馬場 勝尚

Purpose: Congenital tracheal stenosis (CTS) is rare and challenging. Complete tracheal rings cause a wide spectrum of airway-obstructing lesions and varying degrees of respiratory distress. Although surgical reconstruction is the primary option for symptomatic CTS, sometimes an appropriate management strategy may be difficult due to other anomalies. We aimed to identify pitfalls in the management of CTS. Methods: We retrospectively reviewed the records of patients with CTS during the last 10 years in our institution. Results: Sixteen pediatric patients were diagnosed with CTS. Of the 16 patients, 12 (75.0%) had cardiovascular anomalies including seven left pulmonary artery sling. Six patients with dyspnoea caused by CTS and three patients with difficult intubations due to CTS underwent tracheoplasty. Four patients underwent only cardiovascular surgery without tracheoplasty. Three asymptomatic patients were followed up without undergoing any surgical procedure. We repeatedly discussed management of four patients with especially complex pathophysiology at multidisciplinary meetings. Right ventricular outflow tract obstruction, tracheobronchial malacia, increased pulmonary blood flow, and pulmonary aspiration due to gastroesophageal reflux presumably accounted for their severe respiratory distress, and we forewent their tracheal reconstruction. Conclusion: The management of CTS should be individualized, and conservative management is a feasible option in selected cases.

Covered cloacal exstrophy (CCE) is extremely rare condition. In patients with a single perineal orifice and no pubic bone separation, it is very difficult to suspect and/or diagnose CCE based on external signs alone. We present the case of a 2-month-old girl diagnosed with CCE based on cystography, ileostomy contrast study and cystoscopy.

【目的】当科では小児急性虫垂炎に対し，保存的治療を第一選択としている．今回，我々の治療経験から小児急性虫垂炎に対する保存的治療の適応および限界について検討した．<br>【方法】2012 年1 月から2014 年8 月までに急性虫垂炎の診断で入院し抗菌薬を用いた保存的治療を行った53 症例を対象とし，保存的治療奏効群と保存的治療抵抗群に分けて比較検討した．<br>【結果】53 例中，奏効群は36 例，抵抗群は17 例．入院時体温は奏効群が37.4±0.7°C，抵抗群が38.2±0.8°C で抵抗群において高かった（p＝0.01）．入院時血液検査所見では，CRP が奏効群1.4±1.7 mg/dl，抵抗群9.7±7.0 mg/dl と，抵抗群で高値であった（p＜0.01）．画像所見では，虫垂最大径が奏効群8.4±2.7 mm，抵抗群11.3±2.5 mm と抵抗群で有意に腫大していた（p＜0.01）．糞石は奏効群の16.7％（6/36 例），抵抗群の76.5％（13/17 例）に認めていた（p＜0.01）．治療開始後24 から48 時間での白血球数は奏効群6,988.9±2,884.8/μl，抵抗群11,741.2±3,845/μl と，抵抗群で高値であった（p＜0.01）．再発率は奏効群8.3％に対し，抵抗群では36.4％と高率であった（p＝0.042）．治療開始から48 時間での2 群のカットオフ値は，白血球数9,650.0/μl，CRP 値が6.67 mg/dl と推定された．<br>【結論】治療後48 時間において白血球数が9,650.0/μl またはCRP 値が6.67 mg/dl を超える症例では早期の外科治療を検討すべきである．

Purpose: This study aimed to evaluate the use of a transumbilical incision for infants and children, as well as neonates, with various intraabdominal conditions. Methods: A retrospective study of transumbilical incision surgery was performed between June 2007 and June 2013. Patients were divided into two groups: group 1 of neonates and group 2 of infants and children. All operations were performed via an upper circumumbilical incision. Results: Thirty-six patients (22 males, 14 females) were treated via a transumbilical incision, with 20 patients in group 1 and 16 patients in group 2. A transverse incision extension was needed for 1 case in group 1 (intestinal atresia complicated by meconium peritonitis) and 4 cases in group 2 (two with ileus owing to adhesive bands, 1 with malrotation, 1 with ectopic pancreatic tissue in the duodenum). In cases with a dilated intestinal wall or intraabdominal adhesions, an optional extension of the transverse incision might be required. Only 1 case with ileus in group 2 developed a wound infection that was treated by drainage. The postoperative cosmetic results were acceptable in all cases. Conclusion: The transumbilical incision yielded a sufficiently large surgical field, and the surgical condition was easily and directly viewed. In all 36 cases, an adequate operation was safely performed. This approach is a safe and effective method for various intraabdominal disorders in not only neonates but also infants and children, and leads to an imperceptible incision. (C) 2014 Elsevier Inc. All rights reserved.

Nonoperative management is acceptable treatment for minor pancreatic injuries. However, management of major pancreatic duct injury in children remains controversial. We present our experience in treating isolated pancreatic duct injury. We describe the cases of three male patients treated for complete pancreatic duct disruption in the past 5 years at our institution. We performed pancreatic duct repair to avoid distal pancreatectomy and to maintain normal pancreatic function. All patients underwent enhanced computed tomography and endoscopic retrograde cholangiopancreatography in the early period. The injuries were classified as grade III according to the American Association for the Surgery of Trauma classification. In two cases, we performed end-to-end anastomosis of the pancreatic duct during the delayed period. In the third case, we placed a stent across the disruption to the distal pancreatic duct. The patients' postoperative courses were uneventful, and the average hospitalization was 25.6 days after the procedure. At a median follow-up of 36 months (range 14-54 months), all patients remain asymptomatic, with normal pancreatic function, but with persistent distal pancreatic duct dilatation. We suggest that distal pancreatectomy should not be routinely performed in patients with isolated pancreatic duct injury.

The optimal management of microcystic lymphatic malformations (LMs) in children has not been established. We describe how we used the Ligasure (TM) Vessel Sealing System (LVSS) to achieve partial resection of refractory microcystic LMs in a 1-year-old boy. The child was admitted in respiratory distress caused by infection and swelling of cervical LMs. The LMs had been diagnosed prenatally, but had not decreased in size despite three treatments with OK-432 sclerotherapy. We performed direct dissection of the microcystic LMs using the LVSS with minimal intraoperative blood loss or lymphatic leakage. The LMs were resected as completely as possible without damage to the jugular vein or major nerves. His postoperative course was uneventful. Histological examination revealed complete sealing of the lymphovascular channels with obliterated lumens. Resection using the LVSS is effective and easy to perform for partial resection of microcystic LMs. We recommend the combination of initial OK-432 injection therapy and subsequent partial resection using the LVSS for refractory microcystic LMs.

Neonates with congenital tracheal stenosis (CTS) sometimes develop respiratory distress and may be difficult to intubate. We used balloon tracheoplasty with a rigid bronchoscope for emergency airway management in neonates with symptomatic CTS. Herein, we describe the balloon tracheoplasty procedure and the early outcomes following its use as the initial treatment of neonatal symptomatic CTS. We performed a retrospective analysis of five neonates with CTS who were initially treated with balloon tracheoplasty at our institution from January 2010 to December 2013. Five patients with a mean birthweight of 2,117 g were treated during the study period. Of these, four developed respiratory distress after birth, and all patients had difficult intubations. In all five patients, definitive diagnosis of CTS was made by rigid bronchoscopy and 3-dimensional reconstruction scan. A total of nine balloon dilatations were performed in five patients. Following balloon tracheoplasty, two patients were extubated, one was extubated after resection and end-to-end anastomosis following initial balloon dilatation, and one remained hospitalized with tracheostomy for tracheomalacia. The remaining patient died from tracheal bleeding associated with congenital heart disease. Although our sample size was small, balloon tracheoplasty is a potentially effective initial treatment for selected cases with neonatal symptomatic CTS. © 2014 Springer-Verlag.

Blunt trauma to the abdomen accounts for the majority of abdominal injuries in children. Pancreatic injury is the fourth most common solid organ injury, following injuries to the spleen, liver and kidneys. The most common complications are the formation of pancreatic fistulae, pancreatitis and the development of pancreatic pseudocysts, which usually present several weeks after injury. The nonoperative management of minor pancreatic injury is well accepted however, the treatment of more serious pancreatic injuries with capsular, ductal or parenchymal disruption in pediatric patients remains controversial. Based on the data presented in this literature review, although children with pancreatic injuries (without ductal disruption) do not appear to suffer increased morbidity following conservative management, patients with ductal disruption may benefit from operative intervention. © 2013 Springer-Verlag Berlin Heidelberg.

Although many advances have improved the treatment of congenital and acquired laryngotracheal stenosis in children over the past two decades, the therapeutic decision-making process remains challenging for pediatric surgeons and otolaryngologists. Severe subglottic stenosis is a complex laryngeal injury that necessitates multiple airway procedures, and the approach depends on the exact nature of the cicatricial lesion and its effect on the vocal cord mobility. Therefore, it is imperative that the pediatric surgeons and otolaryngologists dealing with this situation should be well trained in endoscopy and laser treatment, in addition to open surgical intervention. Open re-do surgery remains the best choice in cases of severe congenital stenosis, glottic immobility, or after two to three endoscopic procedures have been performed without any significant improvement.

AIM: Congenital tracheal stenosis is an obstructive airway lesion that often presents as a life-threatening emergency. We had introduced the balloon dilatation and placement of the expandable metallic airway stent as a therapeutic option, and this study aimed to clarify the long-term outcomes in pediatric patients. METHODS: A retrospective review of five infants in whom balloon expandable metallic airway stents (10-40 mm long and 6-8mm in diameter) were inserted in 1997 to 2000 was conducted. RESULTS: There was an immediate improvement of respiratory obstruction in all five infants (aged 7 days to 12 months) with four weaned from ventilation. One child died after 9 months of palliative treatment. In all inflammation and granulation tissue developed over the stents, but this could be managed by scraping or balloon compression. Metallic stents have been in place a mean of 12 years (range 6 months to 13 years) after insertion without other complications. Four children are alive and well with their stents in place. Recently, an attempt to remove the stent was done in two patients who showed dyspnea on exertion. They underwent tracheoplasty following successful complete removal of metallic stent using cardiopulmonary bypass. CONCLUSION: Use of expandable metallic airway stents following balloon dilatation can be left for long periods to relieve tracheal obstruction. Development of granulation tissue is a major treatable complication. Removal of the stent was safely completed by open surgical intervention using a cardiopulmonary bypass. The airway stent may provide an important therapeutic option in selected cases with congenital tracheal stenosis.

Intrapericardial extralobar pulmonary sequestration is a very rare congenital lung anomaly. We report a case of this condition, detected as an intrathoracic cystic lesion by using prenatal ultrasonography. The neonate was born at 38 weeks of gestation with no progression of the lesion and no respiratory or cardiac symptoms. Ultrasonography and computed tomography (CT) revealed a 40 × 17 × 17-mm intrapericardial lesion, composed of cystic components and a solid component. Intrapericardial extrapulmonary sequestration was suspected largely because CT showed a vague aberrant artery. At the age of 3 months, elective surgery was performed, and the postoperative course was uneventful.

11歳男児。前胸部の海綿状リンパ管腫摘出術後1ヵ月目に創部離開とリンパ漏が出現した。そのため創部を再縫合し、皮下に閉鎖式ドレーンを再留置したが、皮膚は再離開を繰り返し、閉鎖式ドレーンの留置を断念した。以後、手術から2ヵ月経過でこの創部離開とリンパ漏に対し皮膚欠損用創傷被覆材(バーシバXC)を用いた治療を開始した。特にに滲出液の多い時期には創面と皮下ポケットへアルギン酸塩被覆材(カルトスタット)を使用し、交換時に局所感染を疑う汚染が強い場合には創面にアクアセルAgを用いた。その結果、治療開始1ヵ月で治癒が得られた。

症例1:3ヵ月女児。胎児MRIで右肺上部肺側に多房性嚢胞を認め、congenital cystic adenomatoid malformation of the lung(CCAM)I型が疑われた。出生後嚢胞の縮小傾向はなく、生後3ヵ月時に右上葉切除を行った。症例2:1歳2ヵ月女児。出生後の胸部CTで左上葉に多発する小嚢胞を認め、無症状のため体重が10kgを超えた時点で区域切除を施行し、病理所見はCCAM II型であった。症例3:3歳男児。生後2ヵ月のCTで右肺の下葉に多発小嚢胞を認め、無症状で経過したが病変に変化がなく、3歳時に区域切除を行い、病理所見はCCAM III型であった。症例4:6歳男児。生後3ヵ月時のCTで右肺葉外肺分画症と診断し、経過観察で症状はなく胸部陰影の変化もなかったが、希望により6歳時に摘出術を施行した。症例5:13歳女児。肺炎の治療中にCTで右下葉に多発した嚢胞を認めてCCAMと診断し、夏休みを利用して嚢胞部分の区域切除を行った。5例とも術後経過順調である。

3ヵ月男児。在胎23週時の胎児超音波で胸腔内腫瘤を指摘され、MRIでは心臓左側に2.9×1.9cmの嚢胞性病変を確認した。出生後の心エコーで心嚢内の腫瘤は嚢胞性病変と充実性病変から形成され、胸部CT(MDCT)で脂肪や石灰化は認めず、上行大動脈から左房室間溝を経て腫瘤へ流入する血管が描出され、心嚢内肺分画症を最も疑った。生後の呼吸循環動態は安定しており、哺乳、体重増加も良好で、腫瘤の増大傾向は認めなかった。生後3ヵ月時に開胸術を施行し、左房と左室の間に3cm大の嚢胞性腫瘤を認め、流入動脈および流出静脈を結紮処理後、摘出した。病理組織所見で、充実性病変部位では細気管支から肺胞、軟骨と気管支腺などの肺構造が確認された。嚢胞性病変部位は線毛上皮に覆われた腔が多数を占めており、気管支内腔に分泌物が貯留し嚢胞状に拡張したと考えられ、心嚢内肺葉外肺分画症と診断した。術後経過良好で、8日目に退院した。

新生児臍帯ヘルニアに対して,合目的的でより低侵襲な治療法を模索してきた.今回,新しい治療法を採用し,良好な成績が得られたので報告する.