Seizure outcome in moyamoya after indirect revascularization in pediatric patients: Retrospective study and literature review

Background: Moyamoya disease (MMD) is a unique cerebrovascular disorder characterized by progressive stenosis of anterior cerebral circulation. Moyamoya is not an uncommon disease in Saudi Arabia. Although a less common symptom of the disease, the incidence of seizure in MMD ranges from 6 to 30%. Indirect revascularization through Encephaloduroarteriosynangiosis technique is one of the surgical treatment options for MMD. In our cohort, we aim to evaluate seizure outcome in pediatric patients with moyamoya. Methods: Eleven patients with seizure as primary presentation for MMD over a period of 10 years were included in the study. All patients underwent EDAS surgery. All patients underwent pre- and postoperative assessment of multiple factors contributing to seizure outcome. Patients were evaluated for surgery control clinically and radiologically. Results: About 73% of MMD patients with seizures improved after EDAS surgery (P < 0.0005). Six patients out of 11 became seizure free. Patients with bilateral involvement of disease undergoing bilateral surgery had better seizure control than those undergoing unilateral surgery (P < 0.07). Conclusion: Patients with controlled seizure before surgery are more likely to be seizure free after intervention. Seizure outcome is favorable after indirect surgical revascularization in pediatric moyamoya patients.

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Right frontotemporal craniotomy for ECA-to-MCA direct and indirect bypass and occipital artery indirect bypass to the posterior circulation: case report

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.7.peds20181 ◽

2020 ◽

pp. 1-5

Author(s):

Mitchell W. Couldwell ◽

Samuel Cheshier ◽

Philipp Taussky ◽

Vance Mortimer ◽

William T. Couldwell

Keyword(s):

Case Report ◽

Pediatric Patients ◽

Posterior Circulation ◽

Occipital Artery ◽

Transient Ischemic Attacks ◽

Collateral Vessel ◽

Suboccipital Craniotomy ◽

Uncommon Disease ◽

Indirect Revascularization ◽

The Brain

Moyamoya is an uncommon disease that presents with stenoocclusion of the major vasculature at the base of the brain and associated collateral vessel formation. Many pediatric patients with moyamoya present with transient ischemic attacks or complete occlusions. The authors report the case of a 9-year-old girl who presented with posterior fossa hemorrhage and was treated with an emergency suboccipital craniotomy for evacuation. After emergency surgery, an angiogram was performed, and the patient was diagnosed with moyamoya disease. Six months later, the patient was treated for moyamoya using direct and indirect revascularization; after surgery there was excellent vascularization in both regions of the bypass and no further progression of moyamoya changes. This case illustrates a rare example of intracerebral hemorrhage associated with moyamoya changes in the posterior vascularization in a pediatric patient and subsequent use of direct and indirect revascularization to reduce the risk of future hemorrhage and moyamoya progression.

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Limits and pitfalls of indirect revascularization in moyamoya disease and syndrome

Neurosurgical Review ◽

10.1007/s10143-020-01393-1 ◽

2020 ◽

Author(s):

Pietro Fiaschi ◽

Marcello Scala ◽

Gianluca Piatelli ◽

Domenico Tortora ◽

Francesca Secci ◽

...

Keyword(s):

Internal Carotid ◽

Cerebral Arteries ◽

Surgical Approaches ◽

Cerebrovascular Disorder ◽

Surgical Revascularization ◽

Current State ◽

Middle Cerebral Arteries ◽

Indirect Revascularization ◽

Internal Carotid Arteries ◽

A Current

Abstract Moyamoya vasculopathy is a rare chronic cerebrovascular disorder characterized by the stenosis of the terminal branches of the internal carotid arteries and the proximal tracts of anterior and middle cerebral arteries. Although surgical revascularization does not significantly change the underlying pathogenic mechanisms, it plays a pivotal role in the management of affected individuals, allowing to decrease the risk of ischemic and hemorrhagic complications. Surgical approaches may be direct (extracranial-intracranial bypass), indirect, or a combination of the two. Several indirect techniques classifiable according to the tissue (muscle, periosteum, galea, dura mater, and extracranial tissues) or vessel (artery) used as a source of blood supply are currently available. In this study, we reviewed the pertinent literature and analyzed the advantages, disadvantages, and pitfalls of the most relevant indirect revascularization techniques. We discussed the technical aspects and the therapeutical implications of each procedure, providing a current state-of-the-art overview on the limits and pitfalls of indirect revascularization in the treatment of moyamoya vasculopathy.

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Effectiveness of surgical revascularization for stroke prevention in pediatric patients with sickle cell disease and moyamoya syndrome

Journal of Neurosurgery Pediatrics ◽

10.3171/2017.1.peds16576 ◽

2017 ◽

Vol 20 (3) ◽

pp. 232-238 ◽

Cited By ~ 13

Author(s):

Wuyang Yang ◽

Risheng Xu ◽

Jose L. Porras ◽

Clifford M. Takemoto ◽

Syed Khalid ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Stroke Prevention ◽

Pediatric Patients ◽

Moyamoya Syndrome ◽

Cell Disease ◽

Surgical Revascularization ◽

Transfusion Therapy ◽

Indirect Revascularization

OBJECTIVESickle cell disease (SCD) in combination with moyamoya syndrome (MMS) represents a rare complication of SCD, with potentially devastating neurological outcomes. The effectiveness of surgical revascularization in this patient population is currently unclear. The authors’ aim was to determine the effectiveness of surgical intervention in their series of SCD-MMS patients by comparing stroke recurrence in those undergoing revascularization and those undergoing conservative transfusion therapy.METHODSThe authors performed a retrospective chart review of patients with MMS who were seen at the Johns Hopkins Medical Institution between 1990 and 2013. Pediatric patients (age < 18 years) with confirmed diagnoses of SCD and MMS were included. Intracranial stroke occurrence during the follow-up period was compared between surgically and conservatively managed patients.RESULTSA total of 15 pediatric SCD-MMS patients (28 affected hemispheres) were included in this study, and all were African American. Seven patients (12 hemispheres) were treated with indirect surgical revascularization. The average age at MMS diagnosis was 9.0 ± 4.0 years, and 9 patients (60.0%) were female. Fourteen patients (93.3%) had strokes before diagnosis of MMS, with an average age at first stroke of 6.6 ± 3.9 years. During an average follow-up period of 11.6 years, 4 patients in the conservative treatment group experienced strokes in 5 hemispheres, whereas no patient undergoing the revascularization procedure had any strokes at follow-up (p = 0.029). Three patients experienced immediate postoperative transient ischemic attacks, but all recovered without subsequent strokes.CONCLUSIONSIndirect revascularization is suggested as a safe and effective alternative to the best medical therapy alone in patients with SCD-MMS. High-risk patients managed on a regimen of chronic transfusion should be considered for indirect revascularization to maximize the effect of stroke prevention.

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A Systematic Review of the Omohyoid Muscle Syndrome (OMS): Clinical Presentation, Diagnosis, and Treatment Options

Annals of Otology Rhinology & Laryngology ◽

10.1177/0003489421995037 ◽

2021 ◽

pp. 000348942199503

Author(s):

Jerome Zhiyi Ong ◽

Alex Chengyao Tham ◽

Jian Li Tan

Keyword(s):

Systematic Review ◽

Botulinum Toxin ◽

Traumatic Event ◽

Botulinum Toxin Injection ◽

Treatment Options ◽

Neck Mass ◽

Good Prognosis ◽

Common Symptom ◽

Management Options ◽

Omohyoid Muscle

Objective: Omohyoid muscle syndrome (OMS) is a condition that causes a X-shaped lateral neck lump on swallowing, caused by the failure of the central tendon of the omohyoid muscle to restrict movement of the muscle during swallowing. We aim to review the etiology, pathophysiology, diagnostic tests, and management options for this condition. Data Sources: Pubmed, MEDLINE, EMBASE, and Cochrane databases were searched for all articles and abstracts related to OMS up to 29th July 2020. Review Methods: A systematic review was performed, data extracted from relevant full text articles. Both quantitative data and qualitative data were analyzed. Results: Twenty cases of OMS were reported. Patients presented at a mean age of 36.0. All cases were Asian. There is a 7:3 ratio of males to females. The most common symptom was a transient neck mass. Most cases were managed conservatively with good prognosis. Open or endoscopic transection of the muscle and ultrasound-guided botulinum toxin injection were 3 treatment options, with no recurrence at 4 years, 6 months, and 6 months respectively. Conclusion: OMS could be genetic as all cases were Asian in ethnicity. The deep cervical fascia which usually envelopes the omohyoid muscle may be weakened by stress as 20% of cases had a preceding traumatic event. Real-time ultrasonography establishes the diagnosis, demonstrating the anterolateral displacement of the sternocleidomastoid muscle by a thickened omohyoid muscle during swallowing. Surgical transection can achieve cure, but due to limited studies available, they should be reserved for patients who are extremely bothered. Intramuscular injection of botulinum toxin is an effective alternative, but recurrence is expected.

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Adenomatous Polyps in Adolescent Girl and Boy: A Report of Two Cases

Case Reports in Pathology ◽

10.1155/2016/8256745 ◽

2016 ◽

Vol 2016 ◽

pp. 1-3

Author(s):

Laleh Vahedi Larijani ◽

Maryam Ghasemi ◽

Hassan Karami

Keyword(s):

Pediatric Patients ◽

Colonic Polyps ◽

Definitive Diagnosis ◽

Adenomatous Polyps ◽

Common Symptom ◽

Tubular Adenoma ◽

Tubulovillous Adenoma ◽

Benign Adenoma ◽

Juvenile Type ◽

Rectal Hemorrhage

A polyp is defined as a mass of the mucosal surface that protrudes into the lumen of the gastrointestinal tract. Neoplastic epithelial polyps are classified histologically as either benign adenoma or malignant carcinoma. The colonic polyps that most commonly present in children occur sporadically and individually and are of the juvenile type; they are most frequently associated with painless rectal hemorrhage (which is the most common symptom). Adenomatous polyps are similar to other nontumoral polyps, and it is very rare for children to have symptoms other than rectal bleeding. This report describes two rare cases of polyps in pediatric patients. An 11-year-old girl presented with tubulovillous adenoma and a 13-year-old boy with tubular adenoma; both patients complained of rectal hemorrhage as well as anemia and abdominal pain. Epithelial adenoma is a tumor that is rarely found in adults or children. Colonoscopic perforation and biopsy are mandatory for establishing a definitive diagnosis and avoiding medical mismanagement.

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Tay-Sachs Disease

PEDIATRICS ◽

10.1542/peds.36.3.358 ◽

1965 ◽

Vol 36 (3) ◽

pp. 358-358

Author(s):

ALLEN C. CROCKER

Keyword(s):

Chronic Disease ◽

Pediatric Patients ◽

Single Group ◽

Tay Sachs Disease ◽

Uncommon Disease ◽

History Of ◽

History Of Pediatrics ◽

Disease Hospital

Rarely in this history of pediatrics has a single group of workers so rapidly and effectively brought into focus the special problems of an uncommon disease as have the staff of the Jewish Chronic Disease Hospital in Brooklyn with their studies on Tay-Sachs disease. For a decade they have maintained a steady program of intense interest in this disease led by Drs. Stanley Aronson, Bruno Volk, and Abraham Saifer. A valuable series of personal contributions to the knowledge of this syndrome have resulted, but even more they have stimulated a widespread concern for these pediatric patients in the medical world generally.

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ER stress-mediated BK dysfunction in the DRG underlies pain in a model of multiple sclerosis

10.1101/2020.01.22.915546 ◽

2020 ◽

Author(s):

Muhammad Saad Yousuf ◽

Samira Samtleben ◽

Shawn M. Lamothe ◽

Timothy Friedman ◽

Ana Catuneanu ◽

...

Keyword(s):

Multiple Sclerosis ◽

Er Stress ◽

Treatment Options ◽

Inflammatory Cells ◽

Bk Channels ◽

Resting Membrane Potential ◽

Common Symptom ◽

Chemical Chaperone ◽

Pain Hypersensitivity

ABSTRACTNeuropathic pain is a common symptom of multiple sclerosis (MS) and current treatment options are ineffective. In this study, we investigated whether endoplasmic reticulum (ER) stress in dorsal root ganglia (DRG) contributes to pain hypersensitivity in the experimental autoimmune encephalomyelitis (EAE) mouse model of MS. Inflammatory cells and increased levels of ER stress markers are evident in post-mortem DRGs from MS patients. Similarly, we observed ER stress in the DRG of mice with EAE and relieving ER stress with a chemical chaperone, 4-phenylbutyric acid (4-PBA), reduced pain hypersensitivity. In vitro, 4-PBA and the selective PERK inhibitor, AMG44, normalize cytosolic Ca2+ transients in putative DRG nociceptors. We went to assess disease-mediated changes in the functional properties of Ca2+-sensitive BK-type K+ channels in DRG neurons. We found that the conductance-voltage (GV) relationship of BK channels was shifted to a more positive voltage, together with a more depolarized resting membrane potential in EAE cells. Our results suggest that ER stress in sensory neurons of MS patients and mice with EAE is a source of pain and that ER stress modulators can effectively counteract this phenotype.

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Clinical study of multiple myeloma and treatment responses in a tertiary care centre

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20214417 ◽

2021 ◽

Vol 9 (11) ◽

pp. 3356

Author(s):

Immanni S. M. Giridhar ◽

C. Deepak Yadlapalli ◽

Muralidhar Gullipalli ◽

Venkatesh Mushini ◽

Yerraguntla S. Sarma ◽

...

Keyword(s):

Multiple Myeloma ◽

Treatment Options ◽

Tertiary Care ◽

Elevated Serum ◽

Staging System ◽

Stage Iiib ◽

Common Symptom ◽

Tertiary Care Centre ◽

Incurable Disease ◽

Unknown Significance

Background: Multiple myeloma (MM) evolves from Monoclonal gammopathy of unknown significance (MGUS), a premalignant clinical condition. Second to non-Hodgkin’s lymphoma, MM is the most common haematological malignancy. The aim of the study was to review the clinical profile and response of individuals treated for MM from this part of country.Methods: We evaluated data of patients with MM managed between 2013 and 2019 at a tertiary care cancer hospital in Rajamahenderi, India. Data regarding demographic variables, clinical features, disease characteristics and treatment details were collected and analysed.Results: Total of 54 patients with MM were managed. Mean age was 59.4 years. Males accounted for 63%. Bone pain (90%) was the most common symptom. Elevated serum creatinine was noted in 16.7% and M band in 42 (77.8%). X-ray of skull showed lytic lesions in 41 (75.9%). Mean haemoglobin value was 8.8±1.9 g/dl and serum calcium was 9.12 mg/dl. Majority of subjects, 44 (81.48%) belong to stage IIIA, 9 (16.67%) to stage IIIB, and 1.85% to stage IIA of Durie Salmon staging system. No response was noted in 17 (31.5%), 4 (7.4%) subjects had a progressive disease even on treatment, and 8 (14.8%) subjects had a very good partial response. Median survival of subjects belonging to DSS stage II was 17 months, IIIA was 11.037 months and stage IIIB was 17.463 months.Conclusions: MM has an early onset in India. Though MM is an incurable disease, many promising treatment options are there which lead to increase in survival. Early treatment helps in improving mortality rates, better quality of life and decreases disease burden.

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Single and staged laser interstitial thermal therapy ablation for cortical tubers causing refractory epilepsy in pediatric patients

Neurosurgical FOCUS ◽

10.3171/2018.6.focus18228 ◽

2018 ◽

Vol 45 (3) ◽

pp. E9 ◽

Cited By ~ 13

Author(s):

Zulma Tovar-Spinoza ◽

Robert Ziechmann ◽

Stephanie Zyck

Keyword(s):

Laser Ablation ◽

Minimally Invasive ◽

Pediatric Patients ◽

Refractory Epilepsy ◽

Neuropsychiatric Symptoms ◽

Thermal Therapy ◽

Seizure Outcome ◽

Laser Interstitial Thermal Therapy ◽

Cortical Tubers ◽

The Mean

OBJECTIVEMagnetic resonance–guided laser interstitial thermal therapy (MRgLITT) is a novel, minimally invasive treatment for the surgical treatment of epilepsy. In this paper, the authors report on clinical outcomes for a series of pediatric patients with tuberous sclerosis complex (TSC) and medication-refractory epileptogenic cortical tubers.METHODSA retrospective chart review was performed at SUNY Upstate Golisano Children’s Hospital in Syracuse, New York. The authors included all cases involving pediatric patients (< 18 years) who underwent MRgLITT for ablation of epileptogenic cortical tubers between February 2013 and November 2015.RESULTSSeven patients with cortical tubers were treated (4 female and 3 male). The patients’ average age was 6.6 years (range 2–17 years). Two patients had a single procedure, and 5 patients had staged procedures. The mean time between procedures in the staged cases was 6 months. All of the patients had a meaningful reduction in seizure frequency as reported by Engel and ILAE seizure outcome classifications, and most (71.4%) of the patients experienced a reduction in AED burden. Three of the 4 patients who presented with neuropsychiatric symptoms had some improvement in these domains after laser ablation. No perioperative complications were noted. The mean duration of follow-up was 19.3 months (range 4–49 months).CONCLUSIONSLaser ablation represents a minimally invasive alternative to resective epilepsy surgery and is an effective treatment for refractory epilepsy due to cortical tubers.

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Approach to the Assessment and Management of Pediatric Patients With Atopic Dermatitis: A Consensus Document. Section III: Treatment Options for Pediatric Atopic Dermatitis

Journal of Cutaneous Medicine and Surgery ◽

10.1177/1203475419882647 ◽

2019 ◽

Vol 23 (5_suppl) ◽

pp. 19S-31S

Author(s):

Perla Lansang ◽

Joseph M. Lam ◽

Danielle Marcoux ◽

Vimal H. Prajapati ◽

Shanna Spring ◽

...

Keyword(s):

Atopic Dermatitis ◽

Maintenance Therapy ◽

Pediatric Patients ◽

Treatment Options ◽

Skin Barrier ◽

Active Therapy ◽

Disease Treatment ◽

Consensus Document ◽

Emerging Therapies ◽

Clinical Considerations

Because atopic dermatitis (AD) is a chronic, relapsing disease, treatment requires the use of both active therapy to control flares and preventative maintenance therapy to promote integrity of the skin barrier. In this third of four sections, important clinical considerations for the treatment of pediatric AD are reviewed. Emerging therapies in development for pediatric AD are introduced.

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