Long-term cosmetic outcomes of the slit-slide procedure for umbilical hernia repair in children.

PURPOSE: To assess the long-term cosmetic outcomes of the "slit-slide procedure", designed to provide a more natural appearance for umbilical hernia repair, as perceived by the patients and their parents. METHODS: A total of 149 patients with umbilical hernia underwent the slit-slide procedure at our hospital. The slit-slide procedure allows for the creation of an umbilicus with a more natural and integrated appearance. The patient satisfaction survey questionnaire was mailed to the families (n = 139), and there were 74 (53.2%) respondents. A questionnaire survey on postoperative appearance was also distributed to pediatric surgeons. RESULTS: The median age at the time of operation was 2.5 years (range, 2 months to 14 years) and the average median age at the time of answering the questionnaire was 6.25 years old (range, 2.5-14.8 years). The average median period of observation was 3.2 years (range, 4 months to 8.97 years). Most patients (89.2%) and parents (93.2%) were satisfied with the appearance of the umbilicus. Twenty-seven patients reported improved satisfaction after surgery (36.2%). Surgeons tended to score the elongated-oval shape highly; however, there was no difference in satisfaction among the shapes. CONCLUSION: The slit-slide procedure is not only effective and safe, but it achieves a satisfactory aesthetic outcome.

Early diagnosis of neonatal-onset cyclic vomiting syndrome.

Cyclic vomiting syndrome (CVS) is characterized by recurrent episodes of severe vomiting with a completely asymptomatic interictal interval. Relatively few patients develop CVS in the neonatal period, and an early diagnosis is difficult. We experienced an infant who was diagnosed with neonatal-onset CVS in early infancy. An 8-day-old girl was admitted to our neonatal intensive care unit because of frequent vomiting beginning 12 h after birth and weight loss reaching 84.2% of her birth weight. Despite extensive examinations, no abnormalities to explain the vomiting were found. She continued to vomit, and a cyclical pattern with a vomiting phase lasting for three days followed by a non-vomiting phase lasting for about one to two weeks became obvious. Based on her clinical course, the family history of migraine and the effectiveness of Phenobarbital, she was diagnosed with CVS at three months old. Although CVS is a diagnosis of exclusion, a family history of migraine can aid its early diagnosis. If the illness is suspected in the neonatal period, diagnostic treatment with Phenobarbital may be considered. The case suggests the need to include CVS in the differential diagnosis of neonates with unexplained repetitive vomiting.

"A salvage technique using a fibrous sheath to avoid the loss of the central veins in cases of pediatric intestinal failure".

PURPOSE: The number of accessible central veins (CVs) affects the prognosis of patients with intestinal failure (IF). The loss of residual CVs should be avoided. We, therefore, evaluated the efficacy of a new CV catheter-exchange technique using a subcutaneous fibrous sheath (FS) in pediatric IF patients. METHODS: We retrospectively collected the CV catheter (CVC) data of pediatric IF patients managed from January 2009 to December 2019. The data were divided into two groups; Groups 1 (CVCs placed with the FS method) and Group 2 (CVCs placed by the primary or another insertion). The main outcome was the CVC indwelling time. RESULTS: Eighty-five CVCs were analyzed. The FS method was attempted in 47 cases and succeeded in 40 (85%). No significant difference was observed between the groups regarding characteristics. A log-rank test revealed an equivalent CVC indwelling time between the two groups (Group 1: 268 [126-588] days vs. Group 2: 229 [126-387] days, p = 0.256). CONCLUSIONS: The FS method is highly recommended for pediatric IF patients, as its attempt showed a high success rate with an indwelling time equivalent to primary insertion. The FS method leads to the prolonged use of a single CV and thereby contributes to improving the outcomes of pediatric IF patients.

Spontaneous reduction age for ovarian hernia in early infancy.

BACKGROUND: Many articles recommend early surgery for ovarian hernia to avoid the risk of ovarian torsion. However, while ovarian hernia is known to undergo spontaneous reduction (SR) in early infancy, few reports have described the timing of SR. We therefore investigated the clinical features of SR for ovarian hernia in early infancy. METHODS: A total of 610 girls were diagnosed with inguinal hernia between 2008 and 2018. We focused on infants who had an ovarian hernia onset in the first 3 months of age. We reviewed the age retrospectively at the onset of hernia and age at SR. The data were compared statistically using the Kaplan-Meier method. RESULTS: Sixty-one infants with inguinal hernia were included in this study. Thirty-nine patients (64%) had ovarian hernia. The mean age at the onset of hernia was 44 ± 17 days of age. Thirty cases underwent SR (77%). A Kaplan-Meier analysis showed that 75% of ovarian hernias underwent SR by 6 months of age. There were no cases of ovarian torsion. CONCLUSIONS: Most cases of ovarian hernia underwent SR, so patients with ovarian hernia in early infancy might be treated by elective surgery after 6 months of age.

A totally extraperitoneal approach for endoscopic resection of a congenital prepubic sinus through the pubic symphysis.

BACKGROUND: A congenital prepubic sinus (CPS) is a rare congenital anomaly in which a duct remnant extends from the skin opening near the pubic symphysis to various parts and the lesions are mostly located in the preperitoneal space. The totally extraperitoneal (TEP) approach is an operational method that provides a good field of view for the preperitoneal space. We report the CPS through the pubic symphysis in which complete resection was achieved by a TEP approach. TEP approach was minimally invasive and achieved satisfactory cosmetic outcome. CASE PRESENTATION: We herein report the case of a 13-year-old boy with a fistula opening near the dorsal penis. He was admitted to our hospital due to fever and lower abdominal pain. Abdominal ultrasonography and computed tomography revealed an abscess inside a fistula lumen on the posterior surface of the rectus abdominis muscles in the midline of the lower abdomen. Under a diagnosis of CPS, which was located in the preperitoneal space, endoscopic resection was performed by a totally extraperitoneal approach. After making an umbilical incision, the rectus abdominis muscle was excised outward to expose the preperitoneal space. A single-port system was placed in the preperitoneal space. Three 5-mm-port trocars were inserted. As the preperitoneal cavity was expanded, a sinus connecting to the pubic symphysis was confirmed. The pubic symphysis did not connect with the bladder. Because the fistula was penetrated with the pubic symphysis, the remaining caudal fistula was removed from the body surface with a small spindle-shaped incision around the fistula opening. Finally, the sinus was completely resected, with confirmation from both the cranial side and dorsal side of the pubic symphysis. We were able to perform complete resection of the CPS with good visibility and without any peritoneal damage. There were no intraoperative complications. His postoperative course was uneventful during the 1-year follow-up. CONCLUSIONS: The TEP approach may be feasible for the resection of a CPS and may allow safe and secure resection due to good visibility, even in pediatric patients.

Acute isolated Aspergillus appendicitis in pediatric leukemia.

Aspergillus is a widespread fungus in the environment, usually invades through the respiratory tract. Invasive aspergillosis is a fatal disseminated infection in immunocompromised hosts. Appendicitis occurs scarcely in patients with leukemia. We report a case of Aspergillus appendicitis that underwent an urgent appendectomy. An 11-year-old boy received the diagnosis of acute myeloid leukemia, because of the bone pain and results of the bone marrow study. He obtained a complete remission after cancer chemotherapy and received peripheral blood stem cell transplantation from a histocompatible sibling. Leukemia relapsed 5 months post-transplant. Induction therapy with etoposide, cytarabine and mitoxantrone was started on Candida prophylaxis. Fifteen days after the end of chemotherapy, he presented with febrile neutropenia and abdominal pain, that did not respond to broad-spectrum antibiotics. Serum levels of C-reactive protein, ß-D-glucan and procalcitonin were unremarkable. Computed tomography scan revealed a swollen appendix and the adjacent tissue inflammation. An urgent appendectomy led to a tentative diagnosis of Aspergillus appendicitis based on the histopathological findings of many fungal hyphal forms. Panfungal polymerase chain reaction using DNA extracted from the lesion determined the pathogen of Aspergillus niger. There was no evidence of invasive aspergillosis. During the prolonged anti-fungal therapy, he achieved a remission of leukemia and underwent the second hematopoietic cell transplantation. To our knowledge, Aspergillus appendicitis was reported to occur in 5 leukemia patients. Four of them survived after appendectomy and one died from intestinal perforation. Early surgical intervention is mandatory for a cure of Aspergillus appendicitis in neutropenic patients on Candida prophylaxis.

Which Is the Better Approach for Late-Presenting Congenital Diaphragmatic Hernia: Laparoscopic or Thoracoscopic? A Single Institution's Experience of more than 10 Years.

Aim: We evaluated a series of late-presenting congenital diaphragmatic hernia (Late-CDH) cases and assessed the reliability and risks of laparoscopic and thoracoscopic approaches for Late-CDH at a single institution. Materials and Methods: From 2005 to 2017, we experienced totally 11 patients with Late-CDH who received endoscopic repairs. We retrospectively surveyed the approach, defect size, operating time, and postoperative outcomes, including recurrence. Results: Eleven patients (Bochdalek, n = 10; Morgagni, n = 1) underwent a total of 14 endoscopic repairs (laparoscopy, n = 10; thoracoscopy, n = 4). The average defect size was ∼3.1 × 1.5 cm. In all 14 endoscopic repairs, patients received intracorporeal interrupted nonabsorbable stitches and extracorporeal knot tying were applied, without the use of an artificial patch. In the laparoscopic repairs, 7 patients received left-handed suturing when closing the diaphragmatic defect, because the reduced viscera lay directly below the posterior rim of the diaphragmatic defect, making it difficult to confirm the rim. In contrast, in the thoracoscopic repairs, the viscera were reduced over the diaphragmatic defect, so the surgeons could easily perform suturing. The average operating time was 172 minutes for laparoscopy and 194 minutes for thoracoscopy. No major intraoperative or postoperative complications occurred in association with either of the approaches. Among the 11 patients, 2 experienced a total of 3 recurrences (all after laparoscopic repairs). Conclusion: Although there were few differences between the laparoscopic and thoracoscopic approaches, because of the technical difficulty of the procedure and the possibility of recurrence with the laparoscopic approach, a thoracoscopic approach may be better for the repair of Late-CDH.

Efficacy of Prophylactic Negative Pressure Wound Therapy After Pediatric Liver Transplant.

OBJECTIVES: Wound dehiscence is a common surgical complication, especially among pediatric liver transplant recipients in our center. In 2013, we introduced negative pressure wound therapy as a preventive treatment. We herein report the clinical outcomes of this intervention. MATERIALS AND METHODS: We conducted a retrospective review of the 26 pediatric liver transplant recipients in our center since 2011. We excluded 1 girl whose wound could not be closed due to bowel edema. The first 13 of the 25 remaining patients were treated with conventional wound management (conventional group). The latter 12 were treated with prophylactic negative pressure wound therapy (prophylactic group). Incidences of surgical complications and patient characteristics were compared between groups. RESULTS: Wound dehiscence occurred in 7 of the 13 patients in the conventional group and 3 of the 12 patients in the prophylactic group. When restricted to dehiscence that required surgical debridement, there were 6 cases in the conventional group and no cases in the prophylactic group. Although background data showed that liver insufficiency in the prophylactic group was more severe, this group had a lower incidence of wound dehiscence (P = .015). CONCLUSIONS: Prophylactic negative pressure wound therapy is thought to be effective for preventing wound dehiscence among pediatric liver transplant recipients.

Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease, 2017.

BACKGROUND: Despite the presence of ganglion cells in the rectum, some patients have symptoms similar to those of Hirschsprung's disease. A consensus has yet to be established regarding the terminology for these diseases. We defined this group of diseases as "allied disorders of Hirschsprung's disease" and compiled these guidelines to facilitate accurate clinician diagnosis and provide appropriate treatment strategies for each disease. METHODS: These guidelines were developed using the methodologies in the Medical Information Network Distribution System (MINDS). Of seven allied disorders, isolated hypoganglionosis; megacystis-microcolon-intestinal hypoperistalsis syndrome; and chronic idiopathic intestinal pseudo-obstruction were selected as targets of clinical questions (CQ). In a comprehensive search of the Japanese- and English-language articles in PubMed and Ichu-Shi Web, 836 pieces of evidence related to the CQ were extracted from 288 articles; these pieces of evidence were summarized in an evidence table. RESULTS: We herein outline the newly established Japanese clinical practice guidelines for allied disorders of Hirschsprung's disease. Given that the target diseases are rare and intractable, most evidence was drawn from case reports and case series. In the CQ, the diagnosis, medication, nutritional support, surgical therapy, and prognosis for each disease are given. We emphasize the importance of full-thickness intestinal biopsy specimens for the histopathological evaluation of enteric ganglia. Considering the practicality of the guidelines, the recommendations for each CQ were created with protracted discussions among specialists. CONCLUSIONS: Clinical practice recommendations for allied disorders of Hirschprung's disease are given for each CQ, along with an assessment of the current evidence. We hope that the information will be helpful in daily practice and future studies.

Parameters that help to differentiate biliary atresia from other diseases.

BACKGROUND: The diagnosis of biliary atresia (BA) is still challenging. The aim of this study was to analyze the clinical features and parameters that contribute to a diagnosis of BA. METHODS: From January 2000 to December 2013, 37 patients who underwent operative cholangiography were evaluated retrospectively. The patients were divided into two groups: a BA group, and a group with other cholestatic diseases (non-BA group). The demographic data and preoperative liver function test results were analyzed. RESULTS: Of the 37 patients, 29 patients were confirmed to have BA. Age at operation was not significantly different between the two groups (P = 0.77). On preoperative liver function tests, only the level of γ-glutamyl transpeptidase (γ-GTP) was significantly higher in the BA group (P = 0.015). The predominant non-BA disease was inspissated bile syndrome (IBS). In the IBS patients, the jaundice was relieved after lavage of the biliary tree. CONCLUSION: The preoperative differentiation of cholestasis is difficult based on laboratory data and imaging. Preoperative γ-GTP may be useful for diagnosing BA, but operative cholangiography should be performed when BA is suspected and cannot be ruled out by other methods, given that the most common non-BA disease may be IBS.

Timing and outcome of stoma closure in very low birth weight infants with surgical intestinal disorders.

PURPOSE: Very low birth weight infants (VLBWIs) are at risk of surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI). We conducted this study to verify whether the timing of stoma closure and that of enteral nutrition establishment after stoma closure in VLBWIs differ among the most common disorders. METHODS: A retrospective multicenter study was conducted at 11 institutes. We reviewed the timing of stoma closure and enteral nutrition establishment in VLBWIs who underwent stoma creation for intestinal disorders. RESULTS: We reviewed the medical records of 73 infants: 21 with NEC, 24 with FIP, and 25 with MRI. The postnatal age at stoma closure was 107 (28-359) days for NEC, 97 (25-302) days for FIP, and 101 (15-264) days for MRI (p = 0.793), and the postnatal age at establishment of enteral nutrition was 129 (42-381) days for NEC, 117 (41-325) days for FIP, and 128 (25-308) days for MRI (p = 0.855). The body weights at stoma closure were 1768 (620-3869) g for NEC, 1669 (1100-3040) g for FIP, and 1632 (940-3776) g (p = 0.614) for MRI. There were no significant differences among the three groups. CONCLUSIONS: The present study revealed that the time and body weights at stoma closure and the postoperative restoration of bowel function in VLBWIs did not differ among the three diseases.

The evaluation of rectal mucosal punch biopsy in the diagnosis of Hirschsprung's disease: a 30-year experience of 954 patients.

PURPOSE: For 30 years, we have consecutively performed rectal mucosal punch biopsy to diagnose Hirschsprung's disease. The aim of this study was to evaluate the safety of our technique. METHODS: Patients with suspected Hirschsprung's disease who underwent punch biopsy, including our original "K-PUNCH" method using an S-moid forceps and non-specific blood-collecting tube at our department and branch hospital between April 1986 and March 2016 were included in the present study. Our punch biopsy technique is characterized by excellent visibility and a direct grasping sensation. The backgrounds and complications of the patients were retrospectively investigated. RESULTS: During this period, 954 patients (median age 4 months; range 1 day-73 years) underwent punch biopsy. Although there were no cases of severe complications (i.e., rectal perforation, infection or full-thickness biopsy), one (0.1%) of the 954 cases in the early period showed liver dysfunction and required transfusion due to bleeding. In addition, inappropriate specimens were obtained in 37 patients (3.9%). CONCLUSION: Punch biopsy including the "K-PUNCH" method is considered safe and feasible and is associated with a low rate of complications and inappropriate specimen harvesting among patients of all ages. Comorbidities, including the potential for hemorrhage, should always be considered.

Insufficient Portal Vein Inflow in Children without Major Shunt Vessels During Living Donor Liver Transplantation.

BACKGROUND Liver cirrhosis is frequently accompanied by insufficient portal vein inflow (IPVF) with large portosystemic shunts. However, pediatric cases often manifested IPVF without any apparent major portosystemic shunts. Although IPVF is a very critical issue, the intraoperative assessment has not been well established. In this study, we reviewed the intraoperative approach and the outcome of the IPVF cases at our department. MATERIAL AND METHODS Eighty-three living donor liver transplantations (LDLT) were performed from 1996 to 2014. The IPVF occurred in 5 cases and necessitated some additional assessments and intraoperative PV flow modulations. We retrospectively reviewed the operative records and analyzed the risk factors and the outcome of the IPVF. RESULTS All 5 IPVF cases were biliary atresia and the mean age at LDLT was 0.74±0.19 years old. The mean recipient PV diameter was 4.3±0.8 mm and the donor IMV patch grafts were applied. To increase the PV inflow, the collaterals around the spleen were ligated in all cases. Intraoperative portal venography was performed in 1 case for selective shunt vessel ligation. In 1 case, the graft was removed and returned to the back table to prevent graft loss during the IPVF. As a result, the final PVF/GV increased to 66.4±20.0 ml/min/100 g. CONCLUSIONS IPVF is a very critical problem. Intraoperative portal venography is helpful and collateral veins ligation is crucial. In some cases, returning the graft to the back table during the PV inflow modulation can prevent graft loss.

Bowel obstruction without history of laparotomy: Clinical analysis of 70 patients.

BACKGROUND: Determining the cause of bowel obstruction without a history of laparotomy (BO without HL) is difficult and can result in delay of treatment and development of a potentially life-threatening situation. We herein investigated the clinical characteristics of pediatric patients who underwent laparotomy due to BO without HL. METHODS: All surgical pediatric patients aged <16 age who were diagnosed with BO without HL between January 2004 and September 2014 were included. Etiology of BO, intraoperative findings and postoperative outcome were investigated retrospectively. RESULTS: Seventy patients were diagnosed with BO without HL in this period. In these 70 patients, malrotation (n = 34), intussusception (n = 19), internal hernia (n = 6) and Meckel's diverticulum (n = 5) were predominantly identified. Regarding preoperative definitive diagnosis, prevalence of internal hernia, Meckel's diverticulum or idiopathic volvulus was significantly lower than that of malrotation or intussusception (P < 0.05). Intraoperatively, the rates of strangulation and bowel resection were 55.7% and 30.0%, respectively. The optimal time for emergency operation in order to avoid strangulated bowel resection was <19 h from onset of symptoms. CONCLUSIONS: Malrotation and intussusception are major causes of BO without HL in children, but internal hernia, Meckel's diverticulum and idiopathic volvulus should always be taken into account, particularly because of the preoperative diagnostic difficulty and resulting high rate of intestinal resection. In order to avoid resection of the bowel, surgery should be done within 19 h before bowel ischemic change occurs.

Transient hyperphosphatasemia after pediatric liver transplantation.

BACKGROUND: Transient hyperphosphatasemia (TH), the incidence of which in healthy children is 1.5-2.8%, is associated with a temporary elevation of serum alkaline phosphatase (ALP) without any other liver function test (LFT) abnormalities. Fast α2 band, detected on agarose gel electrophoresis, is known to be a highly sensitive phenomenon in TH. The aim of this study was to elucidate the characteristics of TH after liver transplantation (LT). METHODS: Five TH patients (6.0%) out of 83 who underwent LT between October 1996 and October 2014, were included in this study. Patient background, duration of TH, peak ALP, time course of ALP and its isoenzyme in TH, other LFT, and imaging results were retrospectively investigated. RESULTS: Median patient age at the time of peak ALP during TH was 24 months (range, 16-98 months). Mean duration of TH was 111.0 ± 48.0 days. At 6887 IU/L, median ALP was elevated without any other LFT abnormalities. The first α2 band was detected in all patients, and the band disappeared at the same phase of ALP normalization. All of the patients improved without any treatment. The prevalence of TH was significantly higher in the patients after LT than in age-matched healthy children (P < 0.05). CONCLUSIONS: The detection of fast α2 band in the early phase allows for effective diagnosis of TH after LT, and 3-4 month follow up without treatment is feasible.

Massive pulmonary hemorrhage before living donor liver transplantation in infants.

A massive pulmonary hemorrhage in patients with liver cirrhosis is a life-threatening complication that may result in a contraindication of a liver transplantation because of its high mortality rate. Herein, we present two infant biliary atresia cases that successfully underwent an LDLT that was followed by intensive respiratory care for the pretransplant massive pulmonary hemorrhage. Both cases exhibited severe respiratory failure (minimum PaO2/FiO2; 46 mmHg and 39 mmHg, respectively). To arrest the bleeding, we applied a very high positive pressure ventilation treatment (maximum PIP/PEEP; 38/14 cmH2O and 55/15 cmH2O, respectively), plasma exchange, several FFP transfusions, and recombinant factor VIIa via intrapulmonary administration. In addition, we used CHDF treatment, applied HFOV transiently, and treated the patient with inhalation of nitric oxide. Although we prepared ECMO for intra-operative use, both cases were successfully managed with conventional mechanical ventilation without using ECMO, which may have worsened the pulmonary hemorrhage due to the use of an anticoagulant. Use of an excessive positive pressure management, although it poses a risk for barotrauma, could be acceptable to arrest the pulmonary bleeding in selected cases of liver failure patients who have no time remaining before LDLT.

Therapeutic potential of mesenchymal stem cell transplantation in a nitrofen-induced congenital diaphragmatic hernia rat model.

PURPOSE: The aim of this study was to evaluate the efficacy of mesenchymal stem cells (MSCs) in a nitrofen-induced congenital diaphragmatic hernia (CDH) rat model. METHODS: Pregnant rats were exposed to nitrofen on embryonic day 9.5 (E9.5). MSCs were isolated from the enhanced green fluorescent protein (eGFP) transgenic rat lungs. The MSCs were transplanted into the nitrofen-induced E12.5 rats via the uterine vein, and the E21 lung explants were harvested. The study animals were divided into three: the control group, the nitrofen-induced left CDH (CDH group), and the MSC-treated nitrofen-induced left CDH (MSC-treated CDH group). The specimens were morphologically analyzed using HE and immunohistochemical staining with proliferating cell nuclear antigen (PCNA), surfactant protein-C (SP-C), and α-smooth muscle actin. RESULTS: The alveolar and medial walls of the pulmonary arteries were significantly thinner in the MSC-treated CDH group than in the CDH group. The alveolar air space areas were larger, while PCNA and the SP-C positive cells were significantly higher in the MSC-treated CDH group, than in the CDH group. MSC engraftment was identified on immunohistochemical staining of the GFP in the MSC-treated CDH group. CONCLUSIONS: MSC transplantation potentially promotes alveolar and pulmonary artery development, thereby reducing the severity of pulmonary hypoplasia.

[Progress in and outcomes of neonatal surgery over the past 50 years].

The Japanese Society of Pediatric Surgeons has performed a nationwide survey of neonatal surgery every 5 years during the 50 years since its foundation. The number of neonatal surgical cases has increased 5-fold during those 50 years, while the mortality rate has decreased from 60% to 15% for the main life-threatening diseases (such as esophageal atresia, diaphragmatic hernia, omphalocele, and gastroschisis). Currently, most neonatal patients who undergo surgery for severe cardiac or chromosomal anomalies survive. Therefore, pediatric surgeons are aware of the need for cosmetic considerations. Endoscopic surgical procedures and incisions using natural skin creases have been developed to achieve good cosmetic results and improve patients' quality of life. On the other hand, severe life-threatening neonatal surgical cases are still seen, such as patients with diaphragmatic hernia accompanied by severe pulmonary hypoplasia, huge sacrococcygeal teratomas, and neonatal intestinal perforation. Fetal intervention or early delivery should be considered to improve survival. Appropriate initial treatment strategies for intestinal perforation in very low birth-weight infants are needed.

The utility of muscle sparing axillar skin crease incision for pediatric thoracic surgery.

BACKGROUND: Posterolateral or standard axillar incisions for the pediatric thoracic surgery are occasionally associated with poor motor as well as cosmetic results, including chest deformities and large surgical scars. A muscle sparing axillar skin crease incision (MSASCI) was initially proposed by Bianchi et al. (in J Pediatr Surg 33:1798-1800, 1998) followed by Kalman and Verebely (in Eur J Pediatr Surg 12:226-229, 2002) resulting in satisfactory cosmetics. However, they performed operations through the third or fourth intercostals space (ICS), therefore the target organs were restricted in the upper two-thirds of the thoracic cavity. PATIENTS AND METHODS: Thoracic surgeries were performed using MSASCI in 27 patients (1-day to 9-year old). There were ten patients with esophageal atresia, seven with congenital cystic adenomatoid malformation, five with pulmonary sequestration, two with mediastinal neuroblastoma, two with right diaphragmatic hernia, and one with pulmonary hypertension. A thoracotomy was performed through the appropriate ICS (from third to eighth). RESULTS: In all patients, the expected procedures, including pulmonary lower lobectomy, were successfully performed by MSASCI throughout the thoracic cavity. A good operational field was easily obtained in neonates and infants. Most of the patients achieved excellent motor and aesthetic outcomes. CONCLUSIONS: MSASCI may become the standard approach for the thoracic surgery for small children.

Significance of abnormalities in systems proximal and distal to the obstructed site of duodenal atresia.

BACKGROUND: Duodenal atresia (DA) is a well-known neonatal intestinal disease. Even after surgery, the proximal segment can continue to be severely dilated with hypoperistalsis, resulting in intestinal dysmotility problems in later life. No data have been published regarding the morphologic differences between the proximal and distal regions of obstructed sites of the intramural components in DA. METHODS: Operative duodenal samples (N = 12) from cases with DA (age 1-3 days) were used. Age-matched controls (N = 2) were used. All of the specimens were immunohistochemically stained with antibodies to S-100 protein, α-smooth muscle actin, and c-kit protein. RESULTS: At the proximal segments of the obstructed site in DA, the number of neuronal cells decreased in size and number. The circular musculature was moderately to severely hypertrophic. Unusual ectopic smooth muscle bundles were also identified. The innermost layer of the circular musculature was thinner. Interstitial cells of Cajal are decreased, even around the myenteric plexus. All of the staining in the distal segments in DA was similar to the control tissues. CONCLUSIONS: Proximal and distal segments in DA differ in the neural cells, musculature, and distributions of the interstitial cells of Cajal. Based on the present study, these morphologic changes may contribute to the onset of postoperative duodenal dysmotility.