制度・支援
指定難病 — No.330

先天性気管狭窄症/先天性声門下狭窄症

検索語 Congenital Tracheal Stenosis ・ 最終更新 2026-09-17 14:32 ・ 最新に更新

Data Sheet
指定 No.330
Src PubMed · CT.gov · jRCT

これは医療アドバイスではありません。診断・治療の判断は必ず主治医にご相談ください。論文や治験は「今わかっている研究の状況」を示すもので、効果を保証するものではありません。

( 01 )EVIDENCE / PUBMED · 5件

世界の論文

直近の研究を、やさしい日本語で

各論文の見出しにある「確からしさ」は、その研究がどれくらい信頼できるかの目安です。「理論段階」はまだ仮説に近く、下にいくほど多くの患者で検証されていて、「メタ解析」がもっとも信頼できます。

観察研究
MK-01 · PMID 42663739

Three-dimensional tracheal morphologic changes after left pulmonary artery sling repair with and without tracheoplasty

Abstract / 原文

PURPOSE: In patients with a left pulmonary artery sling (LPAS) and congenital tracheal stenosis, indications for simultaneous slide tracheoplasty remain controversial. This study quantified three-dimensional tracheal morphologic changes after LPAS repair with and without slide tracheoplasty to provide insights into establishing surgical indications. METHODS: Airway geometries were reconstructed from pre- and postoperative computed tomography scans in 13 patients (10 with tracheoplasty, three with LPAS repair alone), measuring equivalent diameter and geometry-based flow resistance in a 30-mm segment proximal to the carina. All results are expressed as median [range]. RESULTS: The tracheoplasty group showed significant pericarinal enlargement (3.3 [2.4-4.1] to 5.2 [4.2-6.7] mm; P = 0.002) and a marked decrease in resistance estimates (2.30 [0.83-9.6] to 0.39 [0.11-1.88] cmH2O/(L/s); P = 0.004). In the LPAS repair alone group, two patients exhibited low preoperative resistance estimates (0.92 and 1.52 cmH2O/(L/s)), comparable to postoperative values in the tracheoplasty group. CONCLUSION: Slide tracheoplasty provides substantial morphologic and functional improvements, but certain patients achieve acceptable airway resistance with LPAS repair alone. Computational flow resistance may represent a useful tool for visualizing or quantifying the effects of surgical intervention.

Journal
Pediatric surgery international(2026 Aug)
Authors
6名
Type
Journal Article
PubMedで原文を見る
ランダム化比較試験(RCT)
MK-02 · PMID 42609557

Association of intraoperative cell salvage combined with standard allogeneic transfusion with hematological parameters and clinical outcomes in pediatric tracheoplasty: a non-randomized cohort study

Abstract / 原文

OBJECTIVE: To investigate associations of intraoperative autologous blood salvage (IOCS) combined with allogeneic transfusion vs. allogeneic transfusion alone in pediatric slide tracheoplasty (STP) for congenital tracheal stenosis (CTS), regarding hematological parameters, coagulation function, clinical outcomes, and safety. METHODS: From October 2023 to February 2025, 69 children meeting inclusion criteria were enrolled in this non-randomized study. Based on parental consent for IOCS, 35 were allocated to the observation group (IOCS + allogeneic transfusion) and 34 to the control group (allogeneic transfusion alone). Pre- and postoperative hematological and coagulation parameters, allogeneic transfusion volume, adverse reactions, postoperative fever, hospital stay, and 6-month mortality were compared. RESULTS: Preoperative indicators showed no significant intergroup differences (P > 0.05). Postoperatively, the observation group had higher red blood cell count, hematocrit, and hemoglobin (all P < 0.05); shorter prothrombin time and higher fibrinogen (both P < 0.05); and no significant differences in APTT or TT (both P > 0.05). Intraoperative allogeneic red blood cell and fresh frozen plasma volumes were significantly lower in the observation group (both P < 0.05). No hemolytic or allergic transfusion reactions occurred. Postoperative fever was higher in the control group (P < 0.05), and hospital stay was shorter in the observation group (P < 0.05). No deaths occurred within 6 months. CONCLUSION: IOCS combined with allogeneic transfusion was associated with better postoperative hematological and coagulation profiles, reduced allogeneic blood requirements, and shorter hospital stay. No transfusion-related adverse reactions or deaths were observed. These findings warrant confirmation in prospective randomized studies.

Journal
Frontiers in pediatrics(2026)
Authors
7名
Type
Journal Article
PubMedで原文を見る
観察研究
MK-03 · PMID 42591013

High-Flow Nasal Cannula Versus Conventional Therapy After Slide Tracheoplasty

Abstract / 原文

BACKGROUND: Few studies have focused on the respiratory management after slide tracheoplasty. This study aimed to evaluate the effect of high-flow nasal cannula (HFNC) compared with conventional therapy in preventing reintubation after slide tracheoplasty. METHODS: This was a retrospective study conducted at a single center, the Department of Pediatric Surgery. The medical records of 68 patients who had undergone slide tracheoplasty for congenital tracheal stenosis (CTS) before the age of 2 years from February 2012 to September 2021 were included. Patients were divided into group C (conventional therapy) or H (HFNC therapy), based on whether conventional oxygen therapy and positive pressure ventilation or HFNC was used after withdrawal from the ventilator, respectively. RESULTS: There were no significant differences in the postoperative reintubation rate and ventilator dependency between groups C and H. There were no HFNC complications. CONCLUSIONS: The use of postoperative HFNC is not inferior to noninvasive ventilation in terms of therapeutic efficacy. HFNC can be used without complications for a limited time after extubation.

Journal
Pediatrics international : official journal of the Japan Pediatric Society(2026)
Authors
6名
Type
Journal Article, Comparative Study
PubMedで原文を見る
観察研究
MK-04 · PMID 42480651

Factors associated with tracheoplasty in children with pulmonary artery sling and congenital tracheal stenosis

Abstract / 原文

OBJECTIVE: The study objective was to investigate the anatomic and clinical factors associated with tracheoplasty in children with pulmonary artery sling and congenital tracheal stenosis. METHODS: This retrospective multicenter study included children with pulmonary artery sling who underwent surgical treatment at 3 cardiac centers in China between January 2010 and September 2025. Preoperative clinical characteristics, computed tomography measurements, and operative data were reviewed. Univariable and multivariable logistic regression analyses were performed to identify factors independently associated with tracheoplasty. Interaction analyses were also conducted. RESULTS: After excluding 24 patients with incomplete preoperative imaging data, 330 patients with pulmonary artery sling and congenital tracheal stenosis were included. Among them, 302 (91.5%) underwent slide tracheoplasty and 28 (8.5%) underwent pulmonary artery sling correction alone. Younger age, long-segment tracheal stenosis, and more severe airway narrowing were independently associated with a higher frequency of tracheoplasty. CONCLUSIONS: Younger age, long-segment tracheal stenosis, and more severe airway narrowing were independently associated with tracheoplasty in this multicenter cohort. Tracheoplasty performance appeared to be influenced by multiple factors rather than any single imaging parameter. Further prospective studies with long-term follow-up are required to evaluate clinical outcomes associated with different management strategies.

Journal
The Journal of thoracic and cardiovascular surgery(2026 Jul)
Authors
9名
Type
Journal Article
PubMedで原文を見る
症例報告
MK-05 · PMID 42267030

Right Atrial Thrombus After Cardiac Surgery With Tissue Expander in a Child With Right Pulmonary Agenesis

Abstract / 原文

We report the case of a child with right pulmonary agenesis, congenital tracheal stenosis, and atrial septal defect (ASD). At age 2 years, an intrathoracic tissue expander was placed to reduce tracheal compression from mediastinal shift and to enable subsequent atrial septal defect closure. At age 5 years, a right atrial mass was incidentally detected along with right atrial compression caused by the expander. Emergency mass resection was performed, and the mass was diagnosed as a thrombus. The tissue expander was subsequently deflated and removed to prevent further compression. The patient had no recurrence of thrombus or respiratory symptoms during 8-year follow-up.

Journal
Annals of thoracic surgery short reports(2026 Jun)
Authors
3名
Type
Case Reports, Journal Article
PubMedで原文を見る
( 02 )TRIALS / JAPAN · 0件

日本で参加できる治験

現在 募集中のもの

日本で現在募集中の治験は見つかりませんでした。下の公式レジストリで条件を変えると見つかる場合があります。
( 03 )REGISTRY / jRCT

治験をもっと探す

日本の公式レジストリで全件を確認

上の一覧は ClinicalTrials.gov の一部です。日本国内の治験の多くは、日本の公式レジストリ jRCT にのみ登録されています。下記から最新の全件を確認できます。

jRCT で検索日本の臨床研究実施計画 公開システム「対象疾患名」に 先天性気管狭窄症/先天性声門下狭窄症 を入力し、「募集状況」で 募集中 にチェックして検索します。ClinicalTrials.gov で全件を見る世界最大の治験データベース(英語)「先天性気管狭窄症/先天性声門下狭窄症・日本・募集中」の条件で一覧が開きます。

※ jRCTは自動の大量データ取得を禁じているため、本サービスは自動収集せず、ご自身が公式サイトで検索できるリンクでご案内しています(規約順守)。

お金・介護・制度先天性気管狭窄症/先天性声門下狭窄症の療養に使えるかもしれない公的サポートを調べる医療費・生活費・介護の支援制度と相談先を、あなたの状況に合わせてご案内(回答は端末内で完結)
( 04 )SUPPORT

患者会・相談窓口

一人で抱え込まないでください

同じ病気の患者・家族とつながる、制度や生活の相談をする、といったときの窓口です。

全国の相談先

※ お住まいの都道府県の「難病相談支援センター」でも、医療費助成や療養生活の相談ができます(難病情報センターから探せます)。