Effect of Revascularization on Headache Associated with Moyamoya Disease in Pediatric Patients

Episodic headache is common in childhood moyamoya disease (MMD). The onset, mechanism, cause of headache and the effect of revascularization surgery on headache are not yet clear. We studied 10 cases of children (7 boys and 3 girls) younger than 18 years who underwent revascularization for MMD between 2009 and 2013. We evaluated frequency of headache and cerebral blood flow changes by single photon emission computed tomography brain imaging with [I123]-labeled iofetamine (IMP­SPECT) before and after surgery. Patients’ ages ranged from 0 to 15 years at onset and 2 to 17 years at the time of surgery, mean age being 6.7 and 8.0 years respectively. 9 of 10 patients presented with ischemic symptoms and 8 had headache. 5 patients underwent indirect bypass and 5 underwent combined direct and indirect bypass. Cerebral blood flow improvement was obtained in 14 of the 15 cerebral hemispheres revascularized mean follow-up duration was 32.9 months. All the patients had good outcomes with improvement of ischemic neurological deficits. Headache improved in 7 (87.5%) of 8 patients. Headache in pediatric moyamoya disease is associated with change in cerebral hemodynamics. Revascularization including combined direct bypass and indirect techniques may be required to reduce headache in patients with MMD

Solitary Cranial Langerhans Cell Histiocytosis : Two case reports

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. LCH has an excellent prognosis when treated with surgical resection, steroids and radiotherapy or chemotherapy. One of our patients is disease free at 7 year follow-up and one patient had regression of lesion on follow-up

Severe growth hormone deficiency is rare in surgically-cured acromegalics

The Author(s) 2012. This article is published with open access at Springerlink.com Abstract Growth hormone deficiency (GHD) in surgi-cally-cured acromegalics has been reported to negatively affect their metabolic condition and quality of life (QOL). The incidence of GHD, its causes, and its effects on their physio-psychological condition remain to be examined in detail. We performed a retrospective study to investigate GH secretory function in surgically-cured acromegalics, prognostic factors of GHD, and its impact on QOL. The study population consisted of 72 acromegalics who were determined to be surgically cured according to the Cortina consensus criteria. We recorded the incidence of impaired GH secretory function based on the peak GH level during postoperative insulin tolerance test (ITT) which lowered their nadir blood sugar to under 50 mg/dL. Their QOL was evaluated by SF-36. In surgically-cured acromegalics, the incidence of severe GHD (peak GH during ITT2 3.0 lg/L) was 12.5 % (9/72). The preoperative tumor size was sig-nificantly larger in patients with severe GHD than without severe GHD (21.9 ± 9.0 vs. 15.5 ± 7.1 mm, p = 0.017). The peak GH levels during postoperative ITT were statistically correlated with the physical but not the mental component summary of the SF-36 score. The incidence of GHD was 12.5 % in our surgically-cured acromegalics. As some QOL aspects are positively related with peak GH levels during postoperative ITT, efforts should be made to preserve pituitary function in acromegalic patients under-going adenomectomy