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腹部淋巴管畸形:一种新的治疗方法

Abdominal Lymphatic Malformations: A Novel Approach in Management.

作者信息

Karunaratne Nuwanthika, Minhas Kishore, Patel Premal, De Coppi Paolo, Giuliani Stefano, Mullassery Dhanya, Curry Joe

机构信息

Department of Paediatric Surgery, Great Ormond Street Hospital, London, WC1N 3JH, UK.

Department of Interventional Radiology, Great Ormond Street Hospital, London, WC1N 3JH, UK.

出版信息

J Pediatr Surg. 2025 Mar;60(3):162011. doi: 10.1016/j.jpedsurg.2024.162011. Epub 2024 Oct 19.

DOI:10.1016/j.jpedsurg.2024.162011
PMID:39489681
Abstract

AIM OF THE STUDY

Abdominal lymphatic malformations (LM) are a challenging clinical entity. Complete excision can be impossible due to the infiltrative nature of some lesions and high rate of complications. Sclerotherapy may require multiple episodes of general anaesthesia and an inability to be definitive in terms of diagnosis and treatment. Subsequently we have adopted a newer algorithm of treatment involving a joint procedure with interventional radiology to enable an intra-operative treatment plan based on the findings. We present our early results.

METHOD

A retrospective observational study in a single tertiary paediatric centre from January 2019 to December 2023. The details of each patient were recorded along with their treatment pathway and outcome.

RESULTS

12 children underwent treatment for abdominal LM. Under GA and laparoscopic vision, pigtail catheters were inserted into the lesion to effect maximal drainage and assessment of surgical anatomy. If resection was feasible this was undertaken through a laparotomy and if not, the drains were retained and sclerotherapy was started. Three underwent drainage with sclerotherapy without resection and 9 underwent drainage with resection. Of the 3 who underwent sclerotherapy 2 have had further treatment and remain under review at a mean follow up of 7.6 months. Of the other 9 there were no signs of recurrence of the LM at a mean follow up of 29 (range 6-56) months.

CONCLUSION

We have found a combined procedure involving IR-guided drainage under laparoscopic vision enabling curative surgical resection to be undertaken, where feasible, with currently no significant morbidity. For those in whom this is not possible then direct intralesional sclerotherapy is the next most useful treatment modality for longer term control of symptoms.

LEVEL OF EVIDENCE

II.

摘要

研究目的

腹部淋巴管畸形(LM)是一种具有挑战性的临床病症。由于某些病变具有浸润性且并发症发生率高,可能无法完全切除。硬化治疗可能需要多次全身麻醉,并且在诊断和治疗方面无法做到明确。随后,我们采用了一种新的治疗方案,即与介入放射学联合进行手术,以便根据术中发现制定治疗计划。我们展示了我们的早期结果。

方法

在一家单一的三级儿科中心进行回顾性观察研究,时间跨度为2019年1月至2023年12月。记录每位患者的详细信息以及他们的治疗途径和结果。

结果

12名儿童接受了腹部LM的治疗。在全身麻醉和腹腔镜视野下,将猪尾导管插入病变部位以实现最大程度的引流并评估手术解剖结构。如果可行,则通过剖腹手术进行切除;如果不可行,则保留引流管并开始硬化治疗。3例未进行切除,仅接受了引流加硬化治疗;9例接受了引流加切除。在接受硬化治疗的3例中,2例接受了进一步治疗,平均随访7.6个月,仍在接受复查。在另外9例中,平均随访29(范围6 - 56)个月,未发现LM复发迹象。

结论

我们发现一种联合手术,即在腹腔镜视野下进行介入放射学引导的引流,在可行的情况下能够进行根治性手术切除,目前没有明显的发病率。对于那些无法进行手术切除的患者,直接病灶内硬化治疗是控制症状的下一个最有效的治疗方式。

证据等级

II级。

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