment if there are no branches supplying cranial nerves or an association with pial feeders. In cases in which endovascular treatment is technically challenging or has resulted in incomplete occlusion, surgical treatment is indicated.

LESSONS

Despite the recent rise in endovascular treatment, it is important to recognize situations in which such treatment is not suitable for intracranial AVFs. Direct surgery is effective in such cases to offer the best possible outcome.