Cyclic neutropenia with a novel gene mutation presenting with a necrotizing soft tissue infection and severe sepsis: case report.

BACKGROUND: Cyclic neutropenia is a rare disease. We report a 31-month-old girl with congenital cyclic neutropenia with a novel mutation in the ELANE gene who developed an acute necrotizing soft-tissue infection on her left axillary legion. CASE PRESENTATION: A 31-month-old girl was admitted to our pediatric emergency room because of a necrotizing soft tissue infection of the left axillary area. The infection progressed rapidly and resulted in septic shock. Despite a medical treatment and surgical debridement, the sepsis was not controlled, and severe inflammation developed. After applying of negative-pressure wound therapy, her clinical symptoms improved. Finally, she was diagnosed with cyclic neutropenia with a novel genetic mutation. One month after admission, she was discharged with a completely recovered wound and no need for skin grafting. CONCLUSION: Both adequate medical treatment and effective control of the source of infection are critically important to reduce morbidity in such complex cases of necrotizing fasciitis as appeared in an immunocompromised pediatric patient.

High occupational stress and low career satisfaction of Korean surgeons.

Surgery is a demanding and stressful field in Korea. Occupational stress can adversely affect the quality of care, decrease job satisfaction, and potentially increase medical errors. The aim of this study was to investigate the occupational stress and career satisfaction of Korean surgeons. We have conducted an electronic survey of 621 Korean surgeons for the occupational stress. Sixty-five questions were used to assess practical and personal characteristics and occupational stress using the Korean occupational stress scale (KOSS). The mean KOSS score was 49.31, which was higher than the average of Korean occupational stress (45.86) or that of other specialized professions (46.03). Young age, female gender, long working hours, and frequent night duties were significantly related to the higher KOSS score. Having spouse, having hobby and regular exercise decreased the KOSS score. Multiple linear regression analysis showed that long working hours and regular exercise were the independent factors associated with the KOSS score. Less than 50% of surgeons answered that they would become a surgeon again. Most surgeons (82.5%) did not want to recommend their child follow their career. Korean Surgeons have high occupational stress and low level of career satisfaction.

Prenatally detected congenital perineal mass using 3D ultrasound which was diagnosed as lipoblastoma combined with anorectal malformation: case report.

We report a case of prenatally diagnosed congenital perineal mass which was combined with anorectal malformation. The mass was successfully treated with posterior sagittal anorectoplasty postnatally. On ultrasound examination at a gestational age of 23 weeks the fetal perineal mass were found on the right side. Any other defects were not visible on ultrasonography during whole gestation. Amniocentesis was performed to evaluate the fetal karyotyping and acetylcholinesterase which were also normal. As the fetus grew up, the mass size was slowly increased more and more. At birth, a female neonate had a perineal mass on the right side as expected. During operation, the anal sphincteric displacement was found near the mass and reconstructed through posterior sagittal incision. This is the first reported case of prenatally diagnosed congenital perineal mass, after birth which was diagnosed as lipoblastoma and even combined with anorectal malformation. This case shows that it can be of clinical importance to be aware of this rare fetal perineal mass in prenatal diagnosis and counseling.

Multiple malignant extragastrointestinal stromal tumors of the greater omentum and results of immunohistochemistry and mutation analysis: a case report.

To report an extragastrointestinal stromal tumor (EGIST) that occurs outside the gastrointestinal tract and shows unique clinicopathologic and immunohistochemical features. In our case, we experienced multiple soft tissue tumors that originate primarily in the greater omentum, and in immunohistochemical analysis, the tumors showed features that correspond to malignant EGIST. Two large omental masses measured 15 cm multiply 10 cm and 5 cm multiply 4 cm sized and several small ovoid fragments were attached to small intestine, mesentery and peritoneum. On histologic findings, the masses were separated from small bowel serosa and had high mitotic count (115/50 HPFs). In the results of immunohistochemical stains, the tumor showed CD117 (c-kit) positive reactivity and high Ki-67 labeling index. On mutation analysis, the c-kit gene mutation was found in the juxtamembrane domain (exon 11) and it was heterozygote. Platelet-derived growth factor receptor (PDGFR) gene mutation was also found in the juxtamemembrane (exon 12) and it was polymorphism. From above findings, we proposed that there may be several mutational pathways to malignant EGIST, so further investigations could be needed to approach this unfavorable disease entity.

Comparison of surgical outcomes among infants in neonatal intensive care units treated by pediatric surgeons versus general surgeons: The need for pediatric surgery specialists.

PURPOSE: This study compared the outcomes of infants who underwent surgery in neonatal intensive care units by pediatric surgeons and by general surgeons. METHODS: This was a retrospective study of infants who underwent surgery in neonatal intensive care units between 2010 and 2014. A total of 227 patients were included. Of these patients, 116 were operated on by pediatric surgeons (PS) and 111 were operated on by general surgeons (GS). The outcome measures were the overall rate of operative complications, unplanned reoperation, mortality rate, length of stay, operative time, and number of total number of operative procedures. RESULTS: The overall operative complication rate was higher in the GS group compared with the PS group (18.7% vs. 7.0%, p=0.0091). The rate of unplanned reoperations was also higher in the GS group (10.8% vs. 3.5%, p=0.0331). The median operation time (90min vs. 75min, p=0.0474) and median length of stay (24days vs. 18days, p=0.0075) were significantly longer in the GS group. The adjusted odd ratios of postoperative complications for GS were 2.9 times higher than that of PS (OR 2.90, p=0.0352). CONCLUSIONS: The operative quality and patient outcomes of the PS group were superior to those of the GS group. LEVEL OF EVIDENCE: III.

Pediatric oncologic endosurgery.

Despite increasing popularity of minimal-invasive techniques in the pediatric population, their use in diagnosis and management of pediatric malignancy is still debated. Moreover, there is limited evidence to clarify this controversy due to low incidence of each individual type of pediatric tumor, huge diversity of the disease entity, heterogeneity of surgical technique, and lack of well-designed studies on pediatric oncologic minimal-invasive surgery. However, a rapid development of medical instruments and technologies accelerated the current trend toward less invasive surgery, including oncologic endosurgery. The aim of this article is to review current literatures about the application of the minimal-invasive approach for pediatric tumors and to give an overview of the current status, indications, individual techniques, and future perspectives.

A Novel Technique of Posterolateral Suturing in Thoracoscopic Diaphragmatic Hernia Repair.

Background Closure of the posterolateral defect in some cases of congenital diaphragmatic hernia (CDH) can be difficult. Percutaneous transcostal suturing is often helpful to create a complete, watertight closure of the diaphragm. A challenge with the technique is passing the needle out the same tract that it entered so that no skin is caught when the knots are laid down into the subcutaneous tissue. This report describes a novel technique using a Tuohy needle to percutaneously suture the posterolateral defect during thoracoscopic repair of CDH. Case We report a case of a 6-week-old infant who presented with a CDH and ipsilateral intrathoracic kidney that was repaired using thoracoscopic approach. The posterolateral part of the defect was repaired by percutaneous transcostal suturing and extracorporeal knot tying. To assure correct placement of the sutures and knots, a Tuohy needle was used to guide the suture around the rib and out through the same subcutaneous tract. The total operative time was 145 minutes and there were no perioperative complications. The patient was followed up for 3 months, during which there was no recurrence. Conclusion Our percutaneous Tuohy technique for closure of the posterolateral part of CDH enables a secure, rapid, and tensionless repair.

A case report of laparoscopic duodenal atresia repair in a neonate using a novel miniature stapling device.

INTRODUCTION: Laparoscopic duodenal atresia repair is an advanced procedure performed in select pediatric surgical centers. Recently, sub-6mm endosurgical staplers have been introduced, facilitating and accelerating the creation of intracorporeal intestinal anastomoses. PRESENTATION OF CASE: We performed a laparoscopic duodenojejunostomy in a one-day-old child with duodenal atresia due to annular pancreas using a novel 5.8mm articulating endostapler with excellent outcome. The technical details are reported. DISCUSSION: Laparoscopic duodenojejunostomy is a technically demanding procedure due to difficulty in hand-sewn anastomosis in a small and restricted space. With this novel 5.8mm articulating endostapler, we were able to perform a quicker and easier anastomosis. CONCLUSION: We report a case of laparoscopic duodenal atresia repair in a neonate using a novel miniature stapling device. This new technique is a safe, quick and easier way to perform laparoscopic duodenal atresia repair.

Multiphoton microscopy: A novel diagnostic method for solid tumors in a prospective pediatric oncologic cohort, an experimental study.

BACKGROUND: The prognosis of solid pediatric tumors strongly correlates with accurate staging and complete local control. Currently, surgeons rely on macroscopic cues and intraoperative cryosection to determine resection borders. Multiphoton Microscopy (MPM) is a real time technique that allows imaging of tissue without time-consuming tissue processing. PURPOSE: This pilot study evaluates the diagnostic potential of MPM in pediatric solid tumors compared to routine histopathology. METHODS: Slides of pediatric tumor samples (nephroblastoma and neuroblastoma [n = 2]; ganglioneuroma, pleuropulmonary blastoma, hepatocellular carcinoma [n = 1]) were prepared to allow direct comparison of MPM with conventional light microscopy. Additionally, we applied MPM to native tumor tissue blocks to evaluate direct visualization of malignant cells through the tumor capsule. Images were interpreted by an attending surgical pathologist. Detectability of tumor-specific features was compared between MPM and conventional histology. RESULTS: A total of 7 tumors from 7 recruited patients were analyzed. All MPM images were accurate in diagnosing typical criteria of each particular neoplasm. In addition, MPM clearly visualized tumors through the capsule without sectioning or labeling procedures. The quality of MPM was sufficient to make the diagnosis and visualize typical entity-specific architectural changes. CONCLUSION: MPM is comparable to conventional histopathology in the diagnosis of pediatric solid tumors without the need for fixation or staining. It therefore has tremendous potential for future real-time intraoperative diagnostics and as an alternative to conventional frozen section histopathology. LEVEL OF EVIDENCE: III.

Comparison of transumbilical laparoscopic-assisted appendectomy versus single incision laparoscopic appendectomy in children: which is the better surgical option?

BACKGROUND: Transumbilical laparoscopic-assisted appendectomy (TULA) is a single incision technique that exteriorizes the appendix through the umbilicus. The aim of this study was to compare the surgical outcomes of TULA with SILA in pediatric appendicitis. METHODS: A retrospective review of medical records between April 2011 and April 2015 identified 250 pediatric patients who underwent single incision laparoscopic appendectomy. Of these, 137 patients underwent TULA and 113 patients underwent SILA. Measured outcomes included patients' demographics, clinical characteristics, operative time, length of stay, pain, and postoperative complications. RESULTS: TULA group had a shorter operative time than SILA group (28.93 vs. 49.19min, p<0.001). The use of rescue analgesics was more frequent in the SILA group (six cases (6.5%) vs. 19 cases (23.4%), p<0.001). There was no significant difference in cosmetic outcome between the two groups. However, TULA was associated with a lower complication rate (2/137, 1.5%) than SILA (11/113, 9.8%) (p=0.0035). In multiple logistic regression analysis, TULA was significantly associated with a lower complication rate (p=0.049). CONCLUSIONS: TULA is preferable to SILA for treating pediatric acute appendicitis because it is technically easier, results in better surgical outcomes, and provides the same excellent cosmetic results.

Transumbilical laparoscopic-assisted appendectomy is a useful surgical option for pediatric uncomplicated appendicitis: a comparison with conventional 3-port laparoscopic appendectomy.

PURPOSE: Transumbilical laparoscopic-assisted appendectomy (TULA) is a single incision technique that uses a combined intra- and extracorporeal method. The aim of this study was to compare surgical outcomes of TULA with conventional 3-port laparoscopic appendectomy (LA). METHODS: A retrospective review of medical records between 2010 and 2014 identified 303 pediatric patients who underwent LA with uncomplicated acute appendicitis. Of these, 85 patients underwent TULA and 218 patients underwent conventional LA. Demographic data, clinical characteristics, perioperative outcomes and postoperative complications were compared between the 2 groups. RESULTS: The mean operation time in the TULA group was 30.39 minutes, which was significantly shorter than that of the LA group (47.83 minutes) (P < 0.001). The first day of oral intake after surgery was earlier (1.05 days vs. 1.32 days; P < 0.001) and the length of hospital stay was also shorter (2.54 days vs. 3.22 days; P < 0.001) for the TULA group than the LA group. Furthermore, the postoperative complication rate was lower in the TULA group (1 of 85, 1.25%) compared to the LA group (19 of 218, 8.7%) (P = 0.018). CONCLUSION: In conclusion, TULA procedure is recommended for uncomplicated appendicitis in children due to its simplicity and better postoperative outcomes.

Comparative Outcomes of Hand-assisted Laparoscopic Donor Nephrectomy Using Midline Incision or Low Transverse Incision for Hand-assisted Port Placement.

OBJECTIVES: Hand-assisted laparoscopic donor nephrectomy is performed in many centers for donor nephrectomy. A midline incision for hand-assisted port placement is generally used but produces an unsightly scar. In this study, patients who had hand-assisted laparoscopic donor nephrectomy with low transverse incision were compared with those who received a midline incision. MATERIALS AND METHODS: Our study group included patients who received hand-assisted laparoscopic donor nephrectomy from February 2012 to December 2014 at Korea University Anam Hospital. We retrospectively compared outcomes of these patients based on midline incision (45 patients) versus low transverse incision (17 patients). Risk factors, including age, sex, body mass index, creatinine level, glomerular filtration rate of allograft, side of graft kidney, number of renal arteries, duration of surgical procedure, and warm ischemic time, were compared between the midline and low transverse incision groups. RESULTS: When we compared the midline versus low transverse incision groups, duration of surgical procedure (P = .043), postoperative day 3 glomerular filtration rate (P = .017), and postoperative day 3 pain score (P = .049) were significantly higher in the low transverse incision group versus the midline incision group. Postoperative day 3 results for duration of hospitalization (P = .030) and pain score (P = .021) were also significantly higher in the low transverse versus midline incision groups when we focused on patients with left nephrectomy. CONCLUSIONS: Hand-assisted laparoscopic donor nephrectomy with low transverse incision is more painful and necessitates a longer hospital stay and longer surgical procedure. Despite these disadvantages, hand-assisted laparoscopic donor nephrectomy with low transverse incision can offer a better cosmetic outcome with no definitive differences regarding renal function compared with a midline incision. Surgeons should consider these aspects when deciding on the best method for donor nephrectomy.

Occupational stress and related factors among surgical residents in Korea.

PURPOSE: The application rate for surgical residents in Korea has continuously decreased over the past few years. The demanding workload and the occupational stress of surgical training are likely causes of this problem. The aim of this study was to investigate occupational stress and its related factors in Korean surgical residents. METHODS: With the support of the Korean Surgical Society, we conducted an electronic survey of Korean surgical residents related to occupational stress. We used the Korean Occupational Stress Scale (KOSS) to measure occupational stress. We analyzed the data focused on the stress level and the factors associated with occupational stress. RESULTS: The mean KOSS score of the surgical residents was 55.39, which was significantly higher than that of practicing surgeons (48.16, P < 0.001) and the average score of specialized professionals (46.03, P < 0.001). Exercise was the only factor found to be significantly associated with KOSS score (P = 0.001) in univariate analysis. However, in multiple linear regression analysis, the mean number of assigned patients, resident occupation rate and exercise were all significantly associated with KOSS score. CONCLUSION: Surgical residents have high occupational stress compared to practicing surgeons and other professionals. Their mean number of assigned patients, resident recruitment rate and exercise were all significantly associated with occupational stress for surgical residents.

Analysis of the occupational stress of Korean surgeons: a pilot study.

PURPOSE: Surgeons serve one of the most challenging and stressful professions. Ineffective control of occupational stress leads to burnout of the surgeon. The aim of this study was to obtain preliminary data on the sources and the degree of stress of surgeons and to determine the feasibility of the survey. METHODS: A total of 63 surgeons in our three affiliated hospitals were enrolled in this study. Fifty-five questions were used to assess the demographics, characteristics and Korean occupational stress scale (KOSS), which were prepared and validated by the National Study for Development and Standardization of Occupational Stress. RESULTS: Forty-seven of the 63 surgeons participated in this study (74.6%). The mean KOSS score of the survey was 50.9 ± 8.55, which was significantly higher than that of other professions (P < 0.01). Drinking and smoking habits were not related to the KOSS score. Doing exercise was related to a low KOSS score in terms of low KOSS total score (P < 0.01). Average duty hours (P < 0.01) and night duty days per week (P = 0.01) were strongly related to higher KOSS in the linear regression analysis. CONCLUSION: This is the first study to evaluate job stress of surgeons in Korea. This study showed that Korean Surgeons had higher occupational stress than other Korean professions. A larger study based on this pilot study will help generate objective data for occupational stress of Korean Surgeons by performing a survey of the members of the Korean Surgical Society.

Laparoscopic hernia sac transection and intracorporeal ligation show very low recurrence rate in pediatric inguinal hernia.

BACKGROUND: There are many variable techniques for laparoscopic inguinal hernia repair in children. The aim of this study was to examine the feasibility of the laparoscopic hernia technique, which is based on the same surgical principles as conventional open herniotomy. SUBJECTS AND METHODS: Between November 2008 and August 2011, 207 pediatric patients with inguinal hernia were included in this study under informed consent. All of the patients underwent the laparoscopic technique of sac transection and intracorporeal ligation. An asymptomatic contralateral patent processus vaginalis (cPPV) was repaired using the same method. Patients were routinely followed for 2 years. Perioperative complications and recurrence rate were evaluated. RESULTS: In total, 202 patients were enrolled in the final analysis. The mean follow-up period was 12.5 months. The percentage of patients with cPPV was 39.2%. The mean operation time was 26 minutes for unilateral hernias and 34 minutes for bilateral hernias. One patient had a mild hematoma in the inguinal area in the immediate postoperative period, but it resolved spontaneously within 7 days. Recurrence and metachronous hernias have not been observed thus far. CONCLUSIONS: Laparoscopic hernia sac transection and intracorporeal ligation can be a safe and effective alternative for conventional herniotomy.

Surgical stress response and clinical outcomes of single port laparoscopic cholecystectomy: prospective nonrandomized study.

The levels of interleukin-6 (IL-6) are proportionate to injury; it is the most commonly used quantitative marker in surgical studies. Cytokines and the acute-phase response play an important role in controlling the human immune system. The objective of this study was to compare the systemic acute cytokine response and clinical outcomes of conventional laparoscopic and single port laparoscopic cholecystectomy. We compared patients who underwent single port laparoscopic cholecystectomy (the single port group) with patients who underwent conventional laparoscopic cholecystectomy (the conventional group) according to the clinical variables, IL-6, leukocyte subpopulations, and visual analog scale (VAS) pain score. The mean age in the single port group was significantly younger (P = 0.010) and the mean operation time in the conventional group was significantly shorter (P = 0.002). Postoperative 4-hour VAS pain score was slightly worse in the single port laparoscopic cholecystectomy group, but was not significantly different. We found no difference in clinical outcomes, the level of serum IL-6, C-reactive protein, leukocyte subpopulations, and complications between the two groups. Stress response in single port laparoscopic cholecystectomy is equal to conventional surgery. Postoperative 4-hour VAS pain score was slightly worse and the operation time is significantly longer in the single port laparoscopic cholecystectomy group.

Vascular characterization of clear cell sarcoma of the kidney in a child: a case report and review.

Clear cell sarcoma of the kidney (CCSK) is uncommon pediatric renal tumor and can present a significant therapeutic challenge in those patients whose tumors spread beyond the kidney. Thus, identifying potential novel targets for treatment may be clinically important. Clear cell sarcoma of the kidney is characterized by a unique vascular pattern, in which nests of tumor cells are separated by regularly-spaced, fine fibrovascular septa. This distinctive histopathology raises the possibility that understanding the factors which drive angiogenesis in CCSK tumors may suggest new therapeutic targets. Here, we describe a case of CCSK and present immunohistochemical studies of its vasculature.

Incidence and long-term outcome of young patients with gastric carcinoma according to sex: does hormonal status affect prognosis?

BACKGROUND: We investigated the clinicopathological features and evaluated the prognostic impact of age and sex on patients with gastric cancer. DESIGN: Retrospective study from 1993 to 2000. SETTING: Korea University Medical Center. PATIENTS: A total of 1299 patients with gastric cancer were divided into young (n = 175 [13.5%]) and older (n = 1124 [86.5%]) groups with an age cutoff of 40 years. MAIN OUTCOME MEASURES: Clinicopathological characteristics were investigated and survival analysis was performed according to sex for each age group. RESULTS: Tumor differentiation was significantly different between the 2 age groups. Among male patients, the young group had more undifferentiated tumors than the older group (P < .001) but, in female patients, both differentiation (P < .001) and operative methods (P = .008) were significantly different between the young and older groups. In male patients, the 10-year survival rate of the young group was higher (62.5%) than that of the older group (44.6%) (P = .03). Although it was not statistically significant, the survival rate of the older female group was higher than that of the young group (56.2% vs 51.9%). On multivariate analysis, tumor stage (P < .001) and sex (P = .042) were proved to be independent prognostic factors. CONCLUSIONS: Only tumor differentiation was an important difference between the 2 age groups, and prognosis was not affected by age. However, when sex was added to age as a factor, the older male and young female groups had an unfavorable prognosis. Therefore, we propose that sex hormones such as estrogens contribute to the survival differences, and further studies are needed to confirm this possibility.

L1 expression as a marker for poor prognosis, tumor progression, and short survival in patients with colorectal cancer.

BACKGROUND: L1, a new target gene for Wnt/beta-catenin-TCF signaling, has been identified in the invasive front of colorectal cancer cells in vitro study. The L1 molecule is localized on the cell surface in tumor tissues, accompanied with loss of beta-catenin and E-cadherin. However, such association between L1 expression and prognosis of colorectal cancer has not yet been investigated in clinical study. We investigated the expression of L1, E-cadherin, and beta-catenin in tumor cells to determine correlations between the clinicopathologic characteristics and the expression of these molecules and to evaluate the efficacy of the use of these molecules as prognostic markers for patient survival. METHODS: We investigated 138 patients who received diagnoses of colorectal cancer and who underwent surgery between January 1995 and December 2000 at the Korea University Hospital. Tissues were obtained from paraffin-embedded blocks of the tumors and studied by tissue microarray analysis. Immunohistochemical staining for L1, beta-catenin, and E-cadherin was performed for each specimen. RESULTS: L1 expression was found to be correlated with advanced cancer stage (P = .001), distant metastasis (P < .001), and tumor recurrence (P = .006). Survival analysis showed that reduced expression of beta-catenin and E-cadherin, and expression of L1 were statistically significantly related to poor survival. Multivariate analysis revealed that L1 expression was an independent prognostic factor for patient survival. CONCLUSIONS: L1 expression is associated with tumor progression and poor survival in patients with colorectal cancer and may be clinically useful as a marker for poor prognosis.