Another Rare Unstable Hemoglobinopathy: Hemoglobin Casper/Southampton Associated with Moyamoya Disease

Haque, Asim; Quint, Douglas J.; Castle, Valerie P.; Leber, Steven M.

doi:10.1159/000381331

Cited by 10 publications

(6 citation statements)

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“…MMD is pathophysiologically complex. Fragile MMD vessels and unstable hemodynamics result in a very high surgical risk, and direct bypass is associated with a high incidence of surgical complications, especially in patients with unstable MMD 14 , 15 . In 2005, Kim et al found that MMD surgery performed within 6 weeks after the last ischemic infarction resulted in a higher incidence of postoperative ischemic complications 16 .…”

Section: Introductionmentioning

confidence: 99%

Progress on Complications of Direct Bypass for Moyamoya Disease

Shi

Guo

et al. 2016

Int. J. Med. Sci.

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Moyamoya disease (MMD) involves progressive occlusion of the intracranial internal carotid artery resulting in formation of moyamoya-like vessels at the base of the brain. It can be characterized by hemorrhage or ischemia. Direct vascular bypass is the main and most effective treatment of MMD. However, patients with MMD differ from those with normal cerebral vessels. MMD patients have unstable intracranial artery hemodynamics and a poor blood flow reserve; therefore, during the direct bypass of superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis, perioperative risk factors and anesthesia can affect the hemodynamics of these patients. When brain tissue cannot tolerate a high blood flow rate, it becomes prone to hyperperfusion syndrome, which leads to neurological function defects and can even cause intracranial hemorrhage in severe cases. The brain tissue is prone to infarction when hemodynamic equilibrium is affected. In addition, bypass vessels become susceptible to occlusion or atrophy when blood resistance increases. Even compression of the temporalis affects bypass vessels. Because the STA is used in MMD surgery, the scalp becomes ischemic and is likely to develop necrosis and infection. These complications of MMD surgery are difficult to manage and are not well understood. To date, no systematic studies of the complications that occur after direct bypass in MMD have been performed, and reported complications are hidden among various case studies; therefore, this paper presents a review and summary of the literature in PubMed on the complications of direct bypass in MMD.

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Section: Introductionmentioning

confidence: 99%

Progress on Complications of Direct Bypass for Moyamoya Disease

Shi

Guo

et al. 2016

Int. J. Med. Sci.

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“…A case of hemoglobin E [6], hemoglobin aleisha [7], hemoglobin Fairfax [8], and 2 cases associated with hemoglobin casper [9,10]. All above reported cases are in children.…”

Section: Discussionmentioning

confidence: 92%

Moyamoya Syndrome with Recurrent Intraparenchymal Hemorrhage from Hemoglobin C Disease

Aradine¹,

Aubert²,

Bayrlee³

2017

J Neurol Disord

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“…[1][2][3][4][5][6][7][8] Reticulocytes were unable to be quantified due to numerous eryth- The findings in this case are suggestive of chronic intracranial ischemia. [1][2][3][4][5][6][7][8] Reticulocytes were unable to be quantified due to numerous eryth- The findings in this case are suggestive of chronic intracranial ischemia.…”

Section: Case Presentationmentioning

confidence: 99%

“…Reported in at least seven individuals, most commonly as a de novo mutation, Hb Southampton is a rare, unstable β-globin variant [HBB:c.320T>C(p.Leu107Pro)]. [1][2][3][4][5][6][7][8] The findings in this case are suggestive of chronic intracranial ischemia. Moyamoya syndrome is a well-recognized sequela of sickle cell disease (SCD) but has also been reported in unstable hemoglobinopathies including Hb Southampton, 1,2 Hb Alesha, 10 and Hb Fairfax (in association with beta thalassemia).…”

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confidence: 99%