Indications for tracheostomy in children with head and neck lymphatic malformation: analysis of a nationwide survey in Japan.

PURPOSE: Airway obstruction caused by lymphatic malformation (LM) in the head and neck may require a tracheostomy. We present the results of our analysis of a nationwide survey on the indications for tracheostomy in children with head and neck LM. METHODS: We analyzed data in relation to tracheostomy based on a questionnaire about 518 children with head and neck LM without mediastinal involvement. RESULTS: Tracheostomy was performed for 43 of the 518 children. Most (32/43) of these children were younger than 1 year of age and the tracheostomy was almost always performed for airway obstruction (40/43). The lesion was in contact with the airway in 32 (72%) of these children, but in only 58 (12%) of the 473 children who were managed without tracheostomy. When the maximum circumferential area of contact was compared, only 20 (27%) of 74 patients with maximum contact of less than a half-circle required tracheostomy, whereas 11 of 13 with maximum contact of more than a half-circle required tracheostomy (P = 0.0001). Six patients without airway contact required tracheostomy because of acute swelling caused by hemorrhage, infection, or both. CONCLUSIONS: Children with head and neck LM required tracheostomy to relieve airway obstruction. Tracheostomy should be considered if the lesion is in contact with the airway and surrounds more than a half-circle, and when it causes acute swelling.

Treatment of mediastinal lymphatic malformation in children: an analysis of a nationwide survey in Japan.

PURPOSE: Clinical guidelines on lymphatic malformation (LM) influencing the airway have been crafted in the Research Project for Intractable Diseases. We herein report an analysis of a nationwide survey of mediastinal LM and the therapeutic recommendations. METHODS: Eighty-seven registered cases with mediastinal involvement were analyzed with a review of the literature. RESULTS: Mediastinal LM was located more often in the upper and anterior mediastinum and was found without any accompanying symptoms in 56/87 cases. Tracheostomy was required in 23/87 cases, mostly < 2 years of age (87%). All patients who needed tracheostomy had a lesion in contact with the airway, while only 55% of those without tracheostomy had contact. Tracheostomy tended to be placed more when the longer segment of the airway was in contact with the LM. Multimodal treatments were performed in 29 patients, but the lesions remained in most cases, and chylothorax, hemorrhaging, nerve palsy, and infections were noted as complications. CONCLUSIONS: In patients with mediastinal LM, tracheostomy may be necessary, especially when the lesion is extensive and contacts the airway. Extirpation of the mediastinal LM may be the only therapeutic option, but in cases with few or no symptoms, non-surgical treatment should be considered in light of potential postoperative complications.

Endoscopic chemocauterization for pyriform sinus fistula in children.

BACKGROUND: Although complete excision is the standard treatment for pyriform sinus fistula (PSF), it has recently been suggested that chemocauterization via the internal orifice of the fistula could be an effective non-invasive treatment for the condition. The present report describes the details of our experience with and the efficacy of endoscopic chemocauterization for pediatric PSF. METHOD: Between January 2010 and June 2015, four patients were diagnosed with PSF and scheduled to undergo endoscopic chemocauterization. Under general anesthesia, trichloroacetic acid (TCA) solution was endoscopically injected through the opening of the fistula using a fine plastic tube. Esophagogram was obtained at 3 weeks after the procedure to search for recurrence. Complete closure of the fistula was confirmed on endoscopy at 2-3 months after chemocauterization. RESULTS: Two of the four patients were treated once, and the remaining patients required further chemocauterization procedures. No recurrence developed in any patient after it had been confirmed that the fistula's internal orifice had been obliterated. Some transient complications, such as sore throat, nausea or temporary vocal fold paresis, occurred. CONCLUSION: Chemocauterization with TCA seems to be a useful first-choice treatment for PSF.

Reappraisal of adhesive strapping as treatment for infantile umbilical hernia.

BACKGROUND: Most umbilical hernias spontaneously close by 3-5 years of age; therefore, surgical repair is considered only in children whose hernias have not closed by this point. At present, adhesive strapping is not the preferred treatment for umbilical hernias because of the lack of supporting evidence regarding its efficacy, and its association with skin complications. This aim of this study was to examine umbilical hernia closure on ultrasonography, and reassess the merits of adhesive strapping. METHODS: Between January 2013 and December 2014, 89 infants underwent adhesive strapping for umbilical hernia. The strapping was changed once a week. The diameter of the hernia orifice was measured on ultrasonography every 2 weeks until closure. The closure speed (CS) of the hernia orifice was compared between the infants treated with adhesive strapping and those undergoing observation alone. The association between CS and birthweight, gestational age, diameter of the hernia orifice, and timing of treatment (before 12 weeks of age vs between 12 and 26 weeks of age) was also analyzed. RESULTS: Closure was achieved after 2-13 weeks of strapping in 81 infants (91%), and the likelihood of closure was not affected by the diameter of the hernia orifice, gestational age, or the timing of treatment. The mean CS of the infants treated with adhesive strapping was significantly faster than that of the infants undergoing observation alone (2.59 vs 0.37 mm/week, P < 0.05). Adhesive strapping was discontinued in five of the 89 infants (5.6%) due to severe skin complications. CONCLUSION: Adhesive strapping promoted early spontaneous umbilical hernia closure compared with observation alone, regardless of the diameter of the hernia orifice. Adhesive strapping is an effective alternative to surgery and observation.

Application of nuclear medicine to achieve less invasive surgery for malignant solid tumors in children.

BACKGROUND: The use of nuclear medicine for the management of malignant tumor, such as radioguided surgery and sentinel lymph node biopsy (SLNB), has been widely accepted in the adult practice. However, there are very few studies to apply those techniques for pediatric diseases. The aim of this study was to investigate the feasibility of application of nuclear medicine in surgery for neuroblastoma (NB) or rhabdomyosarcoma (RMS) in children. METHODS: Radioguided surgery using (123) I-metaiodobenzylguanidine was performed on six children with NB. SLNB using technetium-labeled tin or sulfur colloid was performed on two children with perineal RMS. Histological evaluation of resected specimens was performed to determine the accuracy of intraoperative detection and SLNB. All patients were evaluated for overall survival and complications. RESULTS: Intraoperative tumor localization using hand-held gamma probe was helpful in 85.7% of NB patients. Sensitivity and specificity of this technique were 81.8% and 93.3%, respectively. There were no postoperative complications, and four out of five patients with high-risk NB experienced disease-free survival (median follow up, 57 months). Sentinel lymph nodes were easily detected in patients with perineal RMS, and histological assessment revealed complete consistency with regional lymph node status. CONCLUSIONS: Nuclear medicine may have a potential application in the use of less invasive surgery for advanced NB or perineal RMS, the two most challenging pediatric malignancies.

Effect of an omega-3 lipid emulsion in reducing oxidative stress in a rat model of intestinal ischemia-reperfusion injury.

OBJECTIVES: The usefulness of omega-3 lipid emulsions has been extensively studied. The objectives of the present study were to examine the effect of an omega-3 lipid emulsion in reducing oxidative stress in a rat model of intestinal ischemia-reperfusion injury and the underlying mechanism. METHODS: A total of 66 rats were divided into three dietary groups (lipid-free, soybean oil, and fish oil groups). Each animal was administered total parenteral nutrition for 3 days, followed by induction of intestinal ischemia for 100 min. Animals subjected to sham surgery served as the controls. Intestinal tissue and blood were harvested 6 and 12 h after the surgery, then, assessment of the histological damage score, plasma-related parameters, and statistical evaluation were performed. RESULTS: The histological damage score in the intestinal tissues was significantly lower in the fish oil group than in the soybean oil group (P = 0.0121). The late-phase urinary level of 8-hydroxy-2-deoxyguanosine was also significantly lower in the fish oil group as compared with that in the other groups (P = 0.0267). Furthermore, the plasma level of high-mobility group box 1 protein was also significantly lower in the fish oil group as compared with that in the lipid-free group (P = 0.0398). CONCLUSION: It appeared that intravenous administration of an omega-3 lipid emulsion prior to ischemia-reperfusion injury reduced the oxidative stress and severity of tissue damage. Modification of membrane fatty acids may serve as the mechanism underlying this reduction of tissue damage.

A long-term survival case of tracheal agenesis: management for tracheoesophageal fistula and esophageal reconstruction.

Tracheal agenesis is a very rare disorder which leads to severe respiratory disorders immediately after birth. Reports are very limited on long-term survival cases. We report here a long-term survival case with Floyd's type I tracheal agenesis. During the neonatal stage, the patient underwent abdominal esophageal banding to substitute esophagus for trachea and transection at the cervical esophagus with esophagostomy. Subsequently, airway management was difficult due to a fragile tracheoesophageal fistula, but the fistula was conservatively treated and stabilized with the patient's growth. This patient is a very rare case in whom oral feeding was achieved after esophageal reconstruction using a gastric tube. For this case, we describe mainly (1) the management method of the tracheoesophageal fistula and (2) esophageal reconstruction without thoracotomy.

Skill qualifications in pediatric minimally invasive surgery.

PURPOSE: In 2006, The Japanese Society of Pediatric Endoscopic Surgeons devised a plan to develop a pediatric endoscopic surgical skill qualification (ESSQ) system. This system is controlled by The Japan Society for Endoscopic Surgery. The standard requirement for skills qualification is the ability of each applicant to complete common types of laparoscopic surgery. The main goal of the system is to decrease complications of laparoscopic surgery by evaluating the surgical skills of each applicant and subsequently certify surgeons with adequate skills to perform laparoscopic operations safely. METHODS: A committee of pediatric ESSQ created a checklist to assess the applicant's laparoscopic surgical skills. Skills are assessed in a double-blinded fashion by evaluating an unedited video recording of a fundoplication for pediatric gastroesophageal reflux disease. RESULTS: The initial pediatric ESSQ system was started in 2008. In 2008 and 2009, respectively, 9 out of 17 (53%) and 6 out of 12 (50%) applicants were certified as expert pediatric laparoscopic surgeons. CONCLUSIONS: Our ultimate goal is to provide safe and appropriate pediatric minimally invasive procedures and to avoid severe complications. To prove the predictive validity of this system, a survey of the outcomes of operations performed by certified pediatric surgeons is required.

Idiopathic hypereosinophilic syndrome in a case with ABO-incompatible liver transplantation for biliary atresia complicated by portal vein thrombosis.

Idiopathic HES is characterlized by prolonged eosinophilia without an identifiable underlying cause and multiple-organ dysfunction. We report a case of a LDLT for a 12-yr-old Japanese girl with BA accompanied by HES. Histological examination of the resected liver showed biliary cirrhosis with dense eosinophilic infiltration of portal tracts and the lobules of the liver. She developed portal vein thrombosis on post-operative day 10 and the histopathological findings of the thrombus revealed dense eosinophilic deposition, suggesting that HES might have influenced the formation of this thrombus. Liver graft biopsies also demonstrated the presence of activated eosinophilils with biliary damage. Blood chemistry findings suggested liver dysfunction as a result of the eosinophilic infiltrations. Prednisolone treatment improved the liver dysfunction. Four years after LDLT, she remains clinically well on prednisolone at 0.3 mg/kg/day, with an eosinophil count ranging from 10 to 15%. A literature review has not shown any previous reports of HES with BA. This case demonstrates the possibility of an association between eosinophilic infiltration and liver dysfunction during follow-up for BA and after LDLT.

The developments and achievements of endoscopic surgery, robotic surgery and function-preserving surgery.

The breakthrough in laparoscopic surgery has been the development of a charge-coupled device camera system and Mouret performing cholecystectomy in 1987. The short-term benefits of laparoscopic surgery are widely accepted and the long-term benefit of less incidence of bowel obstruction can be expected. The important developments have been the articulating instrumentation via new laparoscopic access ports. Since 2007, single-incision laparoscopic surgery has spread all over the world. Not only single-scar but also no-scar operation is a current topic. In 2004, Kalloo reported the flexible transgastric peritoneoscopy as a novel approach to therapeutic interventions. In 2007, Marescaux reported transvaginal cholecystectomy in a patient. The breakthrough in robotic surgery was the development of the da Vinci Surgical System. It was introduced to Keio University Hospital in March 2000. Precision in the surgery will reach a higher level with the use of robotics. In collaboration with the faculty of technology and science, Keio University, the combined master-slave manipulator has been developed. The haptic forceps, which measure the elasticity of organs, have also been developed. The first possible sites of lymphatic metastasis are known as sentinel nodes. Otani reported vagus-sparing segmental gastrectomy under sentinel node navigation. This kind of function-preserving surgery will be performed frequently if the results of the multicenter prospective trial of the dual tracer method are favorable. Indocyanine green fluorescence-guided method using the HyperEye charge-coupled device camera system can be a highly sensitive method without using the radioactive colloid. 'Minimally invasive, function-preserving and precise surgery under sentinel node navigation in community hospital' may be a goal for us.

Newly developed haptic forceps enables sensitive, real-time measurements of organ elasticity.

Currently available master-slave manipulators cannot recognize the elasticity of organs or tissues. The aim of this study was to examine whether a newly developed haptic forceps using a linear motor could measure the elasticity of living organs using an animal model. We measured the elasticity values and the disruption limit values of rat organs using the new haptic forceps. The elasticity of the materials was calculated using the formula "power / position", with N/m as the unit. We successfully and reproducibly measured the changes in the elasticity values of various materials in real time. We were also able to perceive tactile changes transmitted through the forceps. The changes in gastrointestinal contraction were synchronized with the visually observed changes, and these changes were monitored and measured as elasticity values in real time using the forceps. The damage limits were also successfully measured. In conclusion, the new haptic forceps enabled highly sensitive, real-time measurements of elasticity in living rat organs. The use of this forceps enables the disruption limit values of organs to be measured, and the device could be useful for setting safety limits when grasping organs during endoscopic surgery.

Prenatal diagnosis of retroperitoneal teratoma: a case report and review of the literature.

We report a case of retroperitoneal teratoma diagnosed prenatally by serial sonographic examinations in the third trimester. A 29-year-old woman was referred for sonographic evaluation at 33 weeks' gestation because of a fetal intra-abdominal mass. Our initial sonographic image suggested a neuroblastoma. Repeat ultrasound images demonstrated an increase in size of the tumor, while the content of the tumor became predominantly solid with areas of calcification. Teratoma should be considered on detection of any cystic or mixed semisolid mass, especially when calcification is present. The fetus was prenatally diagnosed with retroperitoneal teratoma. After birth at 39 weeks, the tumor was removed and histological analysis revealed an immature retroperitoneal teratoma. Intensive monitoring of the changes in ultrasound images of the tumor should provide ground for a precise antenatal diagnosis.

The role of graft and host accommodation in a hamster-to-rat cardiac transplantation model.

BACKGROUND: We evaluated the importance and mechanism of graft and host accommodation in hamster-to-rat cardiac xenotransplantation models. METHODS: To evaluate graft accommodation, accommodated hamster grafts (Group 2) were transplanted to naïve host rats treated with FK506, and compared with naïve hamster grafts (Group 1). To evaluate host accommodation, three groups were evaluated: naive hamster hearts were transplanted to naïve hosts treated with FK506 (Group 3: 0.5 mg/kg, Group 4: 1.0 mg/kg) and splenectomy, and compared with accommodating hosts (Group 5) with FK506 0.5 mg/kg and splenectomy. We examined graft survival, histopathology, antihamster antibodies and B-1 cells in blood. RESULTS: Graft survival in Group 2 (3.4+/-0.9 days) was not significantly different from that in Group 1 (2.8+/-0.4 days). Graft survival in Groups 4 and 5 (>30 days) was significantly prolonged compared with that in Group 3 (6.0+/-0.7 days). Histopathology of Groups 1-3 showed humoral rejection, whereas Groups 4 and 5 showed normal histology and expression of protective genes. In Groups 1-3, antihamster immunoglobulin (Ig) M and B-1 cells increased significantly compared to Groups 4 and 5, where IgM and B-1 cells remained low or were reduced. CONCLUSIONS: Host accommodation was more important than graft accommodation. Accommodating grafts expressing protective genes were rejected with an elevation of both IgM and B-1 cells. In accommodated hosts, both IgM and B-1 cells decreased, suggesting that B-1 cells may be responsible for the production of antihamster antibodies. These results suggest that sufficient suppression of B-1 cells, resulting in decreased titers of antihamster antibodies, may play an important role in host accommodation.

Liver regeneration in donors and adult recipients after living donor liver transplantation.

In living donor liver transplantation, the safety of the donor operation is the highest priority. The introduction of the right lobe graft was late because of concerns about donor safety. We investigated donor liver regeneration by the types of resected segments as well as recipients to assess that appropriate regeneration was occurring. Eighty-seven donors were classified into 3 groups: left lateral section donors, left lobe donors, and right lobe donors. Forty-seven adult recipients were classified as either left or right lobe grafted recipients. Volumetry was retrospectively performed at 1 week, 1, 2, 3, and 6 months, and 1 year after the operation. In the right lobe donor group, the remnant liver volume was 45.4%, and it rapidly increased to 68.9% at 1 month and 89.8% at 6 months. At 6 months, the regeneration ratios were almost the same in all donor groups. The recipient liver volume increased rapidly until 2 months, exceeding the standard liver volume, and then gradually decreased to 90% of the standard liver volume. Livers of the right lobe donor group regenerated fastest in the donor groups, and the recipient liver regenerated faster than the donor liver. Analyzing liver regeneration many times with a large number of donors enabled us to understand the normal liver regeneration pattern. Although the donor livers did not reach their initial volume, the donors showed normal liver function at 1 year. The donors have returned to their normal daily activities. Donor hepatectomy, even right hepatectomy, can be safely performed with accurate preoperative volumetry and careful decision-making concerning graft-type selection.

Novel application of ProteinChip technology exploring acute rejection markers of rat small bowel transplantation.

BACKGROUND: Because no biomarker that reflects small bowel allograft rejection is available, we applied surface-enhanced laser desorption/ionization time-of-flight mass spectrometry (SELDI-TOF MS) to develop noninvasive markers required for routine diagnosis. METHODS: Heterotopic small bowel transplantation (SBT) was performed in rats, and they were divided into four experimental groups: sham-operated rats (sham), syngeneic transplants (syngeneic), allogeneic transplants (allogeneic), allogeneic transplants received FK506 (allo+FK). Plasma samples were analyzed with SELDI ProteinChip arrays to detect peaks that predominated in the allogeneic model. Possible biomarkers were identified in combination with SELDI retentate chromatography mass spectrometry (RCMS) and matrix-assisted laser desorption/ionization mass spectrometry (MALDI-MS). The identified protein was further analyzed by immunohistochemistry. RESULTS: An increase in the level of a 14.8-kDa protein, identified as lysozyme, was observed specifically in the plasma of the allogeneic group; the levels of this protein remained unchanged in the plasma of the other groups. On the other hand, the levels of a 10.1-kDa and a 13.0-kDa protein, identified as migration inhibitory factor-related proteins (MRP), MRP-8 and MRP-14, respectively, began to increase from an early stage of acute rejection. We also observed that lysozyme-positive macrophages had strongly infiltrated the lamina propria during acute rejection. CONCLUSIONS: We identified three plasma proteins-MRP-8, MRP-14, and lysozyme-that increased during small bowel allograft rejection. The identified proteins appeared to be markers for inflammation associated with allograft rejection. This proteomic approach will be useful for the identification of candidate biomarkers.

Fluorescence Visualization of the Enteric Nervous Network in a Chemically Induced Aganglionosis Model.

Gastrointestinal motility disorders, severe variants in particular, remain a therapeutic challenge in pediatric surgery. Absence of enteric ganglion cells that originate from neural crest cells is a major cause of dysmotility. However, the limitations of currently available animal models of dysmotility continue to impede the development of new therapeutics. Indeed, the short lifespan and/or poor penetrance of existing genetic models of dysmotility prohibit the functional evaluation of promising approaches, such as stem cell replacement strategy. Here, we induced an aganglionosis model using topical benzalkonium chloride in a P0-Cre/GFP transgenic mouse in which the neural crest lineage is labeled by green fluorescence. Pathological abnormalities and functional changes in the gastrointestinal tract were evaluated 2-8 weeks after chemical injury. Laparotomy combined with fluorescence microscopy allowed direct visualization of the enteric neural network in vivo. Immunohistochemical evaluation further confirmed the irreversible disappearance of ganglion cells, glial cells, and interstitial cell of Cajal. Remaining stool weight and bead expulsion time in particular supported the pathophysiological relevance of this chemically-induced model of aganglionosis. Interestingly, we show that chemical ablation of enteric ganglion cells is associated with a long lifespan. By combining genetic labeling of neural crest derivatives and chemical ablation of enteric ganglion cells, we developed a newly customized model of aganglionosis. Our results indicate that this aganglionosis model exhibits decreased gastrointestinal motility and shows sufficient survival for functional evaluation. This model may prove useful for the development of future therapies against motility disorders.

[Childhood rhabdomyosarcoma].

Overall five-year survival of Childhood rhabdomyosarcoma (RMS) is reported to be 70% in the Intergroup Rhabdomyosarcoma Study Group (IRSG), however, the figure in Japan is almost 15% lower than that of IRSG. Treatment regimen of RMS essentially depends on the histology of the tumor, site, preoperative staging and postoperative grouping that leads to the risk classification. VAC is a standard chemotherapeutic regimen for low and intermediate risk group. Clinical trial with CPT-11 or high dose chemotherapy are underway for high risk RMS.