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Spontaneous regression of giant arteriovenous fistulae during the perinatal period

Case report

William C. Hanigan, Terry Brady, Michael Medlock, and Edward B. Smith

✓ A unique case of spontaneous regression of giant arteriovenous fistulae during infancy is described in this report. A female infant, the product of normal labor and delivery, demonstrated severe ventriculomegaly and an intracranial hemorrhage at birth. Cerebral angiography at 5 days of age revealed several large fistulae fed by the anterior and middle cerebral arteries draining into the deep venous system through a dilated internal cerebral vein and ectatic vein of Galen. Two days following the angiogram, a second intracranial hemorrhage occurred. Active hydrocephalus developed over the next 6 months and was treated with ventriculoperitoneal shunting. When the child was 8 months of age, angiography failed to demonstrate the fistulae. It was postulated that pressure effects from the intracranial hematoma and long-standing intracranial hypertension as well as stenosis in the anomalous venous outflow resulted in vascular stasis, venous thrombosis, and selective arterial occlusion. Hydrocephalus was a result of the compression of the intraventricular foramina by dilated embryonic vessels. This anomaly, predominantly involving the anterior circulation, may be homologous to the vein of Galen aneurysm in the posterior circulation.

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Seckel syndrome and moyamoya

Case report

Patrick J. Codd, R. Michael Scott, and Edward R. Smith

Seckel syndrome is an autosomal recessive disorder characterized by intrauterine and postnatal growth delay, microcephaly with mental retardation, and facial dysmorphisms including micrognathia, a recessed forehead, and a large beaked nose. Occurring in 1 in 10,000 children without sex preference, it is the most common primordial microcephalic osteodysplastic dwarfism and has been associated with a variety of congenital brain malformations and intracranial aneurysms. Moyamoya syndrome is an idiopathic, chronic, progressive cerebrovascular disorder marked by stenosis of the intracranial internal carotid arteries and concurrent development of hypertrophied collateral vessels. These tortuous arterial collaterals appear radiographically as “puffs of smoke,” giving the syndrome its name. In this report, the authors describe the case of a 16-year-old girl with coincident Seckel and moyamoya syndromes. To their knowledge, this is the first reported case of such an association being treated with surgical revascularization.

The patient presented with persistent headaches and a 2-year history of progressive hand, arm, and face numbness. Imaging studies revealed multiple completed cerebral infarcts, global ischemic changes, and vascular anatomy consistent with moyamoya syndrome. Bilateral pial synangioses successfully revascularized each hemisphere with resolution of the patient's symptoms. The patient died 1 year later of complications related to treatment of a rapidly progressing intracranial aneurysm.

This report documents the first case associating moyamoya and Seckel syndromes. In addition, the report reveals the rapid development of an intracranial aneurysm in a patient with this syndrome. When coupled with previous reports of other types of cerebrovascular disease in patients with Seckel syndrome or other primordial dwarfisms, the authors' findings are important because they suggest that physicians treating patients with dwarfism should consider the diagnosis of moyamoya syndrome when symptoms suggestive of cerebral ischemia are present. Prompt diagnosis and treatment of moyamoya syndrome, including the use of proven surgical revascularization procedures such as pial synangiosis, may significantly improve the long-term outcomes of these patients.

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Editorial: Pediatric moyamoya

Kyu-Chang Wang

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Increasing precision in the management of pediatric neurosurgical cerebrovascular diseases with molecular genetics

Kristopher T. Kahle, Daniel Duran, and Edward R. Smith

Recent next-generation DNA and RNA sequencing studies of congenital and pediatric cerebrovascular anomalies such as moyamoya disease, arteriovenous malformations, vein of Galen malformations, and cavernous malformations have shed new insight into the genetic regulation of human cerebrovascular development by implicating multiple novel disease genes and signaling pathways in the pathogenesis of these disorders. These diseases are now beginning to be categorized by molecular disruptions in canonical signaling pathways that impact the differentiation and proliferation of specific venous, capillary, or arterial cells during the hierarchical development of the cerebrovascular system. Here, the authors discuss how the continued study of these and other congenital cerebrovascular conditions has the potential to replace the current antiquated, anatomically based disease classification systems with a molecular taxonomy that has the potential to increase precision in genetic counseling, prognostication, and neurosurgical and endovascular treatment stratification. Importantly, the authors also discuss how molecular genetic data are already informing clinical trials and catalyzing the development of targeted therapies for these conditions historically considered as exclusively neurosurgical lesions.

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Development of moyamoya arteriopathy following treatment of intracranial tumors: clinical and radiographic characterization

Jong Seok Lee, Armide Storey, Arianna Lanpher, and Edward R. Smith

OBJECTIVE

Moyamoya arteriopathy can develop in patients with brain tumors, particularly when associated with neurofibromatosis type 1 (NF1) or cranial irradiation. The present study aimed to analyze the clinical outcomes of moyamoya after brain tumor treatment and elucidate the effect of revascularization on brain tumors.

METHODS

The authors retrospectively reviewed clinical and radiographic findings in 27 patients with brain tumors who developed moyamoya requiring revascularization surgery between January 1985 and June 2017 at a single institution. The long-term clinical and neuroimaging-based outcomes were analyzed.

RESULTS

Among 27 patients, 22 patients underwent radiotherapy, and 12 patients had NF1. The mean ages at diagnosis of brain tumors and moyamoya were 4.4 years and 10.3 years, respectively. The mean interval between radiotherapy and moyamoya diagnosis was 4.0 years. The mean follow-up period after revascularization surgery was 8.5 years. Among 46 affected hemispheres in 27 patients, the patients who underwent radiotherapy (30 hemispheres in 22 patients) had a higher incidence of Suzuki stage 5 or 6 (20% [6/30] vs 0% [0/8]) and infarction (63.6% [14/22] vs 0% [0/5]) compared with patients without radiotherapy (8 hemispheres in 5 patients). After revascularization, stroke occurred in 4 patients, and 6 hemispheres showed Matsushima grade C, all of which occurred in patients with a history of radiotherapy. The residual brain tumors progressed in 4 of 21 patients (19%) after revascularization, comparable to the progression rates of brain tumors without revascularization in previous literature.

CONCLUSIONS

Patients with brain tumors can develop moyamoya that exhibits characteristic clinical and radiographic features of idiopathic MMD. Moyamoya associated with cranial irradiation has a higher incidence of stroke with less capacity for revascularization, requiring thorough evaluations and timely treatment. Revascularization does not appear to have any effect on the progression of existing brain tumors.

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Indirect bypass for maternal symptomatic moyamoya in the first trimester of pregnancy: case report

Katie P. Fehnel, Craig D. McClain, and Edward R. Smith

There are no practice guidelines for the treatment of moyamoya disease in pregnant women. The need for such guidelines, however, is evidenced by the numerous case reports, case series, and systematic reviews in the literature highlighting an at-risk period for female moyamoya patients of childbearing age. Here the authors review and interpret the existing literature as it applies to their index patient and expand the literature in support of treating select patients during pregnancy. The authors describe what is to their knowledge the first case reported in the literature of a patient successfully treated with indirect surgical revascularization during the first trimester, who went on to deliver a healthy term baby without complications.

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Chorea in the clinical presentation of moyamoya disease: results of surgical revascularization and a proposed clinicopathological correlation

Clinical article

Edward S. Ahn, R. Michael Scott, Richard L. Robertson Jr., and Edward R. Smith

Object

Chorea is a movement disorder characterized by brief, irregular, involuntary contractions that appear to flow from 1 muscle to another. There are a limited number of reports in the literature that have linked moyamoya disease and chorea. The authors describe their experience in treating moyamoya disease in patients in whom chorea developed as part of the clinical presentation.

Methods

The authors conducted a retrospective review of a consecutive series of 316 children who underwent pial synangiosis revascularization for moyamoya disease at the Boston Children's Hospital.

Results

Of 316 surgically treated patients with moyamoya disease, 10 (3.2%; 6 boys and 4 girls) had chorea as a part of their presentation. The average age at surgical treatment was 9.9 years (range 3.8–17.9 years). All patients had evidence of hypertrophied lenticulostriate collateral vessels through the basal ganglia on preoperative angiography and/or MRI on affected sides. Two patients had cystic lesions in the basal ganglia. Nine patients underwent bilateral craniotomies for pial synangiosis, and 1 patient underwent a single craniotomy for unilateral disease. Follow-up was available in 9 patients (average 50.1 months). The mean duration of chorea was 1.36 years (range 2 days to 4 years), with resolution of symptoms in all patients. One patient developed chorea 3 years after surgical treatment, 4 patients had transient chorea that resolved prior to surgery, and 5 patients experienced resolution of the chorea after surgery (average 13 months).

Conclusions

The authors describe children with moyamoya disease and chorea as part of their clinical presentation. The data suggest that involvement of the basal ganglia by the hypertrophied collateral vessels contributes to the development of chorea, which can wax or wane depending on disease stage or involution of the vessels after revascularization surgery. In most patients, however, the chorea improves or disappears about 1 year after presentation.

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Traumatic pericallosal artery aneurysm: a rare complication of transcallosal surgery

Case report

Ian F. Dunn, Graeme F. Woodworth, Adnan H. Siddiqui, Edward R. Smith, G. Edward Vates, Arthur L. Day, and Liliana C. Goumnerova

✓ Traumatic intracranial aneurysms are rare in adults but account for up to 33% of all aneurysms encountered in a pediatric population. The most common location of such lesions in children is the pericallosal or adjacent branch of the anterior cerebral artery, where a head impact exerts sudden decelerating shearing forces on the arteries tethered on the brain surface against an immobile falx cerebri, weakening the arterial wall. This action can lead to dissection of the damaged vascular layers, with resultant expansion of the affected site into a fusiform aneurysm. Pericallosal aneurysms following a penetrating intracranial injury have also been described, and the resultant lesion in some cases can be a pseudoaneurysm. The incidence of iatrogenic pericallosal artery aneurysms, however, is extremely rare.

The authors describe the first reported case of a traumatic pericallosal artery aneurysm following transcallosal surgery. This 6-year-old boy underwent resection of a hypothalamic pilocytic astrocytoma, which was approached via the transcallosal corridor. A follow-up magnetic resonance image obtained within 1 year of surgery disclosed a small flow void off the right pericallosal artery, which was initially interpreted as residual tumor. Serial investigations showed the lesion enlarging over time, and subsequent angiography revealed a round 7-mm pericallosal artery aneurysm with an irregularly shaped 2- to 3-mm lumen. The aneurysm was difficult to treat with clip reconstruction or suturing of the affected segment, and an excellent outcome was ultimately achieved with resection of the lesion and autogenous arterial graft interposition. The authors also discuss the likely pathophysiology of the aneurysm and the surgical procedures undertaken to treat it.

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Long-term clinical and radiographic outcomes after pial pericranial dural revascularization: a hybrid surgical technique for treatment of anterior cerebral territory ischemia in pediatric moyamoya disease

Alaa Montaser, Jessica Driscoll, Hudson Smith, Madeline B. Karsten, Emily Day, Tina Mounlavongsy, Darren B. Orbach, and Edward R. Smith

OBJECTIVE

Isolated anterior cerebral artery (ACA) territory ischemia in pediatric moyamoya disease (MMD) is rare but has been increasingly recognized, particularly in children manifesting progression of disease in a delayed fashion after middle cerebral artery revascularization surgery. Surgical treatment is complicated by limited graft choices, with the small number of case series largely focused on complex, higher-risk operations (omental flap transfers, large interhemispheric rotational grafts); direct bypass (often untenable in children due to vessel size); or, alternatively, the technically simpler method of multiple burr holes (of limited efficacy outside of infants). Faced with the problem of a growing cohort of pediatric patients with MMD that could benefit from anterior cerebral revascularization, the authors sought to develop a solution that was specifically designed for children and that would be lower risk than the more complex approaches adapted from adult populations but more effective than simple burr holes. In this study, the authors aimed to describe the long-term clinical and radiographic outcomes of a novel approach of pial pericranial dural (PiPeD) revascularization, building on the principles of pial synangiosis but unique in using the pericranium and the dura mater as the primary vascular supply, and employing a larger craniotomy with arachnoid dissection to provide robust full-territory revascularization in all ages with reduced risk relative to more complex procedures.

METHODS

The medical records of all pediatric patients with MMD who presented at a single center between July 2009 and August 2019 were retrospectively reviewed to identify patients with MMD with anterior cerebral territory ischemia. Clinical characteristics, surgical indications, operative techniques, and long-term clinical and radiographic follow-up data were collected and analyzed.

RESULTS

A total of 25 operations (5.6% of total procedures) were performed in 21 patients (mean age 9.4 years [range 1–16.5 years]; 12 female and 9 male). Almost one-third of the patients had syndromic associations, with no familial cases. Complications included 1 patient (4.7%) with a superficial infection, with no postoperative strokes, hemorrhage, seizures, or deaths. Long-term follow-up was available in 18 of 21 patients (mean 24.9 months [range 4–60 months]). Radiographic engraftment was present in 90.9% (20/22 hemispheres), and no new strokes were evident on MRI on long-term follow-up, despite radiographic progression of the disease.

CONCLUSIONS

The use of the pericranium and the dura mater for indirect revascularization provided robust vascularized graft with great flexibility in location and high potential for engraftment, which may obviate more complex and higher-risk operations for ACA territory ischemia. Long-term follow-up demonstrated that PiPeD revascularization conferred durable, long-term radiographic and clinical protection from stroke in pediatric patients with MMD. Based on the results of the current study, the PiPeD technique can be considered an additional tool to the armamentarium of indirect revascularization procedures in select pediatric patients with MMD.

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Editorial: The challenges of determining natural history

John Kestle