Yuko Bitoh

Esophageal atresia: Prognostic classification revisited

BACKGROUND Although the Spitz classification is the most widely used prognostic classification for esophageal atresia and/or tracheoesophageal fistula (EA), its discrimination ability remains unclear. We sought to develop a more accurate prognostic classification for EA. METHODS The records of 121 consecutive infants with EA (1980-2005) were reviewed. The independent variables included 6 clinical characteristics, and the dependent variables were survival and mortality. Stepwise logistic regression analysis was used to construct models predicting mortality and create a revised prognostic classification. The discrimination abilities of the revised classification and the Spitz classification were compared using receiver-operating characteristic (ROC) curves. RESULTS Birth weight and the presence of major cardiac anomalies were significant prognostic factors for mortality, and major cardiac anomalies affected mortality more than birth weight. The ROC curve for birth weight suggested that 2,000 g was an appropriate cutoff point. The Spitz classification was revised as follows: the revised class I (low-risk group) consisted of patients without major cardiac anomalies and birth weight >2,000 g; class II (moderate-risk group) consisted of patients without major cardiac abnormalities and birth weight <2,000 g; class III (relatively high-risk group) consisted of patients with major cardiac anomalies and birth weight >2,000 g; and class IV (high-risk group) consisted of patients with major cardiac anomalies and birth weight <2,000 g. The ROC comparisons showed that the revised classification provided a significant improvement (P = .049). CONCLUSION This revised classification can improve the stratification of EA patients and be a useful predictor of survival.

Aortopexy with tracheal reconstruction for postoperative tracheomalacia in congenital tracheal stenosis.

PURPOSE Congenital tracheal stenosis is a rare condition and can be difficult to manage. One source of difficulty is postoperative tracheomalacia requiring long-term tracheal stenting. To prevent symptomatic postoperative tracheomalacia, we have been adding aortopexy to tracheal reconstruction since 2008. The aim of this study was to evaluate efficacy of aortopexy for preventing postoperative tracheomalacia after reconstruction of congenital tracheal stenosis. METHODS Retrospective chart review was conducted. From October 2003 to March 2011, 24 had tracheal reconstruction without aortopexy (group A) and 8 with aortopexy (group B). Statistical analysis was performed using Fishers Exact test. RESULTS One had anastomotic leakage in group A, and 1, in group B (P = .44). Eleven patients required tracheostomy because of postoperative tracheomalacia confirmed by postoperative bronchoscopy in group A vs none in group B (P = .029). CONCLUSIONS We found that aortopexy with tracheal reconstruction reduced the need for postoperative tracheostomy in this patient group. Although there is a potential risk of anastomotic leakage because of the suspension suture on the anterior tracheal wall to aorta, we did not detect an increased incidence after aortopexy. Thus, aortic suspension may be a useful adjunct to prevent symptoms of tracheomalacia in these patients.

Treatment of postoperative tracheal granulation tissue with inhaled budesonide in congenital tracheal stenosis.

PURPOSE Tracheal obstruction by granulation tissue can compromise the postoperative course in congenital tracheal stenosis (CTS). Balloon dilatation and stenting may be required. Budesonide is a corticosteroid with topical anti-inflammatory effects. In 2008, we used inhaled budesonide for treatment of postoperative granulation tissue for the first time in CTS, resulting in significant improvement. The aim of this study was to evaluate the efficacy of inhaled budesonide for treatment of postoperative granulation tissue in CTS. METHODS Retrospective chart review was conducted. From 2004 through 2011, we performed 39 tracheoplasties. Forced stenting ± balloon dilatation (S/B) was required when airway obstruction with tissue granulation was life-threatening. We compared the requirement for S/B between the early group without budesonide (2004-Nov. 2008, Early) and the late group with budesonide (Dec. 2008-2011, Late). Statistical analysis was performed using Fishers Exact test. RESULTS Eleven of 22 in Early and 8 of 17 in Late were successfully extubated, never having had life-threatening tissue granulation. The remaining patients in each group (11 in Early and 9 in Late) required tracheostomies due to postoperative complication. Ten in Early and 5 in Late with tracheostomies developed granulation tissue. Of these patients, the 10 in Early required S/B, while none of the 5 in Late required S/B (P=.0003). Bronchoscopy demonstrated significant regression of granulation tissue in all cases treated with inhaled budesonide. CONCLUSION Inhaled budesonide is effective for treatment of tracheal granulation tissue in patients with tracheostomies after repair of CTS.

Long-term T-tube stenting as definitive treatment of severe acquired subglottic stenosis in children

PURPOSE We analyzed our results of long-term T-tube stenting for severe acquired subglottic stenosis secondary to prolonged endotracheal intubation in a neonatal period. MATERIALS AND METHODS Twenty children treated with T-tube stenting since 1999 were retrospectively analyzed. T-tube stenting consisted of anterior cricoid split and placing silicon T-tube as a stent for expanded subglottic lumen. RESULTS Fourteen of 20 patients were treated as an initial operation and 6 patients as an additional operation after other failed procedures. Decannulation was achieved in 8 (57.1%) of 14 children in the initial operation group and in 4 (66.7%) of 6 patients in the additional operation group. The average duration of T-tube stenting was 16.1 months in the initial group and 65.8 months in the additional group. Four of 20 patients continued to require T-tube stenting. In the other 4 patients, T-tubes were switched to tracheostomy tubes. The voice quality improved gradually after decannulation. CONCLUSION T-tube stenting for severe acquired subglottic stenosis is recommended as a best available treatment in postoperative quality of life. T-tube stenting should be performed as the initial treatment because the time to decannulation was shorter than as an additional operation.

Innominate artery compression of the trachea in patients with neurological or neuromuscular disorders

OBJECTIVES The purpose of this study was to review and assess our surgical management of innominate artery compression of the trachea (IACT) in patients with neurological or neuromuscular disorders (NMDs). METHODS Thirty patients with NMD who underwent surgical treatment for IACT at Kobe Childrens Hospital and Kobe University Hospital from 2002 to 2012, were enrolled in this retrospective study. The clinical outcomes of preventive elective surgery for IACT (Group A, n=20) were assessed and compared with those of emergent surgery (Group B, n=10). RESULTS A total of 27 patients underwent innominate artery transection (17 in Group A and 10 in Group B), and 3 patients in Group A underwent innominate artery reimplantation using prosthetic graft interposition. No operative or early death occurred. There were no cases of postoperative mediastinitis or neurological complications. The operative benefits in Group A included a smaller skin incision, more limited sternotomy, less blood loss, shorter operative time and shorter hospital stay, compared with Group B. No blood transfusion was required in Group A. The number of patients in whom cerebral circulation was assessed before surgery in Group A was significantly higher than those in Group B. CONCLUSIONS Preventive elective surgery for IACT provides many advantages, including minimally invasive procedures and successful postoperative outcomes without neurological complications in patients with NMD. Because this surgical management can prevent the tragic occurrence of a tracheo-innominate artery fistula or an exacerbation of tracheomalacia, it would be an optimal surgical treatment for IACT to improve the quality of life in patients with NMD.

Bronchoscopic assessments and clinical outcomes in pediatric patients with tracheomalacia and bronchomalacia

BackgroundTracheomalacia and bronchomalacia (TM/BM) are one of the serious causes of airway obstruction in infants and children. This study reviewed our bronchoscopic assessments and clinical outcomes in pediatric patients with TM/BM, and investigated risk factors of surgical intervention for TM/BM.MethodsFifty-seven consecutive patients who were diagnosed as TM/BM by bronchoscopy between 2009 and 2013 were reviewed retrospectively. They were divided into two groups according to the presence (group E, n = 26) or absence (group N, n = 31) of acute life-threatening events and extubation failure (ALTE/EF). The severity of TM/BM was evaluated by Oblateness Index which was obtained from bronchoscopic images.ResultsOblateness Index was significantly higher in Group E than in Group N. Patients in Group E underwent surgical intervention for TM/BM more frequently, and had significantly longer intubation period and hospital stay. Clinical symptoms of ALTE/EF, Oblateness Index ≥ 0.70, and multiple malacic lesions were significant risk factors indicating surgical events in patients with TM/BM.ConclusionsPatients with TM/BM who had ALTE/EF had more severe malacic lesions indicating surgical intervention, and worse clinical outcomes. Oblateness Index is a simple and semi-quantitative index for bronchoscopic assessment of TM/BM, and can be one of the prognostic tools to predict clinical severity of pediatric TM/BM.

Thoracoscopic repair of a large neonatal congenital diaphragmatic hernia using Gerota's fascia

A large congenital diaphragmatic hernia needing patch repair has a high risk of recurrence. Thus, managing these large congenital diaphragmatic hernias under thoracoscopy has become a problem. Here, a large congenital diaphragmatic hernia that was repaired using Gerotas fascia under thoracoscopy is reported. In the present case, it was impossible to close the hernia directly under thoracoscopy because the hernia was too large. Gerotas fascia was raised up by the left kidney and used for the repair. The left colon adhering to Gerotas fascia was mobilized, and a large space was made under thoracoscopy. Gerotas fascia was fixed to the diaphragmatic defect. The patients postoperative course was good, and there was no recurrence. This technique could be one option for repairing a large hernia under thoracoscopy.

Three-Dimensional Computed Tomographic Findings of Bridging Bronchus with Pulmonary Artery Compression

Afemale infant was born at 40 weeks gestation with a birth weight of 3040 g. At 4 hours after birth, she was admitted and intubated for respiratory distress. Chest radiograph showed a shift of the mediastinal structures to the right. Echocardiography showed no major heart malformation. She was successfully extubated after 17 hours of mechanical ventilation, but mild inspiratory stridor still continued. Three-dimensional computed tomography demonstrated bridging bronchus (BB) with pulmonary artery compression (Figure). The infant was discharged on the 21st day after birth. Since the age of 15 months, she has been hospitalized several times with bronchitis; however, she has not required surgical intervention for airway obstruction. BB is an extremely rare bronchial branching anomaly. 1-3 Three-dimensional computed tomography is a powerful means for demonstrating the special relationships of arterial and tracheal anomalies. n

Trisomy 8 mosaicism with pyloric atresia and situs ambiguous

A 2544 g male neonate was born at 35 weeks 4 days’ gestation to a 28-year-old, gravida 3, para 2 mother via elective cesarean section. She did not take any medications during pregnancy. The mother was transferred to Hyogo Prefectural Kobe Children’s Hospital Perinatal Center due to polyhydramnios and fetal Correspondence: Sota Iwatani, MD, Department of Neonatology, Hyogo Prefectural Kobe Children’s Hospital Perinatal Center, 1-1-1 Takakuradai, Suma-Ku, Kobe, Hyogo 654-0081, Japan. Email: iwatani_kch@hp.pref.hyogo.jp Received 31 March 2014; revised 15 July 2014; accepted 2 September 2014. a b

Congenital mesoblastic nephroma with focal anaplastic lesion

To the Editor: Congenital mesoblastic nephroma (CMN) is a rare renal tumor that comprises spindle cells and most often arises in newborns and infants. CMNs are classified into three types: cellular, classic, and mixed type. Cellular type CMN expresses the same chimeric protein ETV6-NTRK3 as does infantile fibrosarcoma and is therefore regarded as infantile fibrosarcoma within the kidney. Pathologically, cellular type CMN is sometimes difficult to distinguish from clear cell sarcoma of the kidney (CCSK) because CCSK is also a childhood, renal, mesenchymal tumor. However, differentiating cellular type CMN from CCSK is clinically important because therapy after nephrectomy and prognosis differ greatly between these cancers. CCSKs display several variations of histological patterns, and an anaplastic pattern is seen in approximately 2.6% of primary and recurrent CCSKs. To our knowledge, no cellular type CMN or infantile fibrosarcoma with an anaplastic pattern has been reported. Here, we describe a renal spindle cell tumor with focal anaplastic lesion in a pediatric patient. The presence of focal anaplastic lesion made diagnosis of CMN difficult, and verifying expression of the oncogenic ETV6-NTRK3 fusion was the key to accurately diagnosing cellular type CMN. This case demonstrates that focal anaplastic lesion may be seen in cellular type CMN. The patient was a boy aged one year and five months. His family noticed abdominal enlargement when he was 1 year old, but did not consider the condition serious because his abdomen was soft and he was afebrile and not vomiting. However, his abdomen gradually became harder and more enlarged. He was admitted to our hospital at the age of 1 year and 5 months. A right renal tumor measuring 9 × 12 cm with necrosis was found on abdominal enhanced computed tomography. No distant metastasis, lymphadenopathy, or tumor infiltration into the inferior vena cava was detected. Right nephrectomy was performed. The right kidney was resected with the right ureter and right adrenal gland; this complex weighed 1.1 kg and measured 15 × 12 × 12 cm. No rupture of the capsule was evident. On cut section, the tumor was soft and displayed variegated color with the dark red of hemorrhage and the brown of necrosis in the center and a white-yellow color at the margins. The tumor was fairly well demarcated. Samples for reverse transcriptase-polymerase chain reaction (RT-PCR) were taken from the white-yellow peripheral part of the tumor. On microscopic examination, the tumor margin was sharply and linearly demarcated or had infiltrated only a short distance into the renal parenchyma with a pushing border. The tumor was within the kidney and had not invaded into the renal capsule. No invasion into the renal pelvis or adipose tissue in the renal sinus was found. The cut ends of the ureter and the renal artery and vein were tumor-negative. An old hematoma and wide necrosis were centered within the tumor, and microscopic necrotic foci were scattered within the tumor. The tumor entrapped isolated renal tubules or glomeruli. The tumor comprised interlacing fascicles of spindle cells with bipolar cytoplasmic processes and round to slightly elongated nuclei containing fine chromatin and a few (1 or 2) tiny nucleoli (Fig. 1a). A storiform pattern of cells was observed. Mitotic counts per one high-power field (HPF) were 1 to 7 (mean approximately 4), but karyorrhexis was more frequent than mitosis. Cells with bizarre giant nuclei or with multiple nuclei and with abundant eosinophilic cytoplasm were found in the sharply demarcated nodular region that measured 4 × 2 mm (Fig. 1b). Multipolar mitotic figures were also observed in this region. So these cells fulfill the criteria of anaplasia defined for nephroblastoma, CCSK, and anaplastic sarcoma of the kidney (ASK). There were no transitional features between the anaplastic lesion and the surrounding