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一名5个月大女孩脑干海绵状血管瘤再出血的罕见病例。

A rare case of rebleeding brainstem cavernoma in a 5-month-old-girl.

作者信息

Ndandja Dimitri T K, Musa Gerald, Nosov Ilya, Chmutin Gennady E, Livshitz Matvey I, Annanepesov Nazar S, Mwela Bupe Mumba

机构信息

Department of Neurological Diseases and Neurosurgery, Peoples' Friendship University of Russia, Moscow, Russia.

Department of Neurosurgery, Morozov Children's City Clinical Hospital, Center for the Treatment of Cerebrovascular Pathology in Children and Adolescents, Moscow, Russian Federation.

出版信息

Surg Neurol Int. 2023 Jun 30;14:227. doi: 10.25259/SNI_308_2023. eCollection 2023.

Abstract

BACKGROUND

Brainstem cavernomas (BSCs) are relatively rare intracranial vascular lesions that, if left untreated, can be devastating to the patient. The lesions are associated with a myriad of symptoms, depending on their size and location. However, medullary lesions present acutely with cardiorespiratory dysfunction. We present the case of a 5-month-old child with a BSC.

CASE DESCRIPTION

A 5-month-old child presented for the 2 time with sudden respiratory distress and excessive salivation. On the first presentation, brain magnetic resonance imaging (MRI) showed a 13 × 12 × 14 mm cavernoma at the pontomedullary junction. She was managed conservatively but presented 3 months later with tetraparesis, bulbar palsy, and severe respiratory distress. A repeat MRI showed enlargement of the cavernoma to 27 × 28 × 26 mm with hemorrhage in different stages. After hemodynamic stabilization, complete cavernoma resection was performed through the telovelar approach with neuromonitoring. Postoperatively, the child recovered motor function, but the bulbar syndrome persisted with hypersalivation. She was discharged on day 55 with a tracheostomy.

CONCLUSION

BSCs are rare lesions that are associated with severe neurological deficits due to the compactness of important cranial nerve nuclei and other tracts in the brainstem. Early surgical excision and hematoma evacuation for superficially presenting lesions can be lifesaving. However, the risk of postoperative neurological deficits is still a major concern in these patients.

摘要

背景

脑干海绵状血管瘤(BSCs)是相对罕见的颅内血管病变,若不治疗,可对患者造成严重损害。这些病变会引发多种症状,具体取决于其大小和位置。然而,延髓病变会急性出现心肺功能障碍。我们报告一例5个月大患有脑干海绵状血管瘤的患儿。

病例描述

一名5个月大的患儿因突发呼吸窘迫和流涎过多第二次就诊。首次就诊时,脑部磁共振成像(MRI)显示在脑桥延髓交界处有一个13×12×14毫米的海绵状血管瘤。她接受了保守治疗,但3个月后出现四肢轻瘫、延髓麻痹和严重呼吸窘迫。复查MRI显示海绵状血管瘤增大至27×28×26毫米,伴有不同阶段的出血。在血流动力学稳定后,通过经小脑幕入路并借助神经监测进行了海绵状血管瘤全切术。术后,患儿恢复了运动功能,但延髓综合征持续存在且流涎过多。她在第55天带着气管造口出院。

结论

脑干海绵状血管瘤是罕见病变,由于脑干中重要颅神经核和其他神经束紧密排列,会导致严重神经功能缺损。对于表浅的病变,早期手术切除和血肿清除可能挽救生命。然而,术后神经功能缺损的风险仍是这些患者的主要担忧。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a1ea/10316179/c56545067d33/SNI-14-227-g001.jpg

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