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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

First use of intraventricular nicardipine in a pediatric patient with vasospasm secondary to meningitis: illustrative case

V. Jane Horak, Nirali Patel, Sunny Abdelmageed, Jonathan Scoville, Melissa A LoPresti, and Sandi Lam

BACKGROUND

Cerebral vasospasm is commonly associated with adult aneurysmal subarachnoid hemorrhage but can develop in children. The standard vasospasm treatment includes induced hypertension, avoidance of hypovolemia, systemic use of the calcium channel blocker (CCB) nimodipine, and cerebral angiography for intraarterial therapy. Emerging treatments in adults, such as intraventricular CCB administration, have not been investigated in children. This study demonstrates the successful use of an intraventricular CCB in a pediatric patient with refractory vasospasm secondary to meningitis.

OBSERVATIONS

A 12-year-old female presented with Streptococcus pneumoniae meningitis and ventriculitis with refractory symptomatic cerebral vasospasm. She received a 5-day course of intrathecal nicardipine through an existing external ventricular drain. Her clinical status, transcranial Doppler studies, and radiography improved. Treatment was well tolerated.

LESSONS

Pediatric vasospasm is uncommon and potentially devastating. The management of vasospasm in adults occurs frequently. Principles of this management are adapted to pediatric care given the rarity of vasospasm in children. The use of intraventricular nicardipine has been reported in the care of adults with level 3 evidence. It has not been adequately reported in children with refractory vasospasm. Here, the first use of intraventricular nicardipine in treating pediatric cerebral vasospasm in the setting of meningitis is described and highlighted.

Open access

Arteriovenous malformation with unique drainage through the emissary vein of the foramen ovale: illustrative case

Xiaochun Zhao, Alexander R Evans, Ali Tayebi Meybodi, Nicholas Hopkins, Ira Bowen, Shyian S Jen, Mark C Preul, and Karl Balsara

BACKGROUND

As part of the laterotrigeminal venous system (LTVS), the emissary vein of the foramen ovale (EVFO) is an underrecognized venous structure communicating between the cavernous sinus and pterygoid plexus. The sphenobasal sinus is an anatomical variation of the sphenoparietal sinus that drains directly into the EVFO. The authors present the case of a ruptured arteriovenous malformation (AVM) with a unique drainage pattern through the sphenobasal sinus and EVFO.

OBSERVATIONS

A 9-year-old female initially presented with loss of consciousness and was subsequently found to have a ruptured AVM in the left basal frontal area. She underwent an immediate decompressive hemicraniectomy, with a computed tomography angiogram demonstrating a unique anatomical variation in which the sphenobasal sinus communicated with the EVFO and LTVS. The final venous drainage returned to the pterygoid plexus and external jugular vein. Postoperatively, the patient made a substantial recovery, with generalized right-sided weakness remaining as the sole deficit.

LESSONS

The authors present the case of a ruptured AVM with unique venous drainage into the sphenobasal sinus and EVFO, for which the current literature remains limited. As exemplified by this illustrative case, technique modification may be warranted in the setting of this unique anatomical variation to avoid venous sinus injury.

Open access

Continuous direct intraarterial treatment of meningitis-induced vasospasm in a pediatric patient: illustrative case

Aubrey C Rogers, Aditya D Goyal, and Alexandra R Paul

BACKGROUND

Bacterial meningitis–induced ischemic stroke continues to cause significant long-term complications in pediatric patients. The authors present a case of severe right internal carotid artery terminus and M1 segment vasospasm in a 9-year-old with an infected cholesteatoma, which was refractory to multiple intraarterial treatments with verapamil and milrinone. This is the first report of continuous intraarterial antispasmodic treatment in a pediatric patient as well as the first report of continuous treatment in an awake and extubated patient.

OBSERVATIONS

Arterial narrowing was successfully treated by continuous direct intraarterial administration of both a calcium channel blocker (verapamil) and a phosphodiesterase-3 inhibitor (milrinone). The patient recovered remarkably well and was discharged home with no neurological deficit (National Institutes of Health Stroke Scale score 0) and ambulatory without assistance after 22 days. The authors report a promising outcome of this technique performed in a pediatric patient.

LESSONS

This represents a novel treatment option for the prevention of stroke in pediatric bacterial meningitis. Continuous, direct intraarterial administration of antispasmodic medications can successfully prevent long-term neurological deficit in pediatric meningitis-associated vasospasm. The described method has the potential to significantly improve outcomes in severe pediatric meningitis-associated vasospasm.

Open access

Supracerebellar infratentorial resection of a torcular lesion causing fulminant intracranial hypertension: illustrative case

Jonathan Dallas, Jessica R Lane, Benjamin S Hopkins, Melinda Chang, Mark Borchert, Nestor R Gonzalez, Peter A Chiarelli, and Jason K Chu

BACKGROUND

Venous sinus stenosis has been implicated in intracranial hypertension and can lead to papilledema and blindness. The authors report the unique case of a cerebellar transtentorial lesion resulting in venous sinus stenosis in the torcula and bilateral transverse sinuses that underwent resection.

OBSERVATIONS

A 5-year-old male presented with subacute vision loss and bilateral papilledema. Imaging demonstrated a lesion causing mass effect on the torcula/transverse sinuses and findings of increased intracranial pressure (ICP). A lumbar puncture confirmed elevated pressure, and the patient underwent bilateral optic nerve sheath fenestration. Cerebral angiography and venous manometry showed elevated venous sinus pressures suggestive of venous hypertension. The patient underwent a craniotomy and supracerebellar/infratentorial approach. A stalk emanating from the cerebellum through the tentorium was identified and divided. Postoperative magnetic resonance imaging showed decreased lesion size and improved sinus patency. Papilledema resolved and other findings of elevated ICP improved. Pathology was consistent with atrophic cerebellar cortex. Serial imaging over 6 months demonstrated progressive decrease in the lesion with concurrent improvements in sinus patency.

LESSONS

Although uncommon, symptoms of intracranial hypertension in patients with venous sinus lesions should prompt additional workup ranging from dedicated venous imaging to assessments of ICP and venous manometry.

Open access

Microsurgical resection of a ruptured intraventricular arteriovenous malformation in a neonate: considerations in management. Illustrative case

Lauren Stone, Reid Colliander, Melissa A LoPresti, Ali Shaibani, and Sandi Lam

BACKGROUND

Arteriovenous malformations (AVMs) are the most common cause of intracranial hemorrhage in children, although they are rarer in neonates. Age, location, lesion architecture, and rupture status define treatment options. Sparse literature exists to guide the management of clinically symptomatic intraventricular AVM rupture in neonates. We highlight the case of a neonate with a ruptured intraventricular AVM to showcase considerations in treatment, discuss surgical technique, and help guide management.

OBSERVATIONS

An 18-day-old female presented with lethargy in extremis and was found to have new intraventricular hemorrhage. Angiogram revealed a Spetzler-Martin grade 2 AVM with a right posterior choroidal feeder and deep venous drainage within the ventricle. Her age limited radiosurgical and endovascular interventions. She underwent an interhemispheric, transcollosal, intraventricular approach for complete AVM resection. Perioperative care was managed by a multidisciplinary team, successfully mitigating the patient’s high risk of hemovascular collapse.

LESSONS

Stereotactic radiosurgery, endovascular embolization, and microsurgery are options for AVM obliteration, and multimodal therapy must be tailored to the lesion and patient. Conservative management can also be considered. Each intervention carries risks and varying likelihoods of success. Balancing these outcomes is challenging without definitive, high-quality, evidence-based guidance. The best treatment maximizes the chance of AVM obliteration while minimizing morbidity.

Open access

Ruptured brainstem arteriovenous malformation associated with a thalamoperforating artery aneurysm arising from the P1 segment of the right posterior cerebral artery: illustrative case

Yoshihito Hirata, Masakazu Okawa, Akira Ishii, Yu Abekura, Hisae Mori, Takayuki Kikuchi, Yukihiro Yamao, Susumu Miyamoto, and Yoshiki Arakawa

BACKGROUND

Cerebral aneurysms of perforating arteries are rare and can be difficult to detect on computed tomography angiography (CTA) and digital subtraction angiography. Treatment is challenging and associated with a significant risk of morbidity. Endovascular treatment of a thalamoperforating artery (TPA) aneurysm within the midbrain has not previously been reported.

OBSERVATIONS

A 13-year-old girl with no previous medical history presented with unconsciousness and anisocoria. Head computed tomography showed a right midbrain hemorrhage. CTA showed a midbrain arteriovenous malformation fed by a TPA aneurysm arising from the P1 segment of the right posterior cerebral artery. The feeder had a small distal aneurysm, which increased in size over time. Endovascular embolization was then performed.

LESSONS

Cerebral aneurysms of perforating arteries are rare and can be difficult to treat. This is the first report of the endovascular treatment of a TPA aneurysm within the midbrain. Understanding the individual patient’s brainstem perforator anatomy and the associated blood flow is essential before occluding a TPA aneurysm to avoid causing ischemia or infarction. Arteriovenous malformation embolization within the brainstem should be avoided because of interperforator anastomoses.

Open access

Large saccular intracranial aneurysm in a child with RASA1-associated capillary malformation–arteriovenous malformation syndrome: illustrative case

Marina Weinberger, Daniel S Ikeda, Shawn Belverud, Aaron Cho, Guerard Grice, Mary Willis, and Vijay M Ravindra

BACKGROUND

Large cerebral aneurysms are much less common in children than in adults. Thus, when present, these lesions require careful surgical evaluation and comprehensive genetic testing. RASA1-associated capillary malformation–arteriovenous malformation (RASA1-CM-AVM) syndrome is a rare disorder of angiogenic remodeling known to cause port-wine stains and arteriovenous fistulas but not previously associated with pediatric aneurysms.

OBSERVATIONS

The authors report the case of a previously healthy 6-year-old boy who presented with seizure-like activity. Imaging demonstrated a lesion in the right ambient cistern with compression of the temporal lobe. Imaging characteristics were suggestive of a thrombosed aneurysm versus an epidermoid cyst. The patient underwent craniotomy, revealing a large saccular aneurysm, and clip ligation and excision were performed. Postoperative genetic analysis revealed a RASA1-CM-AVM syndrome.

LESSONS

This is a rare case of a RASA1-associated pediatric cerebral aneurysm in the neurosurgical literature. This unique case highlights the need for maintaining a broad differential diagnosis as well as the utility of genetic testing for detecting underlying genetic syndromes in young children presenting with cerebral aneurysms.

Open access

Focal drug-resistant temporal lobe epilepsy associated with an ipsilateral anterior choroidal artery aneurysm: illustrative case

H. Westley Phillips, Shivani D. Rangwala, Joanna Papadakis, David J. Segar, Melissa Tsuboyama, Anna L. R. Pinto, Joseph P. Harmon, Sulpicio G. Soriano, Carlos J. Munoz, Joseph R. Madsen, Alfred P. See, and Scellig S. Stone

BACKGROUND

The occurrence of both an intracranial aneurysm and epilepsy, especially drug-resistant epilepsy (DRE), is rare. Although the overall incidence of aneurysms associated with DRE is unclear, it is thought to be particularly infrequent in the pediatric population. Surgical ligation of the offending aneurysm has been reported in conjunction with resolving seizure activity, although few cases have cited a combined approach of aneurysm ligation and resection of an epileptogenic focus.

OBSERVATIONS

We present the case of a 14-year-old female patient with drug-resistant temporal lobe epilepsy and an ipsilateral supraclinoid internal carotid artery aneurysm. Seizure semiology, electroencephalography monitoring, and magnetic resonance imaging all indicated a left temporal epileptogenic focus, in addition to an incidental aneurysm. The authors recommended a combined surgery involving resection of the temporal lesion and surgical clip ligation of the aneurysm. Near-total resection and successful ligation were achieved, and the patient has remained seizure free since surgery at 1 year postoperatively.

LESSONS

In patients with focal DRE and an adjacent intracranial aneurysm, a combined surgical approach involving both resection and surgical ligation can be used. Several surgical timing and neuroanesthetic considerations should be made to ensure the overall safety and efficacy of this procedure.

Open access

Terson syndrome secondary to aneurysmal subarachnoid hemorrhage in a child: illustrative case

Jacob M. Mazza, Parth Tank, Melissa A. LoPresti, Jonathan P. Scoville, Brenda L. Bohnsack, and Sandi Lam

BACKGROUND

Terson syndrome is the phenomenon of intraocular hemorrhage in the setting of subarachnoid hemorrhage (SAH). Vision loss can lead to morbidity for the affected individual. Aneurysmal SAH related to intracranial aneurysms is rare in children. Studies have shown the incidence of Terson syndrome in adults with aneurysmal SAH to be over 40%; however, few cases of Terson syndrome in pediatric aneurysmal SAH have been reported.

OBSERVATIONS

A 9-year-old male presented with altered mental status and seizures. Computed tomographic angiography showed aneurysmal SAH from a ruptured, left-sided posterior inferior cerebellar artery aneurysm. The patient underwent endovascular treatment with coiling and external ventricular drainage for SAH. Ophthalmological consultation for blurry vision revealed the diagnosis of Terson syndrome with decreased vision in the left eye, which was managed conservatively.

LESSONS

Terson syndrome after SAH can occur in children. Prompt ophthalmological evaluation in pediatric patients with aneurysmal SAH is vital for recognition and management to decrease overall morbidity.