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IntroductionWe aimed to quantify the DNA of maternal chimeric (MC) cells in the peripheral blood of the BA patients and investigated the impact on the outcome.MethodsPatients with progressive jaundice because of no bile flow, which necessitated liver transplantation, or who showed inadequate bile flow with or without episodes of cholangitis and progressive hepatic fibrosis and portal hypertension were classified into the poor group. Those with adequate bile flow with completely normal liver function tests beyond 2 years were classified into the good group. The qPCR were separately carried out in buffy coat samples and plasma samples, targeting the non-inherited maternal HLA alleles in the DNA samples.ResultsMC-DNA was present in the buffy coat (10–328 gEq per 106 host cells) in seven patients. There was no MC-DNA in the remaining five patients. MC-DNA (214–15,331 gEq per 106 host cells) was observed in the plasma of five patients. The quantity of MC-DNA in the buffy coat showed a significant difference between the two prognostic groups (p = 0.018), whereas there was no significant difference in the quantity of MC-DNA in plasma (p = 0.205). MC-DNA in the buffy coat was significantly associated with the outcome (p = 0.028), whereas MC-DNA in the plasma did not influence the outcome (p = 0.56).ConclusionsPoor outcomes in BA were correlated with circulating maternal chimeric lymphocytes.

Undifferentiated embryonal sarcoma of the liver (UESL) is a highly malignant neoplasm occurring predominantly in children. Complete resection is the only curative treatment. We encountered an 11-year-old boy with UESL arising in the right lobe of the liver extending to the pulmonary valve. He was treated with IREC (irinotecan, etoposide, and carboplatin) neoadjuvant chemotherapy, concurrent extended radical right hepatectomy, and intracardiac tumorectomy under cardiopulmonary bypass, and adjuvant IREC chemotherapy. He remains in remission without complications 48 months after diagnosis. Multidisciplinary treatment, particularly the combination of radical surgery and effective neoadjuvant chemotherapy, may contribute to UESL patients' survival.

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.

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.

Laparoscopic pyloromyotomy (LP) is a well-established approach for treating hypertrophic pyloric stenosis. Although grasping the pylorus with forceps is important in LP, it requires a high level of surgical skill. To make this approach easier, in 2008 we introduced the method of Dozier and Kim, in which a vascular clamp (VC) is used for grasping the pylorus (LP with VC). Here, we retrospectively compared results between traditional LP (TLP) and LP with VC.Forty-three patients underwent LP with VC, whereas 194 patients underwent TLP. Two ports in the umbilicus and the left flank are common in both procedures. Instead of a right flank port, however, in LP with VC, we directly insert a VC to grasp the pylorus.Patients' characteristics were not different between the two groups. There was only one wound infection and no major complication associated with LP with VC, whereas there were three major complications in TLP. Our hospital is a pediatric teaching institution in Japan where many surgeons perform their first LP. Twenty-eight surgeons performed their first LP in our hospital in the past 17 years, and LP with VC was the first LP for nine. The operative time for surgeons' first LP was significantly shorter with LP with VC than with TLP, based on Welch's t test (42.8 versus 54.4 minutes; P = .048).Our results suggest that LP with VC is an easy and safe technique, and it is recommended for beginners in laparoscopic surgery.

The number of female surgeon is continuously increasing, while the total number of surgeon is decreasing. The author has faced many difficulties while working as a pediatric surgeon and a mother of three children. Those difficulties were caused by the traditional sexual role in our society and by a fixed idea that the priority for a surgeon should be his or her profession. Here, the author addressed some suggestions which could lead the society of surgeons to a gender-equal one, and could make surgery a great appeal to female surgeons and to the young generations which consider private life as important as their professions as well. Suggestions include a change the relationship between a female surgeon and her partner, a supplement of surgeons so that hospitals could change the traditional system of surgery. The author proposed to increase female managers intentionally, so that the sense of sexual difference would be diminished and proper evolution of the society would be achieved.

We report a case of gastroschisis in which a paraumbilical band was found at the right margin of the abdominal wall defect and extended into the antimesenteric side of the small intestine. The band consisted of 2 thin cords. Microscopically, 1 band showed a fibrous tissue, and the other 1 revealed a unique vascular structure resembling the vitelline artery and vein, suggesting that the paraumbilical band represents a remnant of the yolk stalk that failed to be incorporated into the umbilical stalk. The origin of the paraumbilical band and an associated pathogenetic hypothesis of gastroschisis are discussed.

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.

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.

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.