• Clinical and Experimental Pediatrics
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• 제53권10호
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• pp.913-916
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• 2010

Congenital factor VII deficiency is a rare autosomal-recessive bleeding disorder. Bleeding manifestations and clinical findings vary widely, ranging from asymptomatic subjects to patients with hemorrhages that may cause significant handicaps. Treatment has traditionally involved factor VII(FVII) replacement therapy using fresh frozen plasma, prothrombin complex concentrates or plasma-derived FVII concentrates. Recombinant activated FVII ($NovoSeven^{(R)}$) is currently considered the first-line treatment for replacement therapy of FVII deficiency. Here we present a case of severe intracerebral and intraventricular hemorrhage in a neonate with congenital FVII deficiency.

• Journal of Cerebrovascular and Endovascular Neurosurgery
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• 제25권1호
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• pp.69-74
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• 2023

Subarachnoid hemorrhage (SAH) due to ruptured posterior cerebral artery (PCA) intracranial arterial dolichoectasia (IADE) is very rare. As these lesions are difficult to treat microsurgically, neurointervention is preferred because the dolichoectatic artery does not have a clear neck, and the surgical field of view was deep seated with the SAH. However, in some cases, neurointervention is difficult due to anatomical variation of the blood vessel to access the lesion. In this case, a 30-year-old male patient presented with a ruptured PCA IADE and an aortic arch anomaly. Aortic arch anomalies render it difficult to reach the ruptured PCA IADE via endovascular treatment. The orifice of the vertebral artery (VA) was different from the usual cases, so it was difficult to find the entrance. After only finding the VA and arriving at the lesion along the VA, trapping was performed. Herein, we report the PCA IADE with aortic arch anomaly endovascular treatment methods and results.

• Investigative Magnetic Resonance Imaging
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• 제25권1호
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• pp.43-46
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• 2021

Arachnoid cysts are benign lesions that are formed between the inner and outer layers of the arachnoid, accounting for 1% of all intracranial space occupying lesions. Usually, arachnoid cysts are asymptomatic. It can be symptomatic in case of complication such as intracystic hemorrhage or acute cyst expansion. We present a case of incidentally prediagnosed arachnoid cyst which undergone intracystic hemorrhage combined with ipsilateral SDH in a young male.

• Korean Journal of Radiology
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• 제2권2호
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• pp.105-107
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• 2001

Meningiomas causing intracranial hemorrhage are rare, and hemorrhage from a lateral ventricular meningioma seems to be even rarer. We report a case of trigonal meningioma in a 43-year-old woman who presented with intraventricular hemorrhage, and describe the CT, MRI and angiographic findings.

• Journal of Yeungnam Medical Science
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• 제29권1호
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• pp.42-44
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• 2012

Plasma cell myelomas generally manifest as bone or soft-tissue tumors with variable mass effects, pain, and infiltrative behavior. Extramedullary involvement occurs most commonly in the spleen, liver, lymph nodes, and kidneys, but intracranial involvement in plasma cell myeloma is a rare extramedullary manifestation. These authors recently encountered a case of intracranial involvement of plasma cell myeloma. A 69-year-old man was hospitalized for headache and mental changes. Brain CT showed subdural hemorrhage caused by plasma cell myeloma. Plasma cell myeloma with intracranial involvement has poor prognosis, and the patient in this case died from acute complications, such as subdural hemorrhage. Based on this case report, it is suggested that more effective treatment regimens of plasma cell myeloma with intracranial involvement be developed. Moreover, a screening method and decision on the appropriate time for intracranial involvement are needed for plasma cell myeloma patients.

• Journal of Korean Neurosurgical Society
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• 제50권1호
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• pp.6-10
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• 2011

Objective : Spontaneous acute subdural hematomas (aSDH) secondary to ruptured intracranial aneurysms are rarely reported. This report reviews the clinical features, diagnostic modalities, treatments, and outcomes of this unusual and often fatal condition. Methods : We performed a database search for all cases of intracranial aneurysms treated at our hospital between 2005 and 2010. Patients with ruptured intracranial aneurysms who presented with aSDH on initial computed tomography (CT) were selected for inclusion. The clinical conditions, radiologic findings, treatments, and outcomes were assessed. Results : A total of 551 patients were treated for ruptured intracranial aneurysms during the review period. We selected 23 patients (4.2%) who presented with spontaneous aSDH on initial CT. Ruptured aneurysms were detected on initial 3D-CT angiography in all cases. All ruptured aneurysms were located in the anterior portion of the circle of Willis. The World Federation of Neurosurgical Societies grade on admission was V in 17 cases (73.9%). Immediate decompressive craniotomy was performed 22 cases (95.7%). Obliteration of the ruptured aneurysm was achieved in all cases. The Glasgow outcome scales for the cases were good recovery in 5 cases (21.7%), moderate disability to vegetative in 7 cases (30.4%), and death in 11 cases (47.8%). Conclusion : Spontaneous aSDH caused by a ruptured intracranial aneurysm is rare pattern of aneurysmal subarachnoid hemorrhage. For early detection of aneurysm, 3D-CT angiography is useful. Early decompression with obliteration of the aneurysm is recommended. Outcomes were correlated with the clinical grade and CT findings on admission.

• Journal of Korean Neurosurgical Society
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• 제51권6호
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• pp.367-369
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• 2012

Terson syndrome was originally used to describe a vitreous hemorrhage arising from aneurysmal subrarachnoid hemorrhage. Terson syndrome can be caused by intracranial hemorrhage, subdural or epidural hematoma and severe brain injury but is extremely rare in intraventricular hemorrhage associated with moyamoya disease. A 41-year-old man presented with left visual disturbance. He had a history of intraventicular hemorrhage associated with moyamoya disease three months prior to admission. At that time he was in comatose mentality. Ophthalmologic examination at our hospital detected a vitreous hemorrhage in his left eye, with right eye remaining normal. Vitrectomy with epiretinal membrane removal was performed. After operation his left visual acuity was recovered. Careful ophthalmologic examination is mandatory in patients with hemorrhagic moyamoya disease.

• Journal of Korean Neurosurgical Society
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• 제48권6호
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• pp.480-484
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• 2010

Objective : To evaluate the role of lumbar drainage in the prevention of shunt-dependent hydrocephalus after treatment of ruptured intracranial aneurysms by coil embolization in good-grade patients. Methods : One-hundred-thirty consecutive patients with aneurysmal subarachnoid hemorrhage in good-grade patients (Hunt & Hess grades I-III), who were treated by coil embolization between August 2004 and April 2010 were retrospectively evaluated. Poor-grade patients (Hunt & Hess grades IV and V), a history of head trauma preceding the development of headache, negative angiograms, primary subarachnoid hemorrhage (SAH), and loss to follow-up were excluded from the study. We assessed the effects on lumbar drainage on the risk of shunt-dependent hydrocephalus related to coil embolization in patients with ruptured intracranial aneurysms. Results : One-hundred-twenty-six patients (96.9%) did not develop shunt-dependent hydrocephalus. The 2 patients (1.5%) who developed acute hydrocephalus treated with temporary external ventricular drainage did not require permanent shunt diversion. Overall, 4 patients (3.1%) required permanent shunt diversion; acute hydrocephalus developed in 2 patients (50%). There was no morbidity or mortality amongst the patients who underwent a permanent shunt procedure. Conclusion : Coil embolization of ruptured intracranial aneurysms may be associated with a lower risk for developing shunt-dependent hydrocephalus, possibly by active management of lumbar drainage, which may reflect less damage for cisternal anatomy than surgical clipping. Coil embolization might have an effect the long-term outcome and decision-making for ruptured intracranial aneurysms.

• Advances in pediatric surgery
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• 제1권1호
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• pp.63-67
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• 1995

Although bleeding was reported before as the first symptom in a few cases of biliary atresia, this association is not generally known. We treated 115-day-old female with extrahepatic biliary atresia presented with subdural hemorrhage rather than with prolonged jaundice. Four years after craniectomy and Kasai's hepatic portojejunostomy, she looks happy without jaundice nor brain damage sequela even though she had recent episode of esophageal variceal bleeding.

• Clinical and Experimental Pediatrics
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• 제46권11호
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• pp.1067-1072
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• 2003

목 적 : 소아에서 사고에 의하지 않은(non-accidental) 두개내출혈은 빈도가 아주 드물지 않으며 성인과 다른 임상 양상을 보이고 높은 사망률과 심각한 후유증을 남긴다. 특히 흔들린 아이증후군은 소아에서만 있는 두개내출혈로 최근 본원에서 여려 사례를 경험하였다. 이에 저자들은 사고에 의하지 않은 두개내 출혈로 한림대학교성심병원에 입원한 소아 환아들을 대상으로 성별, 연령 분포, 원인, 내원 당시 임상 증상, 출혈 유형, 사망률과 후유증에 대해 조사하였다. 방 법 : 1999년 1월부터 2002년 6월까지 3년 6개월간 한림대학교 성심병원 소아과와 신경외과에 사고에 의하지 않은 두개내출혈로 입원한 15세 이하의 환아 중 신생아의 두개내출혈 환아를 제외한 20례를 대상으로 병력지를 검토하여 후향적으로 분석하였다. 결 과 : 1) 남녀비는 1 : 0.8이었고 연령 분포는 1세 미만 9례(45%), 1-5세 2례(10%), 6-10세 3례(15%), 11-15세 6례(30%)로 1세 미만과 11-15세에서 가장 많이 분포하였다. 2) 원인은 흔들린 아이 증후군 5례(25%), 동정맥 기형 5례(25%), 해면상 혈관종 2례(10%), 뇌종양 2례(10%), 동맥류 1례(5%), 원인 질환이 밝혀지지 않은 경우가 5례(25%)로 흔들린 아이 증후군과 동정맥 기형이 가장 많았다. 3) 내원 당시 임상 증상으로 경련 11례(55%), 의식 변화 6례(30%), 구토 5례(25%), 두통 3례(15%), 편측 부전마비 1례(5%), 보챔 1례(5%), 안면 경축 1례(5%)로 경련이 가장 많았다. 4) 출혈 유형은 경막하 출혈 8례(40%), 뇌내 출혈 5례(25%), 복합 두개내 출혈 5례(25%), 지주막하 출혈 2례(10%)였다. 5) 대상 환아 20례 중 사망률은 3례로 15%였고 생존율은 17례로 85%였다. 생존아 중 추적 관찰이 되지 않은 5례를 제외한 12례에서 완전 회복된 경우가 6례였으며 후유증이 남은 경우가 6례였다. 후유증이 남은 6례 중 편측 부전마비 2례, 동측성 반맹 2례, 발달 장애 2례, 사지 마비 2례, 단마비 1례, 학습 장애 1례, 시력 장애 1례였다. 결 론 : 소아에서 사고에 의하지 않은 두개내 출혈의 발생률은 아주 낮지 않으며 사망률과 후유증 발생 가능성이 높기 때문에 조기 진단과 적절한 치료가 필요하다. 또한 최근 의학의 발달로 생존율이 높아지면서 신경학적 후유증을 가진 소아들이 늘어남에 따라 이들에 대한 적극적인 재활 관리가 필요하다고 생각된다.