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Purpose: Radical surgery for congenital tracheal stenosis (CTS) is technically demanding. CTS combined with tracheal bronchus (TB) and pulmonary artery (PA) sling is a particularly challenging condition. We herein report our successfully modified surgical techniques for CTS combined with TB and PA sling. Methods: Nine patients treated at our institution from July 2010 to December 2020 for CTS with TB and PA sling were enrolled. The patients’ characteristics, operative results, and clinical outcomes were reviewed and analyzed retrospectively. Results: The mean age at the operation and body weight were 8.0±4.4 months old and 6.5±0.8 kg, respectively. The mean tracheal diameter and length of stenotic lesion were 3.2±1.0 mm (mean stenosis rate: 46.2%) and 25.4±4.9 mm, respectively. All cases were complicated with PA sling at bifurcation stenosis with tracheobronchomalacia. All patients underwent modified posterior-anterior slide tracheoplasty with an inverted Y-shaped incision at the bifurcation and repositioning of the PA. The mean postoperative intubation period was 25.0±32.1 days. There were no major intraoperative or postoperative complications, including hypoxic ischemic encephalopathy. The mean hospital stay was 92.2±73.4 days. All patients were discharged home without tracheostomy or oxygen support.Conclusion: Our slide tracheoplasty technique for CTS with TB and PA sling achieved excellent outcomes.

The optimal management method for pediatric pancreatic trauma is controversial. Moreover, the efficacy of stent placement via endoscopic retrograde pancreatography (ERP) remains poorly documented. The present, retrospective review of pediatric patients with pancreatic trauma was conducted from 2010 to 2020 at a single institution. Ten, male children with the median age of 9.5 years (range 4–14 years) with a grade I (n = 2), II (n = 4) or III (n = 4) pancreatic injury were identified. Of six of these patients in whom ERP was performed, four had a pancreatic duct injury (PDI). Pancreatic stent placement was performed in all the patients with ERP at a site proximal to the injury in four patients and across the injury in two patients. A pseudocyst or pancreatic fluid collection was detected in five patients, of these, two with a grade II injury were managed successfully with conservative therapy while three with PDI required surgery. In the four patients with PDI, only one in whom the stent was placed across the PDI was able to avoid surgery. Therapeutic ERP might be effective even if a patient has a PDI, therefore, early ERP should be considered as a treatment option.

Minimally invasive surgery (MIS) is appropriate for the treatment of some neuroblastomas (NBs); however, the indications and technical issues are unclear. This study aimed to clarify the current status of MIS for mediastinal NB in Japan. Preliminary questionnaires requesting the numbers of neuroblastoma cases in which MIS was performed from 2004 to 2016 were sent to 159 Japanese institutes of pediatric surgery. Secondary questionnaires were sent to institutions with MIS cases to collect detailed data. One hundred thirty-four (84.2%) institutions returned the preliminary questionnaire and 83 institutions (52.2%) reported a total of 1496 operative cases. MIS was performed for 175 (11.6%) cases. Among the 175 cases, completed forms of 140 patients were returned and 40 (male, n = 28; female, n = 12) cases had mediastinal NB. Fourteen patients received thoracoscopic biopsy, none were converted to thoracotomy. Twenty-eight patients received MIS for radical resection, none were converted to thoracotomy. Perioperative complications (Horner’s syndrome) were recognized after radical resection in one (2.5%) case. MIS was performed in a limited number of mediastinal NB cases. A thoracoscopic approach would be feasible for mediastinal NB.

Purpose: The slide tracheoplasty (STP) is the standard treatment for severe congenital tracheal stenosis (CTS). Understanding the features of the tracheal stenosis in each case and choosing an appropriate incision design are very important for successfully executing the procedure. The present study aimed to evaluate the advantages of three-dimensional (3D) printed models of the trachea for improving CTS. Methods: Three-D tracheal models were created using computed tomography (CT) data from ten patients undergoing STP for CTS. Simulated surgery was performed using the hollow models after reinforcing with them with a coating of gum spray. Clinical outcomes, including patient survival, postoperative surgical interventions, and time required for STP, were compared with the corresponding values in the last ten patients before the introduction of 3D model simulations. Results: All ten patients for whom simulated surgery using a 3D tracheal model were conducted achieved good airway patency after their STP. The surgeons reported feeling that the 3D model simulations were highly effective although there was no significant difference in the clinical outcomes of the groups with or without simulated STP. The models were useful not only for surgical planning but also for sharing important information among the multidisciplinary team and the patients’ family. Conclusion: Our experience using 3D tracheal models demonstrated several features enabling improvement in the surgical treatment of CTS.

The application of slide tracheoplasty (STP) in the treatment of congenital tracheal stenosis (CTS) has improved patient outcomes over the past few decades. We reviewed our experiences with the procedure, elucidated risk factors, and discussed important aspects of perioperative management to improve outcomes.Patients with CTS undergoing STP between July 1998 and December 2020 were enrolled. Patient characteristics, perioperative condition, management, operative details, and outcomes, including mortality and postoperative intervention, were collected from medical records.Eighty patients underwent STP. Sixty-five patients (81.3%) had an associated cardiovascular anomaly. Thirteen patients (16.3%) had unilateral lung agenesis or hypoplasia. Preoperative mechanical ventilation was necessary in 54 (67.5%) patients, and extracorporeal membrane oxygenation (ECMO) was required in eight patients (10.0%). An endotracheal tube was placed before the stenotic entrance to avoid granulation. During STP, the trachea was dissected as little as possible to maintain the blood supply. The one-year survival rate was 88.8% (nine patients died). One patient (1.3%) required postoperative balloon dilation, and none required stenting or granulation removal. Of the survivors, 62 (92.5%) achieved successful extubation without tracheostomy. Multivariable analysis revealed complex cardiovascular anomaly (P = 0.05) and preoperative ECMO (P = 0.019) to be adverse predictors of survival.Although STP can be performed successfully in CTS patients, surgeons and families should be aware of factors that may lead to a more difficult postoperative course or increase the mortality. Meticulous, perioperative positioning of the endotracheal tube and preserving the tracheal blood flow can minimize the need for postoperative intervention.

Background Our group previously proved that the human enteric nervous system can be visualized with confocal laser endomicroscopy after topical application of cresyl violet using surgically resected intestine specimens. The present report documents the first in vivo visualization of the human enteric nervous system with confocal laser endomicroscopy using local cresyl violet staining. The aim of this study was to evaluate the technical feasibility and clinical efficiency of confocal laser endomicroscopy in patients with Hirschsprung’s disease and allied disorders in vivo. Methods Confocal laser endomicroscopy was performed in vivo in two patients to confirm the presence of the enteric nervous system during surgery in patients with Hirschsprung’s disease and allied disorders. Cresyl violet was gently injected from the serosal side into the muscular layer of the intestine, and scanning was performed within 30 min. Then, the scanned intestines were resected, and the visualized area of the specimens was pathologically evaluated. Results The ganglion cell nuclei and the enteric nervous system network were clearly visualized intraoperatively in both cases. The morphological findings were similar to the pathological findings of the enteric nervous system in both cases although the period of visibility was brief. Conclusion This study demonstrated the first, real-time observation of the enteric nervous system in humans using confocal laser endomicroscopy and suggest the potential to identify the enteric nervous system intra-operatively during surgery for Hirschsprung’s disease and allied disorders.

Background: Congenital tracheal stenosis (CTS) is a rare and life-threatening disease in children. Although pulmonary artery sling (PA sling) complicated by CTS sometimes occurs, there are few reports detailing the management of CTS with PA sling. The purpose of this retrospective study was to determine the appropriate indications for surgical intervention for CTS complicated by PA sling. Methods: We evaluated 42 patients (19 males and 23 females) with the median age of 9.9±3.3 months (range, 5–34 months) with CTS complicated by PA sling who were treated at our hospital between 2005 and 2018. Twenty-eight patients received both a slide tracheoplasty and PA re-implantation, and 14 patients were managed conservatively for CTS. Among the latter, nine patients received PA re-implantation only, and five were managed conservatively without any surgery. We determined the surgical indications by retrospectively comparing the DLR value [tracheal diameter (mm)/stenotic length ratio], history of ventilator respiration, mortality rate, and post-operative course of patients at a single institution. Results: The cut-off value for the DLR was determined to be 5.9 (sensitivity: 0.929, specificity: 0.714) by using the ROC curve (AUC 0.89, P

Purpose Lingual thyroglossal duct cysts (L-TGDCs) are rare and sometimes lethal owing to their association with asphyxia. We aimed to analyze our single institutional experience with L-TGDCs. Methods Twelve L-TGDC cases treated at our institution between January 2010 and December 2017 were investigated. Results The male/female ratio was 6/6. The age at the diagnosis was 2 ± 1.4 months (7 days to 6 months), and 3 patients were diagnosed in the neonatal period. The patients presented with stridor (n = 12; 100%), growth retardation (n = 5; 42%), apnea (n = 3; 25%), and vomiting (n = 1; 8.3%). Lateral X-rays were obtained in 8 cases (66.7%); a lingual mass was suspected in 7 (87.5%). Transoral marsupialization of the cyst was performed under direct vision in all cases. All cases were nasally and orally intubated using a laryngoscope, bronchoscope, or airway scope. The mean operative time was 18 ± 2.9 min. The mean cyst size was 10.5 ± 1.8 mm. No recurrence was observed during the follow-up period (37.5 ± 18 months). Conclusion L-TGDC requires a precise diagnosis and rapid intervention because of the risk of asphyxia resulting in sudden death. Transoral marsupialization under direct vision is an effective and secure approach. L-TGDC should be considered when patients younger than six months of age present with respiratory distress. Type of study Retrospective Study. Level of evidence Level IV.

Background: In our institution, we avoid emergency nighttime appendectomies, instead performing the surgery during daylight hours the following day. We examined whether emergency or early appendectomies affect the outcome of patient morbidity. Materials and Methods: Medical records of children treated for appendicitis between 2010 and 2012 were retrospectively reviewed. Outcomes were compared between Group 1, defined as those patients who presented to the hospital during the day and underwent appendectomy on the same day and Group 2, defined as those patients who presented to the hospital at night and underwent appendectomy the next day. Incidences of perforation at surgery, operative time, complications and length of stay were analysed. Cases with perforation were also analysed to determine if the perforations could have been identified preoperatively. Results: A total of 74 patients met the study criteria, including 41 and 33 in Groups 1 and 2, respectively. There were no significant differences in the incidence of perforation at surgery, operative time, complications and length of stay. A total of nine cases of perforation were identified during surgery, and there were no significant differences in the pre-operative characteristics between perforated and non-perforated cases. Conclusions: The results of this study indicate that early appendectomy is safe and did not increase patient morbidity. We, therefore, recommend performing appendectomies in the daytime.

Background and Aim In preceding studies, we identified that the myenteric plexus (MP) could be visualized with confocal laser endomicroscopy (CLE) by applying neural fluorescent probes lacking clinical safety profiling data from the submucosal side. In this study, we evaluated the technical feasibility of MP visualization using probe-based CLE (pCLE) from the serosal side with cresyl violet (CV), which has been used clinically for chromoendoscopy. Methods The dye affinity of CV for MP was first explored in an in vivo transgenic mouse model using neural crest derivatives labeled with green fluorescent protein. We also tested the feasibility of CV-assisted visualization of MP in human surgical specimens, wherein the tissue dying and pCLE observation were performed from the serosal side. In the human study, rate of MP visualization by pCLE was evaluated as the primary outcome. We also evaluated the sensitivity and specificity of MP visualization by pCLE, using pathological presence/absence of MP as the gold standard. Results We confirmed the dye affinity of CV to MP in all tested models. The MP appeared as brightly stained ladder-like structures with pCLE, and in the human study, MP was visualized in 12/14 (85.7%) samples, with 92.3% sensitivity and 100% specificity. In positive cases showing the ladder-like structure of MP by pCLE, the mean maximum and minimum widths of nerve strands were 54.3 (± 23.6) and 19.7 (± 6.0) μm, respectively. A ganglion was detected by pCLE in 10 cases (10/12, 83.3%). Conclusions This study demonstrated the technical feasibility of visualizing the MP in real time by CV-assisted pCLE (UMIN-CTR number, UMIN000015056).

Background Hirschsprung's disease is a congenital abnormality of the enteric nervous system (ENS) presenting severe constipation soon after birth due to the lack of ganglion cells in the distal gut. Surgery for Hirschsprung's disease requires an intraoperative histopathological diagnosis to assess the extent of aganglionosis. Confocal laser endomicroscopy (CLE) is a novel endoscopic technique allowing real-time, in vivo analysis of cellular details during ongoing endoscopy. In this study, we evaluated the possibility of a new application of CLE to provide real-time observations of the ENS in patients with Hirschsprung's disease. In this preclinical feasibility study, we assessed the visualization of the ENS by CLE using surgically resected intestines. Methods The subjects were nine patients who underwent pull-through surgery for Hirschsprung's disease between September 2014 and March 2016. The colon specimens were stained with 0.1% cresyl violet and evaluated using CLE. We compared the CLE findings with those of the histopathological examination. Key results The ENS was clearly visualized as a ladder-like structure in the ganglionic segment but was not observed in the aganglionic segment. Of the 69 samples, corresponding positive and negative results for both CLE and the histopathology were obtained in 61 (88%). In addition, CLE was able to visualize unique, wavy structures comprising thick nerve bundles characteristic of the aganglionic/transition zone in Hirschsprung's disease. Conclusions and inferences As a novel tool for visualizing the human ENS, CLE has the potential to revolutionize how pediatric surgeons identify the level of ganglionosis during surgery for Hirschsprung's disease and may be a superior alternative to intraoperative histopathological diagnosis.

Objectives To identify the imaging features peculiar to congenital tracheal stenosis (CTS) complicated with left pulmonary artery sling (LPAS) with the aim of presenting a hypothesis of tracheal stenosis embryology in LPAS. Methods We retrospectively reviewed CTS patients (with complete cartilaginous rings) admitted between April 2010 and July 2018. All the patients were classified into the LPAS or non-LPAS group, and their clinical characteristics and qualitative variables on computed tomography (CT) imaging were compared. Results Of the 72 patients enrolled, 61 had bilateral lungs. Among the bilateral lung patients, 26 (43%) had LPAS. The tracheal bifurcation was significantly deeper, the stenotic region was longer, and the bronchial angle (especially in the right) was wider, in the LPAS group. The cut-off values for the thoracic vertebral level at the tracheal bifurcation (>4.8), subcarinal angle (>118.1), and right bronchial angle (>61.9) were useful for diagnosing suspected cases of LPAS. In the time-dependent course, LPAS complicated with a congenital heart defect was a statistically significant risk factor of respiratory symptoms (hazard ratio, 3.01; 95% confidence interval, 1.23-7.37; P = .02). Conclusions The CT findings described here should immediately raise suspicion of LPAS on chest X-ray and also suggest tracheal "squeezing and milking" by the surrounding vessels in the embryo. Patients with LPAS complicated with a heart defect should be followed carefully to determine the optimal timing of intervention.

The pathological feature of Hirschsprung’s disease (HSCR) consists in the continuous absence of ganglion cells from the intestine toward the caudocranial direction with most of the aganglionic segment occurring in the hindgut area and no abnormality being present in the pelvic nervous system. Several hypotheses of the cause of HSCR exist. Huther et al. speculated that the lesion was due to abnormalities in the lumbosacral nerves where the ganglion cells in the hindgut originate. The recent studies for ENS on chick, rodent, and human have shown that enteric ganglion cells are derived from neural crest. The majority of neural crest that colonize in gut originate from vagal neural crest adjacent to somite first to 7th. Another smaller but significant contribution that is restricted to the post-umbilical enteric ganglion cells has considered that they come from a second caudal region of the neuraxis, that is, the sacral neural crest. Unfortunately, hypothesis of dual origin of main enteric neurons in the gut has been negated by studies using chick, rodent, and human embryos. Also, the hypothesis that HSCR is caused by an abnormal sacral nerve cannot account for the type of HSCR presenting a long, aganglionic segment. Currently the sacral nerve pathway theory is not considered to be an adequate explanation of the main cause of HSCR.

We herein demonstrate a case of a gastric-type duplication cyst of the pancreas with a bifid tail in a 1-year-old girl, who presented as relapsing pancreatitis with pseudocyst formation. We reviewed the literature concerning pancreatic enteric duplication cysts with and without pancreatitis to search for causes of pancreatitis. The following new information was obtained: 1) The presence of a communication between the pancreatic duct system and enteric duplication cysts is the major cause, but it does not always lead to pancreatitis. 2) Enteric duplication cysts are occasionally connected to abnormal pancreatic lobes or ducts. We suggest that a duplication cyst associated with bifid pancreas is in the same category as the association of enteric cyst formation and pancreatic lobe maldevelopment. Since an aberrant lobe associated with an enteric duplication cyst often evokes pancreatitis, its surgical resection should be considered.

In preceding studies, we identified that the myenteric plexus (MP) could be visualized with confocal laser endomicroscopy (CLE) by applying neural fluorescent probes lacking clinical safety profiling data from the submucosal side. In this study, we evaluated the technical feasibility of MP visualization using probe-based CLE (pCLE) from the serosal side with cresyl violet (CV), which has been used clinically for chromoendoscopy.The dye affinity of CV for MP was first explored in an in vivo transgenic mouse model using neural crest derivatives labeled with green fluorescent protein. We also tested the feasibility of CV-assisted visualization of MP in human surgical specimens, wherein the tissue dying and pCLE observation were performed from the serosal side. In the human study, rate of MP visualization by pCLE was evaluated as the primary outcome. We also evaluated the sensitivity and specificity of MP visualization by pCLE, using pathological presence/absence of MP as the gold standard.We confirmed the dye affinity of CV to MP in all tested models. The MP appeared as brightly stained ladder-like structures with pCLE, and in the human study, MP was visualized in 12/14 (85.7%) samples, with 92.3% sensitivity and 100% specificity. In positive cases showing the ladder-like structure of MP by pCLE, the mean maximum and minimum widths of nerve strands were 54.3 (± 23.6) and 19.7 (± 6.0) μm, respectively. A ganglion was detected by pCLE in 10 cases (10/12, 83.3%).This study demonstrated the technical feasibility of visualizing the MP in real time by CV-assisted pCLE (UMIN-CTR number, UMIN000015056).

The current study aimed to assess the perinatal risk and clinical features of congenital cystic lung diseases (CCLD). Of the 874 CCLD patients identified in a nationwide survey, 428 patients born between 1992 and 2012 and treated at 10 high-volume centers, were retrospectively reviewed. Fetal hydrops was visualized using MRI in 9.2 % of the patients. Prenatal interventions were described for 221 of the 428 patients, including the maternal administration of steroid and pleuro-amniotic shunting. Postnatally, a right-to-left shunt flow through a persistent ductus arteriosus was observed in 7.8 % of the patients. The fetal lung lesion volume ratio (LVR) was significantly higher among these symptomatic patients (2.04 ± 1.71 vs. 0.98 ± 0.50, P

Perivascular epithelioid cell tumour (PEComa) encompasses a group of mesenchymal tumours composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. A subset of PEComa that typically arises from the falciform ligament and/or ligamentum teres is termed clear cell myomelanocytic tumour of the falciform ligament/ligamentum teres. To date, its imaging findings have not been described. Here, we report the first radiological description of a pathologically confirmed tumour. The patient was a 5-year-old girl with a palpable abdominal mass. US, CT, MR and FDG-PET revealed a midline, well-defined, solid anterior abdominal wall tumour below the rectus abdominis and contiguous with the umbilicus that was hypervascular and FDG avid. Awareness of these imaging findings facilitates the diagnosis of this distinctive tumour.

Introduction We have succeeded in classifying the severity of appendicitis by evaluating the structure of the submucosal layer and blood flow in the appendix wall with ultrasonography (US). Here, we propose a new preoperative classification system to guide treatment strategy. Materials and Methods Between February 2005 and December 2011, a total of 440 acute appendicitis cases were diagnosed, and 407 met inclusion criteria for this study. US and Doppler findings were used to classify cases as Grade I, IIa, IIb, III, or appendiceal mass. Grades I and IIa were treated conservatively without antibiotics. Appendectomy for Grades IIb and III and interval appendectomy for appendiceal mass were performed. Results Of 407 cases, 38 were Grade I, 132 were Grade IIa, 130 were Grade IIb, 57 were Grade III, and 50 were appendiceal mass. Improvement occurred without antibiotics in 37 (97.3%) Grade I and 129 (97.7%) Grade IIa cases. Recurrence occurred postdischarge in 9 (23.6%) Grade I and 35 (26.5%) Grade IIa cases. All Grades IIb and III patients had gangrenous or phlegmonous appendicitis. There were no negative appendectomies. Conclusion Even if US findings reveal swelling or an irregular submucosal layer, when power Doppler identifies abundant blood flow, inflammation may improve spontaneously. Decreased appendiceal wall blood flow indicates irreversible necrotic change. Doppler and B-mode US used together improve acute appendicitis treatment.

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.

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.

The current study aimed to assess the perinatal risk of congenital cystic lung diseases (CCLD) and to establish a suitable surgical strategy in consideration of postoperative lung growth and problems during adulthood.Among 874 CCLD patients identified in a nationwide survey, 428 patients were born between 1992 and 2012 and treated at 10 high-volume centers were retrospectively reviewed with statistical analysis.In the 194 patients who were prenatally diagnosed to have CCLD, 16.7% presented with fetal hydrops as observed using ultrasonography. The Apgar score (5 minutes) was lower than 5 in 5.4%. As of postnatal day 30, 14.0% of the neonatal patients required respiratory support, and 3.3% (8/243) had died because of pulmonary hypoplasia. Among those who were asymptomatic immediately after birth, 33.6% of the patients developed the respiratory infectious symptoms during their first year of life, and 22.1% did so between the age of 1 and 2 years. The postoperative percent vital capacity among the prenatally diagnosed patients was significantly higher than that among the postnatally diagnosed patients (98.3 ± 11.9 vs. 81.7 ± 9.7, p0.0222). Late complications included thoracic deformity in 30 patients and persistent lung cyst in 4 patients, whereas malignancy was not observed in the present series.Approximately 10 to 15% of prenatally diagnosed CCLD patients may carry a high risk of perinatal respiratory distress. Early operation before developing episodes of lung infection, seem to be associated with a better development of the reserved lung during later life. The incidence of carcinogenesis among patients with CCLD may be extremely low.

Background/Purpose: The incidence of gastroschisis has increased over the past 3 decades in a number of countries. To elucidate the Japanese status of anterior abdominal wall defects, the Japanese Society of Pediatric Surgeons conducted a national survey in Japan. Methods: Information was obtained by sending out a questionnaire to 192 University Hospitals, Children's hospitals, and general hospitals that each had more than 200 beds. The characteristics of the patients including the birth date, birth weight, gestations, rate of associated anomalies, rate of antenatal diagnosis and prognosis, maternal age, gravidity, history of smoking, and drug use were analyzed. Results: The authors obtained answers from 149 institutions, including 1,785 cases of omphalocele and 970 cases of gastroschisis, which were treated between 1975 to 1997. There was a significant increase in the incidence of gastroschisis, from 0.131 in 1975 to 1980, 0.269 in 1981 to 1985, 0.337 in 1986 to 1990, 0.461 in 1991 to 1995 to 0.467 per 10,000 births in 1996 to 1997. The incidence of omphalocele was 0.322 in 1975 to 1980, 0.567 in 1981 to 1985, 0.657 in 1986 to 1990, 0.741 in 1991 to 1995 to 0.626 per 10,000 births in 1996 to 1997, respectively. In the omphalocele group, 43.1% of the mothers were between 25 to 29 years of age, whereas in the gastroschisis group 42.6% of the mothers were 20 to 24 years of age. In the gastroschisis group, the number of primipara mothers was larger than that of multipara mothers. In the omphalocele group, approximately 10% of the mothers smoked during each period, whereas in the gastroschisis group, the percentage of smoking mothers increased chronologically from 12.9% in 1981 to 1985, 18.7% in 1986 to 1990, 23.5% in 1991 to 1995 and 29.3% in 1996 to 1997. A history of drug use by the mother was approximately 10% for both the omphalocele and gastroschisis groups. In the omphalocele group, 55.9% had associated anomalies against 21.8% in the gastroschisis group. Approximately 10% in the omphalocele group and less than 3% in the gastroschisis group showed chromosomal abnormalities. From 1986, a significant number of cases detected antenatally has been observed. Conclusions: There have been substantial changes in the incidence of anterior abdominal wall defects, particularly regarding gastroschisis in Japan. The reasons for such changes are most likely multifactorial, further epidemiological monitoring is thus called for. J Pediatr Surg 35:66-72. Copyright © 2000 by W.B. Saunders Company.

A 4-year-old boy presented with pneumonia. Computed tomography demonstrated a multicystic mass at the posteromedial bilateral lower lobe segments, which were connected by a bronchus behind the heart. Enhanced computed tomography revealed that an anomalous artery arose from the left gastric artery and inserted into the left sequestrated lung and branched to the right one. The diagnosis of bilateral intralobar sequestration (ILS) with a bridging isthmus was made. After removal of the bilateral ILS, radiologic and pathologic approaches were undertaken to reconstruct the vascular and bronchial architectures. The following observations were made: (1) histologically, the region near the anomalous artery insertion site contained bronchial structures, which looked like an ectopic pulmonary hilus. This bronchial structure was continuously observed in the isthmus and its opening of the right sequestrated lung; (2) radiologically, the shape and course of the bronchi within the ILS indicated a distinct bronchial origin that arose from the pulmonary hilus-like structure, and the anomalous artery that ran along with those bronchi, resembled a pulmonary artery. These features suggested that this bilateral ILS might have originated from an accessory lung tissue.

Intramural bronchogenic cysts are extremely rare. We describe the case of an intramural bronchogenic cyst in a 2 year old boy who underwent tracheal resection and end-to-end anastomosis.

Slide tracheoplasty is a standard treatment for long-segment congenital tracheal stenosis (LCTS). However, in severe cases of LCTS, aggressive divisions of inferior constrictor muscle from the thyroid cartilage and extensive circumferential dissection of the upper tracheal segment are often necessary to mobilize the upper tracheal segment enough to make an anastomosis, but they increase the risks of anastomotic dehiscence, recurrent nerve injury, and impaired deglutition. Alternatively, laryngeal release provides safe mobilization of the upper tracheal segment, minimizing dissection of the inferior constrictor muscle and preserving the lateral tissue pedicle without circumferential dissection. We successfully performed laryngeal release with slide tracheoplasty on six patients with severe LCTS, and report our findings.

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.

Throughout the estrous cycle, Muillerian inhibiting substance (MIS) mRNA signals, as detected by in situ hybridization, were found to be intense in granulosa cells of growing preantral and small antral follicles, especially in the layer closest to the oocyte. Neither primordial follicles, typical atretic follicles, nor CL expressed detectable signals. MIS mRNA signals seen in the cumulus cells of preovulatory follicles at 1000 and 1600 h of proestrus sharply declined at 2400 h, just before ovulation. MIS mRNA signals were intense and uniform in all newly recruited growing antral and Graafian follicles (> 350 pm) at 1000 h of estrus but became heterogeneous in their expression of MIS on metestrus and diestrus and notably absent in atretic follicles; these findings suggest that MIS expression is correlated with recruited follicle selection and suppression with selection for atresia. The fact that all the MIS-positive growing follicles contain oocytes arrested in meiosis, coupled with previous experimental evidence that MIS inhibits germinal vesicle breakdown, suggests that MIS also might act as a regulator of oocyte maturation.

Although the etiology of Hirschsprung's disease and neuronal intestinal dysplasia remains obscure, both have histological abnormalities involving ganglion cells and neuronal elements. Searching for a common pathway that may inhibit normal maturation of neurogenic precursors, we examined the possible role of an immune mechanism in the maldevelopment of the enteric neural network. Six patients with Hirschsprung's disease were studied by comparing biopsy specimens from diseased colon with ones taken from proximal ganglionic colon in the same patients. These were similarly compared with colonic biopsy specimens from patients studied with chronic constipation or bowel removed at the time of operation for other disorders. Biopsies were taken from four other patients with neuronal intestinal dysplasia. Each was examined by hematoxylin & eosin staining, acetylcholinesterase histochemistry, and immuno-histochemistry of major histocompatibility complex (MHC) class I and class II antigens. All rectal samples from Hirschsprung's disease patients exhibited elevated acetylcholinesterase histochemistry and absent ganglia to confirm the diagnosis. These findings were correlated with marked elevation of class II MHC in the aganglionic area, whereas the proximal normal ganglionic segments showed no elevation. Rectal biopsy specimens from patients with chronic constipation exhibited no such elevation. A similar elevation of class II MHC was detected in the mucosa and submucosa of all four patients with the rare neuronal intestinal dysplasia disorder whose diagnosis was confirmed by giant ganglia in Auerbach's plexuses, aberrant Meissner's ganglia in the lamina propria mucosa, and giant neurofibrils in the mucosa and submucosa. The correlation of elevated class II MHC in these two neuronal dysfunction disorders may indicate an underlying autoimmune mechanism as is seen in thyroiditis and insulin dependent diabetes mellitus. These observations confirm those found in a small number of Hirschsprung's disease patients reported earlier, and extend the observation to other neuronal dysfunction disorders. These findings provoke a reexamination of the etiology of Hirschsprung's disease and may predict alternate therapies.

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.

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 twobeam 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.

We evaluated the long-term pulmonary function after lobectomy for congenital cystic lung disease, in both infants and children, using radionuclide imaging (RI).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.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.The optimal age for surgery in patients with congenital cystic lung disease appears to be less than 1 year.

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.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.The whole liver MTT showed a significant correlation with both the clearance (y = 590.3x + 10.3; r = 0.51; P0.0001) and the receptor (y = -1,836.2x + 2,038.8; r = -0.66; P0.0001) 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.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.

In males, Mullerian inhibiting substance (MIS) mRNA was first detected on the medial aspect of the urogenital ridge early on the morning of day 13 of gestation before testicular differentiation was evident, and localized to the more obvious Sertoli cells later on embryonic day 13. MIS transcripts remained at maximal levels between 14.5 and 17.5 days gestation, while the Mullerian duct involutes, and remained high until birth. MIS gene expression decreased progressively after birth and, as germ cell meiosis increased, became barely detectable in the Sertoli cells of the seminiferous tubules. In female rats, MIS mRNA was first detected in the single layer of cuboidal granulosa cells surrounding larger primary follicles 3 days after birth, coincident with the initiation of follicular growth. As follicular growth progressed, MIS mRNA expression was high in preantral and small antral follicles, especially in those granulosa cells closest to the oocyte. MIS mRNA expression decreased gradually in larger antral follicles, remaining prominent only in the cumulus cells and the dividing population of granulosa cells closest to the lumen. MIS gene expression was absent in follicles with features of atresia and in the larger antral follicles. The expression of MIS mRNA in actively dividing Sertoli and granulosa cells correlates with the stages of germ cell division. These findings are suggestive of a role for MIS in the control of germ cell maturation.