腹腔镜下成功切除罕见的局灶性先天性高胰岛素血症的临床病例及文献综述

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先天性高胰岛素血症是一种严重的疾病，它导致大脑皮层的不可逆性损伤。本文介绍了对一名患有极为罕见的高胰岛素血症的儿童成功进行手术治疗的临床观察。该患儿在胰腺两端里有两 个病灶。自出生以来，由于反复出现低血糖，该患儿的情况一直很危重。该患儿在2周大的时候被诊断为高胰岛素血症。开始了使用二氮嗪和奥曲肽进行特殊治疗。但事实证明了，这种治疗是无效的。把葡萄糖注射到静脉中的需求仍然很高。药物奥曲肽只产生了部分效果。在孩子1个月大的时候， 对患儿进行了正电子发射计算机断层扫描结合18F-DOPA计算机断层扫描。在胰腺的尾部和头部发现了放射性药物的局灶性超固定。这可能表明，存在两个病理病灶或尾部有一个病灶，头部有放射性药物的生理性积累。在孩子1个月零20天大的时候，患儿被带到了手术室。当对胰腺进行腹腔镜检查时，发现了尾部有一个病灶。快速活检的数据证实了该病灶。进行了尾部切除术（15%），并确认了切除边缘干净。进一步检查发现了头部有一个病灶。它被切除了。通过组织学检查确认了切除的完整性。患儿出院时没有接受任何治疗，术后第12天就实现了临床康复。对该孩子正在进行随访工 作（超过6个月）。患儿随着年龄增长和发育。已经进行了禁食试验。排除了高胰岛素血症的复发。 文章中详细描述了临床和放射学检查方法的结果、详细的病史及手术治疗技术。对手术的结果进行了分析。在本文中在讨论的框架内分析了关于对患有先天性高胰岛素血症的儿童进行腹腔镜胰腺切除术的文献数据。

作者简介

Anna A. Sukhotskaya

Almazov National Medical Research Center

Email: dxirurgia@mail.ru
ORCID iD: 0000-0002-8734-2227
SPIN 代码: 6863-7436

MD, Cand. Sci. (Med.), assistant professor

俄罗斯联邦, Saint Petersburg

Ilya M. Kagantsov

Almazov National Medical Research Center

Email: ilkagan@rambler.ru
ORCID iD: 0000-0002-3957-1615
SPIN 代码: 7936-8722

MD, Dr. Sci. (Med.), assistant professor

俄罗斯联邦, Saint Petersburg

Vladimir G. Bairov

Almazov National Medical Research Center

Email: vbairov@gmail.com
ORCID iD: 0000-0002-8446-830X
SPIN 代码: 6025-8991

MD, Dr. Sci. (Med.), Professor

俄罗斯联邦, Saint Petersburg

Mikhail G. Vitovshchik

Almazov National Medical Research Center

Email: mg@vitovshchik.ru
ORCID iD: 0000-0002-7795-8108
SPIN 代码: 2992-1533

pediatric surgeon

俄罗斯联邦, Saint Petersburg

Irina L. Nikitina

Almazov National Medical Research Center

Email: Nikitina_IL@almazovcentre.ru
ORCID iD: 0000-0003-4013-0785
SPIN 代码: 7707-4939

MD, Dr. Sci. (Med.), Professor

俄罗斯联邦, Saint Petersburg

Daria V. Ryzhkova

Almazov National Medical Research Center

Email: Ryzhkova_DV@almazovcentre.ru
ORCID iD: 0000-0002-7086-9153
SPIN 代码: 7567-6920

Dr. Sci. (Med.)

俄罗斯联邦, Saint Petersburg

Lubov B. Mitrofanova

Almazov National Medical Research Center

Email: Mitrofanova_LB@almazovcentre.ru
ORCID iD: 0000-0003-0735-7822
SPIN 代码: 9552-8248

Dr. Sci. (Med.)

俄罗斯联邦, Saint Petersburg

Leila R. Sarakaeva

Almazov National Medical Research Center

编辑信件的主要联系方式.
Email: sarale723@gmail.com
ORCID iD: 0000-0002-2752-861X
SPIN 代码: 5536-4623
俄罗斯联邦, Saint Petersburg

参考

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2. Fig. 1. Combined positron emission tomography and computed tomography with 18F-DOPA for the patient. 1 — a focus in the tail of the pancreas, 2 — a focus in the head of the pancreas, 3 — accumulation of radiopharmaceutical in the kidneys, and 4 — accumulation of radiopharmaceutical in the bladder

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3. Fig. 2. Fixation of the stomach to the anterior abdominal wall with traction sutures: a — process of fixing the large curvature of the stomach to the anterior abdominal wall and b — traction seam is completed

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4. Fig. 3. Focus of pathological tissues in the tail of the pancreas

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5. Fig. 4. Mobilization and resection of the tail of the pancreas

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6. Fig. 5. Sealing of the stump of the tail of the pancreas

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7. Fig. 6. Focus in the head of the pancreas

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8. Fig. 7. Resection of the focus of hyperinsulinism from the head of the pancreas: а — resection of the focus of pathological tissues and b — area of the head of the pancreas after resection (the bed of the focus)

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