Browse

You are looking at 1 - 10 of 11 items for :

  • Refine by Access: all x
Clear All
Open access

Arteriovenous malformation presenting as complex regional pain syndrome: illustrative case

Dayna C Sloane, Diego D Luy, Atul K Mallik, Joseph C Serrone, and Douglas E Anderson

BACKGROUND

Complex regional pain syndrome (CRPS) is typically described as a peripheral nerve disorder in which exaggerated allodynia and hyperalgesia follow a minor injury. Some researchers propose a central mechanism, although current evidence is lacking.

OBSERVATIONS

A 14-year-old female presented with classic CRPS symptoms of left upper-extremity weakness and hyperalgesia after a bout of sharp pain in her thumb while shoveling snow. A possible seizure prompted magnetic resonance imaging, revealing a right frontal Spetzler-Martin grade II arteriovenous malformation (AVM) adjacent to the primary motor cortex. Brodmann areas 1, 3a, and 3b, which are responsible for localizing and processing burning and painful sensations, were also involved. The patient underwent transarterial Onyx embolization in two sessions and microsurgical resection, after which her CRPS symptoms completely resolved.

LESSONS

To our knowledge, this is the first reported case of a cerebral AVM presenting as CRPS, which supports a central mechanism. The authors propose that rapid growth of the AVM led to a vascular steal phenomenon of surrounding parenchyma, which disrupted the patient’s normal motor function and nociceptive processing. Further validation in other series is needed.

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

Rare median and musculocutaneous nerve fusion with intraoperative electrical confirmation: illustrative case

Audrey Huang, Sima Vazquez, Jose Dominguez, Avinash Mohan, Jin Li, and Jared M Pisapia

BACKGROUND

Nerve transfer is a surgical technique in which a redundant or expendable fascicle is transferred or coapted to an injured nerve distal to the site of injury for the purpose of reinnervation. Successful nerve transfer is dependent on correct intraoperative identification of donor and recipient nerves.

OBSERVATIONS

An 8-year-old male was recommended for ulnar nerve fascicle to biceps branch of musculocutaneous nerve transfer to restore elbow flexion weakness after a demyelinating spinal cord injury. The biceps branch was identified approximately midway along the upper arm. Proximal musculocutaneous nerve stimulation induced hand movement and electromyography activity in the median nerve muscles. Neurolysis of the thickened proximal structure revealed fusion of the musculocutaneous and median nerves. Because of the proximity of the median and musculocutaneous nerves, median rather than ulnar nerve fascicles were used as donors for transfer.

LESSONS

The authors provide the first reported intraoperative finding of an anatomical variant in which the musculocutaneous nerve and median nerve were fused in the upper arm, confirmed through intraoperative electrical stimulation. Surgeons should be aware of this rare anatomical variant to ensure correct nerve identification when performing nerve transfers in the proximal upper extremity.

Open access

Vein of Galen malformations in the newborn: case series

Erik F. Hauck, Jeremy A. Yarden, Lily I. Hauck, Joseph M. Bibawy, Shervin Mirshahi, and Gerald A. Grant

BACKGROUND

Vein of Galen malformations (VoGMs) in newborns often represent life-threatening emergencies. Outcome is difficult to predict. The authors review 50 VoGM cases to correlate anatomical types with treatment and outcome.

OBSERVATIONS

Four distinct types of VoGMs are identified: mural simple (type I), mural complex (type II), choroidal (type III), and choroidal with deep venous drainage (type IV). Seven patients presented with mural simple VoGMs with a “single hole” fistula supplied by only one large feeder. These patients were treated electively at >6 months; development was normal. Fifteen patients presented with complex mural VoGMs. Multiple large feeders joined a single fistulous point within the wall of the varix. Patients typically presented with congestive heart failure (CHF) and required emergent transarterial intervention. Mortality was 7.7% with less than two-thirds developing normally. Twenty-five patients presented with choroidal VoGMs. Multiple large arterial feeders joined at multiple fistulous sites. Severe CHF in most patients required emergent transarterial and sometimes transvenous intervention. Mortality was 9.5%; two-thirds of the patients had a normal development. Three babies presented with choroidal VoGMs with deep intraventricular venous drainage. This phenomenon caused fatal “melting brain syndrome” in all three patients.

LESSONS

Recognition of the specific VoGM type determines treatment options and sets outcome expectations.

Open access

Radial nerve myofibroma: a rare benign tumor with perineural infiltration. Illustrative case

Kitty Y. Wu, David J. Cook, Kimberly K. Amrami, and Robert J. Spinner

BACKGROUND

Myofibromas are benign mesenchymal tumors, classically presenting in infants and young children in the head and neck region. Perineural involvement, especially in peripheral nerves within the upper extremity, is extremely rare in myofibromas.

OBSERVATIONS

The authors present the case of a 16-year-old male with a 4-month history of an enlarging forearm mass and rapidly progressive dense motor weakness in wrist, finger, and thumb extension. Preoperative imaging and fine needle biopsy confirmed the diagnosis of a benign isolated myofibroma. Given the dense paralysis, operative management was indicated, and intraoperative exploration showed extensive involvement of tumor within the radial nerve. The infiltrated nerve segment was excised along with the tumor, and the resulting 5-cm nerve gap was reconstructed using autologous cabled grafts.

LESSONS

Perineural pseudoinvasion can be an extremely rare and atypical feature of nonmalignancies, resulting in dense motor weakness. Extensive nerve involvement may still necessitate nerve resection and reconstruction, despite the benign etiology of the lesion.

Open access

Clival intraosseous myxoma treated with an endoscopic endonasal approach: illustrative case

Mokshal H. Porwal, Daniel M. Aaronson, Abrahim N. Razzak, and Nathan T. Zwagerman

BACKGROUND

Solitary lesions of the clivus, especially nonchordomatous ones, are exceptionally rare pathologies representing only 0.1%–0.2% of intracranial tumors that may present diagnostic and therapeutic challenges. Intraosseous myxomas are benign tumors arising from mesenchymal cells with an unknown pathophysiology.

OBSERVATIONS

In this article, a 15-year-old male with clival intraosseous myxoma who was treated via an endoscopic endonasal surgical approach is presented. The patient became symptomatic after a baseball struck his head, leading to throbbing headaches and visual difficulties for tracking objects. Examination noted diplopia, cranial nerve VI palsy, and ptosis. After imaging revealed a tumor extending to the left cavernous sinus with bulging of the dorsal clivus against the basilar artery/ventral pons, resection was performed. Postoperatively, the patient noted ocular motility and alignment improvement without further complications.

LESSONS

Clival masses present with symptoms from compromised neurovascular structures including visual disturbances and trigeminal sensory deficits. Given the rarity of these entities, patients may postpone further treatment until workup; this patient was misdiagnosed for possible concussion until several weeks passed. A PubMed database review of cranial myxoma cases was conducted to identify solitary clival intraosseous myxoma cases. This case is one of the few in which this pathology was treated through an endoscopic endonasal operative approach without complications, demonstrating its safety and effectiveness.

Open access

Surgical outcome of a patient with Bertolotti’s syndrome in whom the established Castellvi classification system failed: illustrative case

Richard J. Chung, Camryn Harvie, John O’Donnell, Sarah Jenkins, and Arthur L. Jenkins III

BACKGROUND

Bertolotti’s syndrome is a condition of the lower back and/or L5 distribution leg pain caused by a lumbosacral transitional vertebra (LSTV). Diagnosing the LSTV as the cause of the symptoms and condition is essential for accurate management of this syndrome. Castellvi’s classification system is widely accepted for LSTV anatomy, but it measures only one aspect of transitional anatomy and was intended primarily to identify target-level disk herniations.

OBSERVATIONS

In this case, the Castellvi classification system failed to identify the patient (with 2 years of back and L5 pain) as having an LSTV, even though he displayed LSTV-like anatomy because both L5 transverse process heights measured less than 19 mm. He attained brief but significant relief from bilateral injections into the L5–S1 transverse/ala region and underwent a minimally invasive bilateral decompression of L5–S1 with almost complete relief of his symptoms maintained more than 6 months postoperatively.

LESSONS

Given that the patient gained significant relief from treatment of transitional anatomy that failed to be identified using Castellvi’s classification system, this case suggests that transverse process height may not be adequate or even the most clinically relevant indicator in identifying LSTV anatomy, which is a precursor to the diagnosis of Bertolotti’s syndrome.

Open access

A deceptive mass on neonatal ultrasound and a fetal brain MRI-confirmed complex dural arteriovenous fistula postnatally: illustrative case

Elliot T. Varney, Charlotte S. Taylor, Allen G. Crosthwait, Kristin Weaver, and Todd Nichols

BACKGROUND

Dural arteriovenous fistulas (dAVFs) are direct, aberrant connections between dural arteries and cerebral veins. In neonates, delayed diagnosis results in grim outcomes. Treatment involves endovascular management because of its success and tolerability. Here, the authors present a case of a complex dAVF initially recognized with an in utero neurosonogram and fetal magnetic resonance imaging (MRI).

OBSERVATIONS

A 21-week fetal ultrasound of a nonspecific brain mass was confirmed with fetal MRI as a 2.7-cm T1-hyperintense posterior fossa mass. Although a large flow void in the left middle cranial fossa was present, postnatal computed tomography angiography ultimately revealed a high-flow dAVF communicating with the left transverse sinus. In the early postnatal period, the patient developed hydrocephalus. After successful partial embolization, 6-week postangiogram brain MRI indicated disease progression with the development of a venous varix causing brainstem compression. Repeat embolization resulted in complete cessation of early venous drainage.

LESSONS

Neonatal dAVFs are exceedingly rare and result in futile outcomes; however, detection in utero is possible. Although definitive therapy must be performed postnatally, constant monitoring and early delivery can prevent complications. Attention to fetal ultrasound is essential, and knowledge of fetal MRI in the detection of these complex lesions can significantly improve outcomes.

Open access

Torcular pseudomass in a 14-month-old child: illustrative case

Lance M. Villeneuve, Murali Nagarajan, Benjamin O. Cornwell, Laura Propester, Xiaochun Zhao, Jami Valenzuela, Joanna Gernsback, Virendra Desai, and Andrew Jea

BACKGROUND

“Torcular pseudomass,” or redundant soft tissue in the torcular region, is not an infrequent incidental finding on advanced imaging of the brain in infants and young children. It was recently codified among pediatric neuroradiologists; however, its report in the pediatric neurosurgical community has not previously been elucidated.

OBSERVATIONS

The authors present a case of a 14-month-old child who presented with fever and a first-time seizure. Computed tomography of the head suggested an epidural abscess; however, magnetic resonance imaging characteristics of the lesion were consistent with torcular pseudomass, a normal variant. At the 3-month follow-up, the child was continuing to do well and had not had another seizure. There have been no indications for surgical intervention or additional radiographic surveillance.

LESSONS

The differential diagnosis for torcular pseudomass includes dural venous sinus thrombosis, dermoid cysts, occipital encephalocele, eosinophilic granuloma, and primary and metastatic tumors, such as neuroblastoma. The management of each of these disorders in the differential diagnosis may be much more invasive than continued observation in the case of torcular pseudomass. Therefore, it is important for pediatric neurosurgeons to become familiar with this developmental anomaly of the dura and occipital skull.

Open access

Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case

Grant Koskay, Patrick Opperman, Frank M. Mezzacappa, Joseph Menousek, Megan K. Fuller, Linden Fornoff, and Daniel Surdell

BACKGROUND

Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient.

OBSERVATIONS

A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae.

LESSONS

Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.