Japanese guidelines for the management of intussusception in children, 2011.

BACKGROUND: The Japanese Society of Emergency Pediatrics has formulated evidence-based guidelines for the management of intussusception in children in order to diagnose intussusceptions promptly, to initiate appropriate treatment as early as possible, and to protect intussuscepted children from death. METHODS: Literature was collected systematically via the Internet using the key words "intussusception" and "children." The evidence level of each paper was rated in accordance with the levels of evidence of the Oxford Center for Evidence-based Medicine. The guidelines consisted of 50 clinical questions and the answers. Grades of recommendation were added to the procedures recommended on the basis of the strength of evidence levels. RESULTS: Three criteria of "diagnostic criteria,""severity assessment criteria," and "criteria for patient transfer" were proposed aiming at an early diagnosis, selection of appropriate treatment, and patient transfer for referral to a tertiary hospital in severe cases. Barium is no longer recommended for enema reduction (recommendation D) because the patient becomes severely ill once perforation occurs. Use of other contrast media, such as water-soluble iodinated contrast, normal saline, or air, is recommended under either fluoroscopic or sonographic guidance. Delayed repeat enema improves reduction success rate, and is recommended if the initial enema partially reduced the intussusception and if the patient condition is stable. CONCLUSIONS: The guidelines offer standards of management, but it is not necessarily the purpose of the guidelines to regulate clinical practices. One should judge each individual clinical situation in accordance with experiences, available devices, and the patient's condition.

Proton beam therapy for inoperable recurrence of bronchial high-grade mucoepidermoid carcinoma.

We report the case of a 17-year-old patient who received four courses of proton beam therapy for inoperable recurrent high-grade bronchial mucoepidermoid carcinoma of the chest wall and lymph nodes. The equivalent doses in conventional fractionation of 79.2-80.6 Gy were applied to the tumor from the first to third courses of proton beam therapy; the hemi-chest wall was also irradiated prophylactically in the third course. The irradiated tumor recurred marginally and liver metastasis developed, but tumor size within the irradiated field was suppressed. Proton beam therapy was also applied to the marginally recurrent tumor in the fourth course. The patient died of cancer about 5 years after the first course of proton beam therapy-about 9 years after the initial diagnosis and surgery. Repeated irradiation of the mediastinum and chest wall with photon radiotherapy is often limited by side-effects in the heart, esophagus and spinal cord. However, no severe late complications in critical organs were detected in this case. Only a Grade 2 skin reaction and lymphatic edema were observed. Therefore, high-dose proton beam therapy may be an option as a salvage therapy with less toxicity to normal tissues compared with photon radiotherapy and provide an alternative to repeated surgery.

Radionuclide imaging study of long-term pulmonary function after lobectomy in children with congenital cystic lung disease.

PURPOSE: We evaluated the long-term pulmonary function after lobectomy for congenital cystic lung disease, in both infants and children, using radionuclide imaging (RI). METHODS: We performed a retrospective review of 93 patients who underwent resection of cystic lung lesions between 1974 and 2001. The results of postoperative lung volume/perfusion scintigraphy at 1 (n = 64), 5 (n = 32), and 10 years (n = 18) after surgery (V1, 5, 10/Q1, 5, 10) and mean transit time (MTT-a marker for air-trapping) at 1, 5, and 10 years after surgery (MTT1, 5, 10) were compared with respect to age at operation, preoperative infection, underlying disease, and type of surgery. RESULTS: Patients who were younger than 1 year at the time of surgery showed a significantly lower MTT5 (1.09 +/- 0.08) and MTT10 (1.15 +/- 0.11) than patients who were older than 1 year at the time of surgery (MTT5, 1.49 +/- 0.67; MTT10, 1.54 +/- 0.33). The noninfected group had significantly higher Q10 and lower MTT10 values (P < .05) compared to the infected group. No significant differences were observed between patients with single lobe vs multiple lobe resection. CONCLUSIONS: The optimal age for surgery in patients with congenital cystic lung disease appears to be less than 1 year.

Modified slide tracheoplasty for congenital tracheal stenosis.

Slide tracheoplasty has become a standard procedure to treat long-segment congenital tracheal stenosis because it is a reasonable and simple technique. Slide tracheoplasty does not affect the carina during long-segment congenital tracheal stenosis management, and thus lesions of the carina, such as stenosis and tracheobronchomalacia can become important causes of extubation failure after surgery. In this manusript, we describe the effectiveness of our modified slide tracheoplasty, which includes reconstruction of the carina. We have performed this technique on three patients, all of whom were extubated without developing any respiratory symptoms.

Aortopexy for tracheomalacia with dextrocardia, pulmonary artery sling, and congenital tracheal stenosis.

We report a rare case of coexisting pulmonary artery sling, congenital tracheal stenosis, and dextrocardia caused by right lung hypoplasia. Successful treatment of severe postoperative tracheomalacia was achieved by aortopexy, aiming displacement of the aortic arch across orthogonally in front of the trachea due to dextrocardia. The aim of this surgery was different from the usual aortopexy for tracheomalacia, which lifts the tracheal wall with the aorta. The three-dimensional evaluation considering the patient's associated malformations led to a successful result.

A randomized controlled trial of an at-home preparation programme for Japanese preschool children: effects on children's and caregivers' anxiety associated with surgery.

OBJECTIVES: To determine whether the implementation of at-home psychological preparation programme for children and family prior to surgery can reduce anxiety for Japanese preschool children undergoing herniorrhaphy and their caregivers assessed as an appropriate outpatient care. METHODS: Patients were randomly assigned to either of two groups: the usual care group or the at-home preparation group. Both two groups viewed a patient-educational video for herniorrhaphy once as outpatients with other patients prior to hospitalization. The control group later underwent surgery without any further preparation. The experimental group watched the same educational video at home again with an auxiliary booklet prior to hospitalization. Children's anxiety was measured by the Wong-Baker FACES Rating Scale (FACES Rating Scale), while caregivers' anxiety was measured by the Spielberger's State Trait Anxiety Inventory (STAI). Both outcomes were measured repeatedly from pre-intervention to 1 month after surgery. RESULTS: Of the eligible 161 patients participating, 158 (98.1%) were randomly assigned to the control group (n = 81) and the experimental group (n = 77), and 144 (89.4%) completed the study. The experimental group gained more information and knowledge about surgery from parents and showed significantly lower scores than the controls for FACES and STAI. CONCLUSION: A specially designed at-home preparation programme as an outpatient care is effective to encourage parent-child verbal interaction concerning surgery and reduce both children and caregivers' anxiety associated with surgery.

Surgical management of congenital tracheal stenosis.

Congenital tracheal stenosis (CTS) is a rare but life-threatening disorder, particularly in neonates and infants. The stenotic lesions are commonly composed of complete rings of cartilage varying in length, location, and severity. A definitive diagnosis of CTS may be delayed because of the rarity of this disorder and thus its unfamiliarity among physicians, its variable onset time, its various clinical symptoms, and the diversity of associated clinical conditions due to the cardiovascular disorders that may accompany it. More than half of the patients who manifest clinical symptoms during early infancy show a long-segment stenosis. Long-segment CTS is problematic and challenging to manage. Over the last two decades several surgical techniques for long-segment CTS have been devised and developed, but no definitively advantageous surgical procedure has been established because of insufficient experience and the lack of large-scale studies. Although rib cartilage tracheoplasty and pericardial patch tracheoplasty have provided limited early to midterm success for infants with long-segment CTS, these procedures are associated with early and late complications, including granulation tissue formation, patch collapse, and restenosis necessitating reintervention. By contrast, slide tracheoplasty has given rise to better surgical outcomes. This procedure enables reconstruction of the stenotic trachea using native tracheal walls with preserved blood supply. The trachea is foreshortened by only one-half of the original stenosis, resulting in reduced tension on the anastomosis. Consequently, the technique has several advantages, including less formation of granulation tissue, satisfactory subsequent growth, and infrequent reoperation for restenosis. Slide tracheoplasty is currently recognized as the preferred technique for long-segment CTS.

Patterns of local recurrence after intraoperative radiotherapy for advanced neuroblastoma.

OBJECTIVE: The purpose of this study was to retrospectively evaluate local recurrence patterns after intraoperative radiation therapy (IORT) combined with total or subtotal resection and intensive chemotherapy for advanced neuroblastoma. METHODS: The outcomes of 27 patients (14 boys and 13 girls) with advanced-stage neuroblastoma who received IORT as part of multimodality therapy between November 1988 and December 2006 were reviewed in order to evaluate the impact of IORT. Of particular interest was the local recurrence patterns observed. RESULTS: Six patients relapsed in the abdominal area: three out of six relapsed adjacent to the radiation fields. Other three relapsed in the field of electron ports. Among them, one relapsed in paraspinal lymph nodes, which are behind the irradiated volume but out of the reach of the electron beam, while another relapsed in the lymph nodes of the mesocolon, which had been displaced outside the irradiation field at the time of IORT. The last case relapsed beside the vertebral column near the left ureter, which had been shielded by a lead plate. These three 'in-field' recurrences would have been irradiated if external opposite two-beam radiations had been performed, instead of electron beams. CONCLUSIONS: In spite of a complete tumor control in the treated volume, some 'marginal' recurrences were observed. Further investigation--for example, a combination of IORT and external-beam radiotherapy--should be considered to achieve higher local control and decrease complication rates.

Accumulation of gallium-67 within mature and immature teratoma in pediatric patients: investigation for the uptake mechanism.

OBJECTIVE: We encountered cases of mature and immature teratoma with positive uptake of (67)Ga. The objective of this study is to investigate the mechanism of (67)Ga accumulation within mature and immature teratomas by comparing the findings of gallium scan, computed tomography (CT), and autoradiography of surgical specimens with the pathological findings. METHODS: The subjects comprised 14 children who underwent surgical resection for intra-abdominal mature and immature teratomas, which were histologically proved to be of the mature and immature subtype. Their age ranged from 24 days to 14 years. The origins of the mature teratomas consisted of seven ovaries including one bilateral case, two retroperitoneal, and two sacrococcygeal regions. The origins of the immature teratomas were retroperitoneum in two cases, an ovary and a sacrococcygeal region. Complete surgical excision was feasible in all children. They underwent both gallium scan and CT prior to surgery. Single-photon emission computed tomography was added in some cases. For two gallium-positive cases, radiography and scintigraphy (autoradiography) of the resected specimen were performed. RESULTS: Of the 14 children, 5 (one with immature and four with mature subtype) showed positive (67)Ga uptake within tumors, which originated from the retroperitoneum in the 3 boys, and from the ovary in the 2 girls. All had typical CT findings of teratoma, including calcifications, fat components, cystic areas, and solid parts. (67)Ga accumulation in the four mature teratomas appeared discretely strong, and was considered to correspond with intralesional calcifications. However, in the remaining one immature teratoma, the gallium distribution was diffuse within the tumor. The comparison between radiography and autoradiography of the resected mature teratomas confirmed the correlation between the intralesional calcifications and areas of (67)Ga accumulation. CONCLUSIONS: A high-uptake ratio of (67)Ga in benign teratoma was indicated. A close correlation between gallium scan and CT helps to ascertain whether (67)Ga uptake results from malignant and/or immature elements, or mature tissue components.

A newly developed small-caliber videoduodenoscope for endoscopic retrograde cholangiopancreatography in children.

BACKGROUND: In neonates and younger children, the procedure of endoscopic retrograde cholangiopancreatography (ERCP) is now optimally performed using a small-caliber fiberoptic duodenoscope. However, there have been no reports on a specially designed videoduodenoscope for pediatric ERCP. GOAL: To evaluate performance and clinical usefulness of a newly developed small-caliber videoduodenoscope for ERCP in children. STUDY: We have developed a side-viewing videoduodenoscope with a 8.0-mm outside diameter of the tip and a 2.0-mm diameter instrument channel (Olympus PJF-240). In a total of 10 pediatric patients aged 11 months to 15 years (mean age, 6.1 years) who underwent ERCP using this videoduodenoscope, its feasibility was evaluated. Clinical diagnoses included pancreatitis (n = 4), choledochal cyst (n = 3), sclerosing cholangitis (n = 1), cholecystitis (n = 1), and choledocholithiasis (n = 1). In all patients, ERCP was done in the standard fashion under general anesthesia. RESULTS: ERCP was successful in 9 of 10 patients (90%). In the procedure, entering the descending duodenum was smooth and positioning the papilla on the endoscopic image was proper. In an 11-month-old infant, extraction of biliary stones by endoscopic balloon dilation was successfully done. Post-ERCP complications, including a significant increase of serum amylase levels, were not reported in any patients. CONCLUSION: This new videoduodenoscope has a potential for ERCP in all age groups of children.

The mean transit time and functional image in asialoglycoprotein receptor scintigraphy: a novel modality for evaluating the regional dynamic function of hepatocytes.

UNLABELLED: In this study, we attempted to evaluate the regional dynamic function of hepatocytes by introducing unique parameters in (99m)Tc-diethylenetriaminepentaacetic acid-galactosyl-human serum albumin (99m)Tc-GSA) scintigraphy. (99m)Tc-GSA scintigraphy provides valuable information for the receptor population density. However, the conventional indices are the results of the analyses of 2 fixed points and, as a result, it is not possible to accurately estimate the regional dynamic function. METHODS: We performed (99m)Tc-GSA scintigraphy 100 times on a total of 54 pediatric patients. The average age at examination was 7.4 +/- 5.8 y. Ninety-one of the 100 scintigraphy cases were available for this study. We converted the time-activity curve for the liver of (99m)Tc-GSA to a horizontal mirror image curve, and, on the basis of the height-over-area method, calculated the mean transit time (MTT) in each pixel and depicted the functional image as unique parameters, which were thus compared with the conventional indices. For these parameters, we used the time-activity curve for only the liver. RESULTS: The whole liver MTT showed a significant correlation with both the clearance (y = 590.3x + 10.3; r = 0.51; P < 0.0001) and the receptor (y = -1,836.2x + 2,038.8; r = -0.66; P < 0.0001) indices. On the basis of the MTT in each pixel, we could depict the functional image of the liver. In actual clinical situations, the functional image was quite useful for making a visual evaluation of the dynamic distribution of (99m)Tc-GSA. The functional image indicated that, even at an extremely early stage of biliary atresia, the hepatic functional reserve might be exacerbated earlier in the right lobe than in the left lobe. CONCLUSION: The MTT and the functional image enable us to elucidate the regional dynamic function of hepatocytes both quantitatively and visually. In addition, this diagnostic modality can be used at virtually all medical institutions using a modified analytic program already in public use.