Optimal timing of surgery for prenatally diagnosed choledochal cysts.

Objective: Choledochal cysts are increasingly being diagnosed antenatally. The appropriate time of surgical treatment has the greatest impact on the prognosis of choledochal cyst treatment. The purpose of this study was to compare the clinical outcomes of prenatally diagnosed choledochal cysts in infants according to the surgical treatment timing. Methods: We retrospectively reviewed the medical records of infants who underwent surgery for choledochal cysts with antenatal diagnoses. We investigated each patient's demographic information, type of choledochal cyst, serum liver enzyme levels, and surgical outcomes according to the surgical intervention timing. Results: Between May 2006 and December 2020, 93 infants underwent surgery to treat choledochal cysts; among them, 68 had antenatally suspected choledochal cysts. Of the 68 patients, 21 developed symptoms directly after birth. While 38 patients remained asymptomatic, 9 developed symptoms before operation. To compare surgical outcomes, asymptomatic patients were divided into early (13 cases) and late (25 cases) operation groups based on an age benchmark of 30 days. The early surgical group experienced longer times to resume a full diet (6.0 ± 1.6 vs. 4.5 ± 0.7, p < 0.001) and longer postoperative hospital stays (11 ± 3.9 vs. 7.5 ± 0.8, p < 0.001). Surgical complications occurred in two patients in the early operation group. Minimally invasive surgery was performed in 12 patients in the late operation group. In both groups, postoperative liver function recovered at 6 months, with no significant difference. Conclusion: The results of this study showed longer hospital stays, increased diet durations, and postoperative complications in early surgery patients. However, liver function recovery was not different between the early and late operation groups. Thus, asymptomatic patients should be closely monitored, and we recommend that definitive surgical intervention be postponed until 4 months of age or until weight reaches 7 kg.

Comparison of Surgical Outcomes of Laparoscopic Glue and Laparoscopic Suture Hernioplasty in Pediatric Female Inguinal Hernia.

This study aimed to report the surgical outcomes of laparoscopic glue hernioplasty (LGH) compared with conventional laparoscopic suture hernioplasty (LSH) in pediatric female inguinal hernia repair. We retrospectively analyzed 465 female pediatric patients who underwent laparoscopic inguinal hernia repair between January 2013 and December 2020. LGH and LSH were performed in 95 and 370 cases, respectively. Surgical outcomes (length of hospital stay, operative time, complications, and recurrences) were compared between the LGH and LSH groups. We found that the operation times for bilateral hernia repair were shorter in the LGH group (LGH: 35.5 ± 8.2 min, LSH: 45.2 ± 11.6 min; p < 0.001). No significant differences in complications or recurrences were observed between the two groups during the follow-up period. Our findings suggest that LGH is a feasible and easily applied surgical technique for the treatment of pediatric female inguinal hernia.

Changes in outcomes and operative trends with pediatric robot-assisted resection of choledochal cyst.

BACKGROUND: This study aimed to report our experience with a robot-assisted resection of choledochal cysts (CCs) in pediatric patients, especially focusing on changes in outcomes and operative trends. METHODS: We retrospectively reviewed medical records of all 158 patients under 18 years of age who underwent robot-assisted resection of CC in a single tertiary center between July 2008 and January 2021. Patients were divided into the first period (P1, July 2008-March 2016; N = 79) and second period (P2, April 2016-January 2021; N = 79) with equal number of participants. The patients of P2 were compared with those of P1 to assess clinical outcomes with operative details. Operative characteristics and postoperative prognosis were compared for each group. RESULTS: The mean operative time was 383.6 min for the P2 group and 462.6 min for the P1 group (p < 0.001). The mean estimated blood loss was 28 mL in the P2 group and 63 mL in the P1 group (p = 0.025). The rate of emergency department visit after the operation was lower in the P2 group (3.8% vs. 13.9%, respectively, p = 0.047). The two groups showed no significant differences in the rate of late postoperative complications and reoperations. CONCLUSION: With the increase in the center's experience, robot-assisted resection of CC can be safely adopted and feasible, especially for pediatric patients. LEVELS OF EVIDENCE: Treatment Study, Level III.

Multidisciplinary aerodigestive program at a children's hospital: A protocol for a prospective observational study.

BACKGROUND: Children with complex chronic multisystemic diseases frequently require care from multiple pediatric subspecialists. The aerodigestive program is a multidisciplinary program that diagnoses and treats pediatric patients with complex multi-systematic problems affecting airway, breathing, feeding, swallowing, or growth. The aim of this study is to present the protocol of the aerodigestive program of a children's hospital. METHODS AND DESIGN: This study is a prospective study to evaluate and compare the overall improvement of patients' objective and subjective conditions before and after the AeroDigestive Team (ADT) program. Among children from 1 month to 18 years of age, patients with complex problems of the airway, breathing, feeding, swallowing, or growth meeting at least two parameters of the inclusion criteria were enrolled. The overall process included referral based on the inclusion criteria, enrollment of ADT program with informed consents, interview and questionnaire for assessing patients' medical condition, prescheduling appointment, multi-specialists' evaluation, monthly team meetings, wrap-up discussion with the patients and family, therapeutic intervention, and follow-up at 6 months with the assessment of outcome measures. The outcome was evaluated objectively and subjectively. The objective outcome measure was divided into surgical or medical intervention, assessment of changes in medical condition, and follow-up study. Both caregiver interviews and questionnaires using a scoring system were used as subjective outcome measures before and after the ADT program. Children were scheduled to be followed-up at 6 months after the interventions or ADT meeting. DISCUSSION: The aerodigestive program is expected to provide comprehensive and multidisciplinary management of children with complex airway and digestive tract disorders.

Development of a Multidisciplinary Aerodigestive Program: An Institutional Experience.

We share our experience on the implementation of a multidisciplinary aerodigestive program comprising an aerodigestive team (ADT) so as to evaluate its feasibility. We performed a retrospective chart review of the patients discussed at the monthly ADT meetings and analyzed the data. A total of 98 children were referred to the ADT during the study period. The number of cases increased steadily from 3.5 cases per month in 2019 to 8.5 cases per month in 2020. The median age of patients was 34.5 months, and 55% were male. Among the chronic comorbidities, neurologic disease was the most common (85%), followed by respiratory (36%) and cardiac (13%) disorders. The common reasons for consultation were suspected aspiration (56%), respiratory difficulty (44%), drooling/stertor (30%), regurgitation/vomiting (18%), and feeding/swallowing difficulty (17%). Following discussions, 58 patients received active interventions, including fundoplication, gastrostomy, laryngomicrosurgery, tracheostomy, and primary dilatation of the airway. According to the questionnaire of the caregiver, the majority agreed that the main symptoms and quality of life of patients had improved (88%), reducing the burden on caregivers (77%). Aerodigestive programs may provide comprehensive and multidisciplinary management for children with complex airway and digestive tract disorders.

Comparison of the outcomes of biliary atresia with cystic degeneration and isolated biliary atresia: A matched-pair analysis.

BACKGROUND: Patients with biliary atresia (BA) with extrahepatic cystic degeneration (BACD) have a unique pathophysiology; however, clinical outcomes and progression of perinatal degeneration are not well-defined. We aimed to investigate the differences in clinical characteristics and outcomes between BACD and isolated BA (IBA). METHODS: We performed a retrospective analysis of patients with BA who underwent Kasai portoenterostomy (KPE) from August 1997 to January 2018 and compared the clinical features and outcomes between BACD (n = 21) and IBA (n = 237). Matched-pair analysis for age and sex was performed between BACD and IBA groups to reduce confounding. RESULTS: Before matched-pair analysis, we found that BACD patients were younger at KPE (45 vs. 64 days, p = 0.008), showed lower total bilirubin at the 3-month follow-up (0.5 vs. 1.4 mg/dL, p = 0.002), and higher 5-year native liver survival rate (95.2% vs. 61.4%, p = 0.006) than IBA patients. After matching, the BACD group showed significantly lower total bilirubin levels at the 3-month follow-up (0.5 vs. 1.5 mg/dL, p = 0.036) and higher 5-year native liver survival rate (95.2% vs. 57.5%, p = 0.006) than the IBA group. CONCLUSION: BACD demonstrated higher bilirubin clearance and native liver survival rates than IBA. LEVELS OF EVIDENCE: Treatment Study, Level III.

Clinical characteristics and conservative treatment of perineal groove.

PURPOSE: Perineal groove is a rare congenital anomaly of the perineum, and only a few papers describing a small number of cases have been reported in the medical literature. This study aimed to evaluate the clinical characteristics and proper management of perineal groove. METHODS: We performed a retrospective review of 26 pediatric patients who were diagnosed with perineal groove between January 2012 and October 2018 at our institution. RESULTS: Perineal groove was extremely prevalent among the females: 25 of the 26 patients were girls, and only one patient was a boy. All the patients presented with an unusual lesion at the anus or perineum, but no symptoms related to this anomaly. The median age at the first visit to our clinic was 1.5 month (range, 0.3-11.4 month). Two types, complete and partial, were used to categorize the appearance of perineal groove. In a sample group, 55% (12/22) of the patients had complete perineal groove while 45% (10/22) had partial perineal groove. One patient underwent an anoplasty at another hospital following the diagnosis of an imperforate anus. One male and 13 female patients were followed beyond the age of two, and 10 patients (71%) showed a natural healing process. CONCLUSION: Perineal groove manifested as two types of appearance and showed excellent results with conservative treatment in our study. A natural healing process can be expected in the long-term follow-up. Perineal groove must be differentiated from other defects to avoid unnecessary surgical treatment. LEVELS OF EVIDENCE: Therapeutic Study, Level IV.

Pediatric Adenocarcinoma in Korea: A Multicenter Study.

PURPOSE: Adenocarcinoma is an extremely rare malignancy in the pediatric population. Research regarding pediatric adenocarcinoma is very rare in Korea. This study aimed to investigate the clinical features of pediatric adenocarcinomas of various primary organ sites in Korea. MATERIALS AND METHODS: Pediatric patients under 18 years, diagnosed with adenocarcinoma of various sites between January 1995 and December 2016, were included. We retrospectively reviewed patient and tumor characteristics and calculated survival estimates, reported as 5-year survival rate and 95% confidence interval. RESULTS: Of 80 patients (median age, 15 years; range, 10 to 17 years), 37 (46.3%) were men, and 24 (30%) had a family history of cancer or underlying disease relevant to malignancy. The cancer locations were the colon and rectum (n=32), ovaries (n=18), stomach (n=15), lung (n=4), small bowel (n=1), and other sites (n=10). Totally, 54.8% patients (42/77) had stage 3 or 4 disease. The median follow-up period was 2.0 years (range, 0 to 20.4). The 5-year overall survival estimate for all patients, and for those with stomach, colorectal, ovarian, and other cancer sites were 57.9%±11.5%, 58.2%±25.7%, 41.5%±18.2%, 87.5%±16.2%, and 64.0%±34.4%, respectively. The 5-year survival rate differed significantly between categories of adenocarcinomas into gastrointestinal (GI) (44.7%) and non-GI adenocarcinomas (78.8%) (p=0.007). The 5-year survival rate also differed significantly according to carcinoembryonic antigen level (69.3% in < 3 ng/mL, 23.8% in > 3 ng/mL; p < 0.001). CONCLUSION: In pediatric patients, adenocarcinomas arise from various organs and are often diagnosed at advanced stages. Large, prospective studies for their accurate clinical characteristics and prognostic factors are needed.

Mesenteric venous thrombosis as a complication of appendicitis in an adolescent: A case report and literature review.

RATIONALE: Mesenteric venous thrombosis is an uncommon but potentially fatal condition that can cause bowel ischemia. It results from a systemic hypercoagulable state or abdominal infection draining into the portal venous system. Several cases regarding portomesenteric venous thrombosis as a complication of appendicitis were reported in adults, but there are far fewer reports in pediatric patients. The mortality rate of the condition is high if untreated, especially in children, reaching up to 50%. PATIENT CONCERNS: A healthy 15-year-old male with no significant past medical history presented with right lower quadrant pain, lethargy, and fever. The computed tomography scan showed a focal thrombosis at the superior mesenteric vein branch and an inflamed appendix. DIAGNOSES: Mesenteric venous thrombosis complicating acute appendicitis. INTERVENTIONS: Intravenous antibiotics along with anticoagulants and laparoscopic appendectomy OUTCOMES:: After 1 month, a follow-up ultrasonography revealed full resolution of the thrombosis. LESSONS: Appendicitis is one of the most frequently encountered causes of pediatric surgical emergencies; therefore, physicians should be conscious of mesenteric venous thrombosis as a possible complication of acute appendicitis, irrespective of whether patients have thrombophilic conditions or not.

A periodic comparison of the survival and prognostic factors of biliary atresia after Kasai portoenterostomy: a single-center study in Korea.

PURPOSE: We explored the chronologic changes in prognostic factors and clinical outcomes of Kasai portoenterostomy (KPE) for biliary atresia (BA). METHODS: Patients undergoing KPE between 1997 and 2016 were analyzed retrospectively. Ninety-two consecutive patients who underwent KPE from 1997 to 2006 (Era 1) were compared with 150 patients who underwent KPE from 2007 to 2016 (Era 2) for clinical outcomes and prognostic factors. RESULTS: The jaundice clearance rate increased by 8.8% (66.7% vs. 75.5% for Eras 1 and 2, respectively, p = 0.180), and the 5-year native liver survival (NLS) rate improved slightly (62.5% vs. 64.0% for Eras 1 and 2, respectively, p = 0.617) in Era 2. The hazard ratio for age at KPE (≥ 90 days) with regard to 5-year NLS and the odds ratio for age at KPE (< 90 days) with regard to jaundice clearance were both lower in Era 2 than in Era 1 (1.95 vs. 2.25 and 2.67 vs. 5.21, respectively). CONCLUSION: The clinical outcomes improved over a period in a single surgeon's practice. We demonstrated that the impact of age at the time of KPE on operative outcomes became less significant over time with the increase in the single surgeon's experience and improvement in medical treatment for BA.

Predicting Survival of Congenital Diaphragmatic Hernia on the First Day of Life.

BACKGROUND: This study aimed to determine perinatal risk factors for 30-day mortality of congenital diaphragmatic hernia (CDH) patients and develop a prognostic index to predict 30-day mortality of CDH patients. Identifying risk factors that can prognosticate outcome is critical to obtain the best management practices for patients. METHODS: A retrospective study was performed for patients who were diagnosed with CDH from November 2000 to August 2016. A total of 10 prenatal risk factors and 14 postnatal risk factors were analyzed. All postnatal variables were measured within 24 h after birth. RESULTS: A total of 95 CDH patients were enrolled in this study, including 61 males and 34 females with mean gestational age of 38.86 ± 1.51 weeks. The overall 30-day survival rate was 63.2%. Multivariate analysis revealed that five factors (polyhydramnios, gestational age at diagnosis <25 weeks, observed-to-expected lung-to-head ratio ≤45, best oxygenation index in 24 h >11, and severity of tricuspid regurgitation ≥ mild) were independent predictors of 30-day mortality of CDH. Using these five factors, a perinatal prognostic index for 30-day mortality was developed. Four predictive models (poor, bad, good, and excellent) of the perinatal prognostic index were constructed, and external validation was performed. CONCLUSIONS: Awareness of risk factors is very important for predicting prognosis and managing patients. Five independent perinatal risk factors were identified in this study. A perinatal prognostic index was developed for 30-day mortality for patients with CDH. This index may be used to help manage CDH patients.

Laparoscopic repair of inguinal hernia in infants: Comparison with open hernia repair.

PURPOSE: This study aimed to evaluate the usefulness of laparoscopic repair of inguinal hernia (LR) in infants in comparison with open hernia repair (OR). METHODS: We retrospectively analyzed the clinical data of 465 infants treated for inguinal hernia from January 2006 to December 2015. Among them, 124 underwent LR and 341 underwent OR. RESULTS: In the OR group, 16.1% (55/341) primarily underwent bilateral inguinal hernia repair and 13.6% (42/308) subsequently developed metachronous contralateral inguinal hernia during follow-up. In the LR group, 75.8% (94/124) underwent primary bilateral inguinal hernia repair and only 1.6% (2/123) developed metachronous contralateral inguinal hernia. The mean operation times of unilateral inguinal hernia repair showed no statistical differences between LR and OR. However, the mean operation times of bilateral inguinal hernia repair were shorter in LR (39.8±10.4 vs. 51.1±14.4min, p<0.001). Postoperative recurrence and wound infection showed no statistical differences between the groups, but postoperative scrotal swelling was more common in OR (0.0% vs. 4.0%, p=0.006). CONCLUSION: LR in infants showed a lower incidence of metachronous hernia, shorter operation times, and better postoperative course than OR. LR could be considered the primary operation method in infants with inguinal hernia. LEVELS OF EVIDENCE: Prognosis Study, Retrospective Study, Level III.

Correlation between gamma-glutamyl transpeptidase activity and outcomes after Kasai portoenterostomy for biliary atresia.

BACKGROUND/PURPOSE: The role of serum gamma-glutamyl transpeptidase (GGT) levels in predicting clinical outcomes after Kasai portoenterostomy (KPE) is unknown. This study analyzed whether postoperative GGT along with the aspartate aminotransferase-to-platelet ratio index (APRi) predicted prognosis of biliary atresia (BA). METHODS: Data were retrospectively reviewed for 169 BA patients categorized into jaundice-free (JF) (total bilirubin <2.0 mg/dL ≤6 months post-KPE) and persistent jaundice (PJ) groups (total bilirubin ≥2.0 mg/dL ≤6 months post-KPE). Serum biochemical markers, including GGT levels, were measured monthly after KPE, and mean GGT levels and APRi were compared between groups. Factors predicting native liver survival (NLS) were determined using a Cox regression analysis. RESULTS: GGT concentrations >550 IU/L at month 5 (hazard ratio: 1.74, P < 0.05), an APRi >0.605 at month 4 (hazard ratio: 3.78, P = 0.001), and being jaundice-free at 6 months (hazard ratio: 5.49, P < 0.001) were independent risk factors for decreased NLS. CONCLUSIONS: Serum GGT concentrations >550 IU/L at month 5 and an APRi >0.605 at month 4 post-KPE were associated with significantly lower NLS rates. Among JF patients, those with GGT concentrations >550 IU/L at month 5 and APRi >0.605 at month 4 showed poorer outcomes. TYPE OF STUDY: Retrospective comparative study LEVEL OF EVIDENCE: Level III.

Laparoscopic Surgical Glue Injection Hernioplasty: A Single-Institution Experience.

PURPOSE: The aim of this study was to provide clinical evidence supporting the safety and effectiveness of laparoscopic surgical glue injection hernioplasty (LSGIH) for indirect inguinal hernia repair in female patients. METHODS: We conducted a prospective study of LSGIH beginning in January 2013. N-butyl-2-cyanoacrylate was used as the surgical glue for closure of the hernia sac. Twenty-eight female patients with a mean age of 51.1 ± 35.3 months (24 days to 10 years) received LSGIH. The total number of LSGIH operations was 42, including bilateral inguinal hernioplasties. RESULTS: Postoperatively, all the patients were discharged from the hospital within 24 hours without incident. The mean operation time for LSGIH was 41.0 ± 9.7 minutes (30-66 minutes). The mean follow-up period was 23.2 ± 8.3 months (14.6-38.2 months) up to now. There were no postoperative complications or recurrences during the follow-up period. CONCLUSIONS: Our prospective study supports LSGIH as a simple, safe, and useful technique for pediatric inguinal hernioplasty. This method required only one working port and one camera port. However, further clinical prospective trials, including male patients, should be performed to confirm the long-term safety and effectiveness of LSGIH.

Does international study group on pancreatic fistula (ISGPF) classification need modification after distal pancreatectomy?

BACKGROUNDS/AIMS: Few reports have validated the clinical postoperative pancreatic fistula (PF) after distal pancreatectomy. The study intended to validate the predictability for clinical PF of drain amylase and lipase and to find out more appropriate postoperative day (POD) for diagnostic criterion of PF. METHODS: A total of 154 patients underwent distal pancreatectomy. We used the clinical database registry system of the Gangnam Severance Hospital and Severance Hospital, Yonsei University Health System for these analyses. The receiver operating characteristic curve of the drain amylase or lipase concentration on each day was used to predict clinical PF (International Study Group on Pancreatic Fistula [ISGPF] grade B or C) and areas under the curves (AUC) were compared. RESULTS: Amylase and lipase AUC values poorly predicted clinical PF before POD 3 and, gradually increased until POD 5 and became well correlated with clinical PF (ISGPF grade B or C). In contrast, the prediction of clinical PF using drain lipase did not differ from that using drain amylase. The drain amylase concentration on POD 6 was most precisely correlated with clinical PF. CONCLUSIONS: Clinical PF prediction was validated by using drain amylase and lipase concentrations, in which drain amylase assessment at POD 6 appeared to be an appropriate diagnostic criterion of PF after distal pancreatectomy. We suggest some modification of ISGPF definition, especially for distal pancreatectomy.