Moyamoya Disease Associated With Hereditary Spherocytosis

Van, Philippe Vo; Sabouraud, Pascal; Mac, Gratiela; Abély, M.; Bednarek, Nathalie

doi:10.1016/j.pediatrneurol.2010.08.002

Cited by 7 publications

(4 citation statements)

References 13 publications

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“…Three cases of moyamoya syndrome and its association with hereditary spherocytosis have so far been reported [3–5]. Since our case is the fourth one with such an association, the foremost question that arises in one's mind is if this association of moyamoya disease and hereditary spherocytosis could be an incidental finding or this coincidence could be explainable on definite physiopathological grounds.…”

Section: Discussionmentioning

confidence: 79%

Laparoscopic Splenectomy in a Child with Moyamoya Syndrome, Hereditary Spherocytosis, and Interstitial Lung Disease: A Mere Coincidence or Partnership Based on Genetic Similarities

Karvandian

Khan²,

Zebardast³

et al. 2011

Case Reports in Anesthesiology

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A case of moyamoya syndrome and spherocytosis with concurrent interstitial lung disease who underwent laparoscopic splenectomy is being reported. A theory regarding their coexistence is being forwarded together with their anesthetic management. According to our search, this is the fourth case of moyamoya syndrome and the first case with an associated interstitial lung disease in a 10-year-old child.

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Section: Discussionmentioning

confidence: 79%

Laparoscopic Splenectomy in a Child with Moyamoya Syndrome, Hereditary Spherocytosis, and Interstitial Lung Disease: A Mere Coincidence or Partnership Based on Genetic Similarities

Karvandian

Khan²,

Zebardast³

et al. 2011

Case Reports in Anesthesiology

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“…Early diagnosis and management of this potentially treatable condition is pivotal. 18 Pyruvate kinase deficiency 19 Paroxysmal Nocturnal Hemoglobulinuria 20 Fanconi anemia 21 Congenital and acquired thrombotic thrombocytopenic purpura 10,11 Hemolytic uremic syndrome 12…”

Section: Discussionmentioning

confidence: 99%

Congenital Thrombotic Thrombocytopenic Purpura Associated With Moyamoya Syndrome in a 3-Year-Old Girl

et al. 2012

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A 3-year-old girl who presented with anemia, thrombocytopenia, and recurrent strokes is described. The cerebral angiography revealed moyamoya vasculopathy. Her younger brother also had anemia and thrombocytopenia but no neurologic abnormalities. Both children had severe deficiency of ADAMTS13 (A Disintegrin And Metalloprotease with Thrombospondin like domain activity) confirming the diagnosis of congenital thrombotic thrombocytopenic purpura. The children responded well to regular fresh-frozen plasma infusions. This report expands the spectrum of hematologic diseases associated with moyamoya syndrome. Unexplained thrombocytopenia, especially in the presence of neurologic symptoms, should prompt an evaluation for ADAMTS13deficiency. The diagnosis has significant implications not only for therapy but also for genetic counseling.

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“…Vo Van P et al reported a 5-year-old patient with hereditary spherocytosis. The patient received an MRA exanimation due to a transient ischemic attack (TIA) and was found to have intracranial MMD-like lesions 46 . Ramesh K et al examined a 14-month-old infant with MMD and suggested that the onset was related to renal agenesis and external iliac artery stenosis.…”

Section: Possible Pathogenic Mechanisms Of MMD In Childrenmentioning

confidence: 99%

Research Progress of Moyamoya Disease in Children

Piao¹,

Wu²,

Yang³

et al. 2015

Int. J. Med. Sci.

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During the onset of Moyamoya disease (MMD), progressive occlusion occurs at the end of the intracranial internal carotid artery, and compensatory net-like abnormal vessels develop in the skull base, generating the corresponding clinical symptoms. MMD can affect both children and adults, but MMD in pediatric patients exhibits distinct clinical features, and the treatment prognoses are different from adult patients. Children are the group at highest risk for MMD. In children, the disease mainly manifests as ischemia, while bleeding is the primary symptom in adults. The pathogenesis of MMD in children is still unknown, and some factors are distinct from those in adults. MMD in children could result in progressive, irreversible nerve functional impairment, and an earlier the onset corresponds to a worse prognosis. Therefore, active treatment at an early stage is highly recommended. The treatment methods for MMD in children mainly include indirect and direct surgeries. Indirect surgeries mainly include multiple burr-hole surgery (MBHS), encephalomyosynangiosis (EMS), and encephaloduroarteriosynangiosis (EDAS); direct surgeries mainly include intra- and extracranial vascular reconstructions that primarily consist of superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis. Indirect surgery, as a treatment for MMD in children, has shown a certain level of efficacy. However, a standard treatment approach should combine both indirect and direct procedures. Compared to MMD in adults, the treatment and prognosis of MMD in children has higher clinical significance. If the treatment is adequate, a satisfactory outcome is often achieved.

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Moyamoya Disease Associated With Hereditary Spherocytosis

Cited by 7 publications

References 13 publications

Laparoscopic Splenectomy in a Child with Moyamoya Syndrome, Hereditary Spherocytosis, and Interstitial Lung Disease: A Mere Coincidence or Partnership Based on Genetic Similarities

Laparoscopic Splenectomy in a Child with Moyamoya Syndrome, Hereditary Spherocytosis, and Interstitial Lung Disease: A Mere Coincidence or Partnership Based on Genetic Similarities

Congenital Thrombotic Thrombocytopenic Purpura Associated With Moyamoya Syndrome in a 3-Year-Old Girl

Research Progress of Moyamoya Disease in Children

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