Kobe Childrens Hospital

Kōbe-shi, Japan

Kobe Childrens Hospital

Kōbe-shi, Japan
SEARCH FILTERS
Time filter
Source Type

Yokoi A.,Kobe Childrens Hospital | Oshima Y.,Kobe Childrens Hospital | Nishijima E.,Takatsuki General Hospital
Journal of Pediatric Surgery | Year: 2017

Purpose: Congenital tracheal stenosis (CTS) and a single right lung treated with slide tracheoplasty (ST) has relatively high rates of mortality and morbidity. We report a single institution's experience with adjunctive procedures at the time of ST to reduce postoperative tracheal obstruction in patients with a single right lung and CTS. Methods: With IRB approval, 8 patients with a single right lung and CTS who underwent ST in our institution between 2008 and 2016 were reviewed. Results: Seven of 8 patients (88%) survived, with a median follow-up period of 35 (3-89) months. The only mortality was because of complications unrelated to the airway. Six of 8 patients underwent ST anterior to the aortic arch (tracheal translocation, TT). This was combined with tracheopexy concomitantly in 3 patients and subsequently in one patient. Two patients had insufficient tracheal length for TT. Both underwent aortopexy. Of three patients undergoing TT with tracheopexy concomitantly, two have been successfully extubated, and a third has required tracheostomy for subglottic stenosis. All patients undergoing TT without tracheopexy have experienced severe tracheobronchomalacia. Conclusion: We conclude that adjunctive TT with tracheopexy might be associated with lower rates of tracheobronchial obstruction in patients with a single right lung undergoing ST for CTS. Level of evidence: Level IV. © 2017.


Nino N.,Kobe Childrens Hospital
[Rinsho ketsueki] The Japanese journal of clinical hematology | Year: 2016

We herein describe a 2-year-old boy with severe congenital neutropenia (SCN) who was successfully treated with reduced-intensity bone marrow transplantation (HSCT). He had suffered recurrent episodes of bacterial pneumonia from 12 months of age, and was found to have severe neutropenia with white blood cell counts below 100/μl. The patient harbored a heterozygous missense mutation in ELANE exon 4 (p.Gln134Pro, NM_001972.2: c.401A>C). This was a novel mutation. Due to intractable pneumonia and severe persistent neutropenia, reduced-intensity HSCT was performed from an HLA-matched sibling donor. The preparative regimen consisted of melphalan, fludarabine, and 4 Gy of total body irradiation. Hematopoietic engraftment was rapidly obtained, i.e., by day +14, and complete donor chimerism was subsequently achieved. The lung complications observed pre-transplantation markedly improved after neutrophil recovery, i.e., by day +60. We concluded that HSCT is a useful treatment for SCN patients, especially for those at high risk of leukemic transformation. Fludarabine-based reduced-intensity HSCT may represent a safe and effective therapeutic option for patients with SCN who need HSCT even if they have intractable infectious complications.


Miki Y.,Kobe Childrens Hospital | Tanaka T.,Kobe Childrens Hospital | Oshima Y.,Kobe Childrens Hospital
Cardiology in the Young | Year: 2017

Absent pulmonary valve with an intact ventricular septum is a rare malformation. We report a case of absent pulmonary valve and intact ventricular septum with functional near-tricuspid atresia caused by pulmonary regurgitation. Initial palliation with main pulmonary artery ligation and bilateral pulmonary artery banding was performed at 1 day of age. More antegrade flow across the tricuspid valve was recognised postoperatively, resulting in a successful right ventricular outflow tract reconstruction by a hand-sewn bileaflet polytetrafluoroethylene valve and modified Blalock-Taussig shunt at 11 days of age. © Cambridge University Press 2016.


Maeda K.,Kobe Childrens Hospital
Pediatric Surgery International | Year: 2017

Pediatric airway surgery is a challenging field in pediatric surgery. Laryngotracheal stenosis has a variety of congenital and acquired conditions that require precise assessment and tailored treatment for each individual patient. About 90% of acquired conditions are represented by subglottic stenosis (SGS) resulting as a complication of tracheal intubation. Congenital tracheal stenosis (CTS) is a rare and life-threatening malformation, usually associated with complete tracheal rings along a variable length of the trachea. Tracheomalacia (TM) is a process characterized by flaccidity of the supporting tracheal cartilage, widening of the posterior membranous wall, and reduced anterior-posterior airway caliber. The clinical presentation can vary from almost asymptomatic patients to near fatal airway obstruction. There is considerable variation in both the morphologic subtypes and the prognosis of pediatric airway. The patients are divided into three clinical groups (mild, moderate, and severe). A further division was proposed according to the presence or absence of associated anomalies. The definitive diagnosis of pediatric airway was made by means of rigid bronchoscope and computed tomography scan with three-dimensional reconstruction (3D-CT). Rigid bronchoscopy and 3D-CT confirmed the diagnosis in all the cases. Other associated anomalies include congenital heart disease, vascular anomalies, and BPFM (maldevelopment of aerodigestive tract). After definitive diagnosis of pediatric airway lesions, surgical intervention should be considered. Surgical strategy was presented on each lesion. © 2017 Springer-Verlag Berlin Heidelberg


Hasegawa T.,Kobe Childrens Hospital | Oshima Y.,Kobe Childrens Hospital | Kadowaki T.,Kobe Childrens Hospital
Cardiology in the Young | Year: 2014

Adequate arch augmentation for interrupted aortic arch repair is quite important to avoid post-operative recoarctation and bronchial compression. We describe here two successful cases of aortic arch reconstruction using autologous materials such as a pulmonary artery patch and a reversed left subclavian artery flap in infants with an interrupted aortic arch type B complex. © Cambridge University Press 2013.


Hasegawa T.,Kobe Childrens Hospital | Oshima Y.,Kobe Childrens Hospital | Tanaka T.,Kobe Childrens Hospital | Maruo A.,Kobe Childrens Hospital | Matsuhisa H.,Kobe Childrens Hospital
Journal of Thoracic and Cardiovascular Surgery | Year: 2016

Objective To investigate whether echocardiographic characteristics in the descending aorta of patients with cyanotic congenital heart disease who have received a systemic-to-pulmonary artery (SP) shunt can indicate shunt flow volume and predict postoperative adverse events related to high-flow shunting. Methods Among the 73 consecutive patients who received an SP shunt between 2010 and 2014, data for 53 patients who underwent postoperative Doppler echocardiographic assessment of diastolic retrograde flow in the descending aorta (dAo-RF) were reviewed retrospectively. Results The mean dAo-RF ratio was 0.50 ± 0.15 at intensive care unit admission and reached its peak level (0.56 ± 0.12) at 24 hours after surgery. All of the patients with a maximal dAo-RF ratio of ≥0.80 had experienced acute heart failure or cardiogenic shock due to postoperative high-flow shunting and required emergent surgical interventions to reduce pulmonary blood flow. Pulse oximetry-measured oxygen saturation and serum lactate level were significantly correlated with dAo-RF ratio, but they had some clinical dispersion to match the postoperative adverse events. Conclusions The dAo-RF ratio is a simple, repeatable, and noninvasive index for postoperative assessment of SP shunt flow volume. A high dAo-RF ratio is a significant predictor of postoperative adverse events of high-flow shunting. Copyright © 2016 by The American Association for Thoracic Surgery.


Onoda M.,Kobe Childrens Hospital | Fujikawa M.,Osaka General Medical Center | Koh K.,Osaka General Medical Center
Annals of Plastic Surgery | Year: 2010

Among many methods to reconstruct a full-thickness eyelid defect, we have applied a new design of orbicularis oculi musculocutaneous flap for the anterior wall reconstruction in 7 cases. In all cases, flap survived without any complications. In the design proposed in the article, the flap was raised from the lateral orbital region and the pivot point was placed around the lateral canthus. The method was considered to be safe, easy, and reliable, and at the same time, effective in minimizing the donor site morbidity. Copyright © 2010 by Lippincott Williams & Wilkins.


Kobayashi D.,Kobe Childrens Hospital | Satsuma S.,Kobe Childrens Hospital | Kuroda R.,Kobe University | Kurosaka M.,Kobe University
Journal of Bone and Joint Surgery - Series A | Year: 2010

Background: Adult patients may present with acetabular dysplasia without a history of developmental dysplasia of the hip. The purpose of the present study was to clarify the development of primary acetabular dysplasia in patients under the age of eighteen years by evaluating the contralateral hip in those with unilateral developmental dysplasia of the hip. Methods: Radiographs of the contralateral hip of eighty-eight patients with unilateral developmental dysplasia of the hip were reviewed retrospectively. The center-edge angle was measured at the age of eighteen years. The primary acetabular dysplasia group included hips with a center-edge angle of <20°, and the normal group included hips with an angle of ≥20°. The acetabular index at the age of three years, the center-edge angle between the ages of three and eighteen years, and the acetabular angle of Sharp between the ages of six and eighteen years were measured. Results: According to our classification system, twelve hips (13.6%) were assigned to the primary acetabular dysplasia group. At the age of three years, there were no significant differences between the two groups radiographically. A significant difference in the center-edge angle between the two groups was seen at each evaluation period after the age of six years. However, twenty-two patients in the normal group had poor acetabular coverage and three patients in the primary acetabular dysplasia group had good acetabular coverage at the age of nine years. After the age of nine years, improvements in the center-edge angle and the acetabular angle of Sharp were noted in the normal group, whereas no acetabular growth was seen in the primary acetabular dysplasia group. There was no patient with a center-edge angle of <15° at the age of twelve years in the normal group. Conclusions: After the age of six years, a difference in acetabular growth develops between patients with primary acetabular dysplasia and those with normal hips. However a final prognosis for acetabular development appears to be difficult to determine until the age of twelve years. Level of Evidence: Prognostic Level II. See Instructions to Authors for a complete description of levels of evidence. Copyright © 2010 by The Journal of Bone and Joint Surgery, Incorporated.


Hasegawa T.,Kobe Childrens Hospital | Oshima Y.,Kobe Childrens Hospital | Maruo A.,Kobe Childrens Hospital | Matsuhisa H.,Kobe Childrens Hospital | And 3 more authors.
Journal of Thoracic and Cardiovascular Surgery | Year: 2015

Objective Aortic arch reconstruction in the Norwood procedure is occasionally associated with postoperative airway and branch pulmonary artery stenosis, as well as recoarctation. This study investigated geometric changes in the aortic arch after the Norwood procedure and evaluated the efficacy of arch angle augmentation with glutaraldehyde-treated autologous pericardium in aortic arch reconstruction. Methods Of 52 consecutive patients who underwent the Norwood procedure between 1998 and 2013, a total of 36 patients who underwent postoperative multidetector-row computed tomographic angiography were reviewed retrospectively, and divided into 2 groups according to whether arch angle augmentation with glutaraldehyde-treated autologous pericardium was performed (AAA group, 26 patients) or not (NA group, 10 patients). Results The neoaortic arch in the Norwood procedure was characterized by having a significantly smaller angle, being lower in height, and being narrower than that in age-matched control patients with a normal aortic arch. Moreover, all of these arch geometric measurements were significantly larger in the AAA group than in the NA group. Freedom from reintervention and reoperation for bronchomalacia or branch pulmonary artery stenosis, caused by extrinsic compression of the neoaorta, was significantly better at 1 year in the AAA group than in the NA group. Recoarctation of the neoaorta was documented in only 1 (3.8%) patient in the AAA group versus 3 patients (30%) in the NA group. Conclusions Arch angle augmentation with glutaraldehyde-treated autologous pericardium in the Norwood procedure creates more aortopulmonary space with a smoother arch angle and diminished incidence of postoperative recoarctation, bronchial compression, or branch pulmonary artery compression. © 2015 The American Association for Thoracic Surgery.


Hisamatsu E.,Kobe Childrens Hospital | Nakagawa Y.,Kobe Childrens Hospital | Sugita Y.,Kobe Childrens Hospital
Urology | Year: 2014

Objective To present our experience of vaginal reconstruction in female cloacal exstrophy patients. Patients and Methods The records of 7 postpubertal female cloacal exstrophy patients (median age, 17 years; range, 11-26 years) were retrospectively reviewed. Complete duplication of the uterus and vagina was noted in all patients. All of them underwent various types of vaginoplasty at the time of urinary tract reconstruction. When a pull-through vaginoplasty was difficult, the native vagina was anastomosed to the neovagina using the large or small bowel. Alternatively, the native bladder remaining connected to the vagina was used as a neovagina with simultaneous construction of the gastroileal composite reservoir. Results The median age at vaginoplasty was 6 years (range, 5-8 years). The techniques were pull-through vaginoplasty in 1 patient, intestinal vaginoplasty in 2 patients, and vaginoplasty using the native bladder in 4 patients. Five patients required revision surgery for hematometrocolpos after the onset of puberty (median age, 14 years; range, 11-16 years). Three patients underwent an end-to-side reanastomosis of the uterus to the neovagina using the bowel or the native bladder, whereas 2 patients received a side-to-side anastomosis of the uterus to the neovagina using the native bladder. No patient developed recurrence of hematometrocolpos after the revision surgery, with a mean follow-up of 71 months. Conclusion Our recent approach to vaginoplasty can be an alternative for female cloacal exstrophy patients. A side-to-side anastomosis of the uterus to the neovagina is a technically easy procedure in surgical management of hematometrocolpos. © 2014 Elsevier Inc. All Rights Reserved.

Loading Kobe Childrens Hospital collaborators
Loading Kobe Childrens Hospital collaborators