Pitfalls in the management of congenital tracheal stenosis: is conservative management feasible?

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.

Impact of botulinum toxin A injection on esophageal anastomosis in a rabbit model.

PURPOSE: The management of esophageal atresia is established, but the rate of postoperative complications remains high. We focused on a new, recently reported method of esophageal elongation using botulinum toxin type A (BTX-A) and evaluated the efficacy of BTX-A injection around esophageal anastomoses with tension in a rabbit model. METHODS: Twenty rabbits aged 8-10 weeks and weighing 1.27-1.72 kg underwent resections of the esophagus measuring 1.5 cm long using an anterior cervical approach. Esophagoesophagostomies were performed after intramural administration of Xeomin™ (3 U/body) in the BTX-A group and saline in the control group. Morphological and histological evaluations were examined on postoperative day 14. RESULTS: Six rabbits in each group survived. The BTX-A group showed significantly less postoperative anastomotic stricture and less fibrosis than the control group. Changes in wall thickness on both sides of the anastomotic areas were equivalent between the two groups, and no muscle fracturing was observed. CONCLUSION: Local administration of BTX-A for esophagoesophagostomy significantly reduced postoperative anastomotic stricture with less fibrosis than that observed in the control group. Reduced anastomotic tension with BTX-A presumably contributed to better anastomotic healing. Determining the optimum dose of BTX-A is necessary for clinical application.

New operative strategy for refractory microcystic lymphangioma.

The optimal management of microcystic lymphatic malformations (LMs) in children has not been established. We describe how we used the Ligasure™ 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.

Balloon tracheoplasty as initial treatment for neonates with symptomatic congenital tracheal stenosis.

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.

Surgical reconstruction and endoscopic pancreatic stent for traumatic pancreatic duct disruption.

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 efficacy of double-balloon enteroscopy for intrahepatic bile duct stones after Roux-en-Y hepaticojejunostomy for choledochal cysts.

INTRODUCTION: Intrahepatic bile duct (IHBD) stones are one of the most complicated morbidities that occur after Roux-en-Y hepaticojejunostomy (RYH); however, the optimal therapeutic approach is controversial. METHODS: Double-balloon enteroscopy (DBE) has been widely and frequently performed even in pediatric patients. We herein report the successful management of IHBD stones by biliary lithotripsy using DBE after RYH for a choledochal cyst (CC). DBE has made it possible to perform endoscopic therapeutic intervention, including balloon dilatation of an anastomotic stricture and removal of IHBD stones, without any major complications. CONCLUSION: DBE is a less invasive and safe treatment method for IHBD stones in pediatric patients, which is capable of reaching the bilioenteric anastomosis after RYH for CC.

A novel diagnostic and treatment strategy for small intestinal lesions in children: hybrid treatment using transumbilical minimal incision surgery combined with double-balloon enteroscopy.

INTRODUCTION: Double-balloon enteroscopy (DBE) is a useful and feasible modality for evaluating small intestinal lesions, even in children. METHODS: DBE makes it possible to perform biopsy, diagnosis, polypectomy and endoscopic therapies including hemostasis, tattooing and clipping of the small intestinal lesions. However, endoscopic procedures in the small intestines of children are more difficult than in adults, because the intestinal wall is thin and the lumen is narrow. A novel hybrid treatment was developed using DBE for small bowel lesions combined with transumbilical minimal incision surgery. CONCLUSION: This hybrid treatment is safe, effective, provides excellent cosmetic results and can be used as an alternative for traditional open laparotomy or endoscopic surgery.

Recanalized umbilical vein as a conduit for mesenterico/porto-Rex bypass for patients with extrahepatic portal vein obstruction.

PURPOSE: Mesenterico-left portal vein (meso-Rex) bypass is as an effective modality for restoring intrahepatic portal perfusion in patients with extrahepatic portal vein obstruction. Achieving sufficient patency is difficult with end-to-side anastomosis of a bypass graft to a small or hypoplastic left portal vein in the Rex recessus. Here, we describe the use of a recanalized umbilical vein in the round ligament as a conduit for bypass construction in two patients. METHODS: Case 1 was an 11-year-old boy diagnosed with rupture of the esophageal varices and hypersplenism due to congenital extrahepatic portal hypertension. Because of persistent hypersplenism and thrombocytopenia, he underwent meso-Rex bypassing with a left iliac vein graft interposed between the umbilical vein and the superior mesenteric vein. Case 2 was a neonate with a large hepatic tumor (mesenchymal hamartoma) that developed abdominal compartment syndrome at birth. The tumor was removed by right hepatectomy with excision of the portal vein bifurcation at 3 days of age. Porto-Rex bypassing was accomplished by end-to-end anastomosis between the portal vein trunk and the umbilical vein. RESULTS: Sufficient hepatopetal portal flow through the umbilical vein was achieved in both patients and maintained for over 16 and 13 months, respectively. Although hypersplenism remained in Case 1, intrahepatic portal vein branches gradually widened and the cavernoma in the hepatic hilum disappeared within 2 months. Neither patient had symptoms or signs of portal hypertension at the most recent follow-up. CONCLUSION: Using the umbilical vein as a vein conduit may facilitate construction of a meso/porto-Rex bypass and restore intrahepatic portal vein perfusion in patients with extrahepatic portal vein obstruction.

Effect of dai-kenchu-to on obstructive bowel disease in children.

The aim of this study was to evaluate the effects of a traditional Chinese herbal medicine, dai-kenchu-to (DKT), on obstructive bowel diseases in children. We have treated 46 pediatric patients with various obstructive bowel diseases with DKT: six patients with postoperative ileus, 12 with large abdominal surgery (including three neonates), one with ano-rectal anomaly, three with Hirschsprung's disease, two with functional bowel obstructions, one with SMA syndrome, and 21 patients with chronic constipation. DKT (0.1-0.15 g/kg) was mixed with 5-10 ml of warm water, and was given orally two to three times a day. DKT was effective for 39 patients (85%) and their clinical symptoms improved. DKT was ineffective in seven patients: two with postoperative ileus, two with Hirschsprung's disease, and three with chronic constipation. DKT had mild but significant effects for various obstructive bowel diseases in children, while no side effects were encountered. Our current strategy for pediatric patients with obstructive bowel disease is to use DKT first and then test its efficacy. If DKT is effective, the regimen is continued. However, in cases where DKT is not effective, we will consider laparotomy or will further investigate the illness.

A new paradigm of scarless abdominal surgery in children: transumbilical minimal incision surgery.

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.

Papillary carcinoma with extensive squamous metaplasia arising from thyroglossal duct cyst in an 11-year-old girl: significance of differentiation from squamous cell carcinoma: a case report.

We report a case of papillary carcinoma (PC) with extensive squamous metaplasia arising from a thyroglossal duct cyst (TDC) that required differential diagnosis from squamous cell carcinoma (SCC). An 11-year-old Japanese girl presented with a 9-month history of an anterior-midline neck mass that was clinically diagnosed as TDC. Open neck biopsy revealed nested proliferation of atypical squamous cells within the cystic structures, and SCC arising from TDC was initially suspected. Further examination, however, including immunohistochemistry, revealed the tumor to be of thyroid cell origin. The patient underwent wide local resection of the thyroglossal duct carcinoma by Sistrunk procedure and cervical lymph node dissection. Microscopically, the diagnosis was of PC with extensive squamous metaplasia and metastasis to the medial submandibular lymph node. Distinction of squamous metaplasia in PC from SCC is sometimes difficult, but has a significant effect on postoperative management.

Severe gastric damage caused by button battery ingestion in a 3-month-old infant.

Ingestion of a button battery has been considered a serious problem, causing necrosis and perforation, when impacted in the esophagus. However, such batteries in the stomach rarely cause any harm to the gastric wall, which is regarded as evidence supporting the use of conservative treatment. We present the rare case of a 3-month-old infant with severe gastric wall injury caused by a button battery lodged in the stomach. The present case suggests that button batteries located in the stomach should be removed as soon as possible, especially in infants.

Experimental and numerical investigations of axisymmetric wave propagation in cylindrical pipe filled with fluid.

Acoustic wave propagation in fluid-filled cylindrical pipe with arbitrary thickness is investigated numerically and experimentally. The vibrational properties of the coupled fluid-pipe system are evaluated by a layerwise approach, which is similar to the finite-strip method. In this approach, the thick cylindrical wall is divided into a number of thin cylindrical layers in the thickness direction. The displacements in the thickness direction for each layer are approximated by linear-shape functions. The governing equation is obtained by using an energy minimization principle. The dispersion curves, distribution of vibrational energy between pipe wall and contained fluid, and displacement fields are examined. The dependence of the dispersion curves on wall thickness is discussed. Two PZT ring transducers adhered to the outer surface of pipe are used as source and receiver, respectively. The propagating waves generated by burst signals are measured. To localize transient signal both in time and frequency domains, the discrete wavelet transform is applied to decomposing the receiving signal into several components. Each component is limited to a narrower bandwidth. Therefore the frequency-dependent group velocity is estimated. The experimental and numerical results are compared.

Management for fistula-in-ano with Ginseng and Tang-kuei Ten Combination.

BACKGROUND: Non-operative management for patients with fistula-in-ano in infancy using a traditional herbal medicine Ginseng and Tang-kuei Ten Combination (GTTC) is described. METHODS: A total of 22 patients with peri-anal abscess or fistula-in-ano were treated with GTTC. Eighteen patients showed no improvement using the conventional non-operative management (frequent curettage of abscess cavity), after which administration of GTTC was commenced. The other four patients received GTTC from the beginning of their treatment. A dose of 0.1-0.2 g/kg of GTTC was given orally twice a day. The period between onset of peri-anal abscess and the commencement of GTTC (pre-GTTC period) was compared to the period until remission after administration of GTTC (post-GTTC period). The relationship between the GTTC dose, age at onset of peri-anal abscess, post-GTTC period, and relapse rates were investigated. RESULTS: Twenty-one patients reached remission, but a 4-year-old boy failed to reach remission and he required fistelectomy. Pre-GTTC periods were from 11 days to 3 years (median 50 days) and statistically longer than post-GTTC periods (4-65 days, median 7 days). There was a reverse correlation between the age at onset of peri-anal abscess and post-GTTC period (r = -044, P < 0.05). There was no statistical correlation between the dose of GTTC and post-GTTC period. Five patients showed relapse after discontinuing GTTC, all of them could be resolved with augmentation of the dose. The onset of peri-anal abscess in patients who relapsed were all < 3 months of age. CONCLUSION: Ginseng and Tang-kuei Ten Combination seemed to accelerate the recovery of patients with fistula-in-ano in infancy.