Unicentric Series of 82 Consecutive Patients With Ultralong Hirschsprung Disease: How Experience Leads Management

IF 2.4 2区医学 Q1 PEDIATRICS

Journal of pediatric surgery Pub Date : 2025-03-20 DOI:10.1016/j.jpedsurg.2025.162283

A. Pini Prato , G. Mottadelli , A. Bertolino , L. Giacometti , S. Casella , M. Novi , M. Erculiani

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引用次数: 0

Abstract

Introduction

Total Colonic (TCSA), Extended (EA), and Total Intestinal Aganglionosis (TIA) are rare forms of Hirschsprung (HSCR) grouped under the term Ultralong HSCR. We aim at presenting the features of the largest unicentric series reported so far and at sharing the algorithm for management adopted in our Center for Digestive Diseases.

Materials and methods

We enrolled all consecutive patients with Ultralong HSCR admitted between 2017 and 2024. Data regarding clinical features, diagnosis, management, and outcome have been recorded along with details regarding definition, classification and management.

Results

A total of 82 patients were included, 69 being TCSA, 7 EA and 6 TIA. Length of involved ileum in TCSA ranged between 5 and 75 cm. Average age at pull-through (PT) was 2 years. Surgical complications were experienced by 36 % of patients regardless of type of PT. Postoperative enterocolitis (HAEC) were more frequent after Duhamel procedures. Stoma was fashioned 40 cm from the Ligament of Treitz (LOT) in TIA or as a levelling jejunostomy in EA. Three EA patients underwent Skipped Aganglionic Lengthening Transposition (SALT) to improve enteral autonomy with good results. Other lengthening procedures or autologous reconstructions proved to be less effective. Five patients with EA/TIA received total enterectomy nullifying the possibility for subsequent lengthening procedures. Mortality rate was 2.4 %.

Conclusions

Based on these results and on literature data, we propose an outcome-driven classification based on the length of ganglionated bowel below the LOT. We also suggest a management protocol aimed at improving survival for these delicate subgroups of HSCR patients.

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连续 82 例超长型赫氏肺病患者的单中心系列研究：经验如何指导治疗。

导言：全结肠（TCSA）、扩展（EA）和全肠芒刺病（TIA）是罕见的赫氏脓肿（HSCR），被归类为超长型 HSCR。我们旨在介绍迄今为止报道的最大单中心系列的特征，并分享我们消化疾病中心采用的治疗算法：我们登记了2017年至2024年期间收治的所有连续超长型HSCR患者。我们记录了有关临床特征、诊断、管理和结果的数据，以及有关定义、分类和管理的详细信息：共纳入82例患者，其中69例为TCSA，7例为EA，6例为TIA。TCSA患者受累回肠的长度在5至75厘米之间。拉通（PT）时的平均年龄为 2 岁。36%的患者出现了手术并发症，无论PT类型如何。术后小肠结肠炎（HAEC）在 Duhamel 手术后更为常见。TIA患者的造口在距特雷兹韧带（LOT）40厘米处，EA患者的造口为平整空肠造口。三位 EA 患者接受了跳过 Aganglionic 拉长转位术 (SALT)，以改善肠道自主性，效果良好。其他延长术或自体重建术的效果较差。五名 EA/TIA 患者接受了全肠切除术，从而失去了接受后续延长手术的可能性。死亡率为2.4%：根据上述结果和文献数据，我们提出了一种基于 LOT 下方节段性肠管长度的结果驱动型分类法。我们还提出了一种管理方案，旨在提高这些微妙的 HSCR 患者亚群的生存率。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of pediatric surgery 医学-外科

CiteScore

1.10

自引率

12.50%

发文量

569

审稿时长

38 days

期刊介绍： The journal presents original contributions as well as a complete international abstracts section and other special departments to provide the most current source of information and references in pediatric surgery. The journal is based on the need to improve the surgical care of infants and children, not only through advances in physiology, pathology and surgical techniques, but also by attention to the unique emotional and physical needs of the young patient.