Intraoperative OK-432 pleurodesis for preventing recurrence of primary spontaneous pneumothorax in children and adolescents: a single-center experience.

BACKGROUND: Primary spontaneous pneumothorax (PSP) commonly occurs in lean, tall, male children and adolescents. To reduce recurrence rates of PSP, chemical pleurodesis could be helpful for patients undergoing video-assisted thoracoscopic surgery (VATS) wedge bullectomy. The efficacy and safety of intraoperative OK-432 (Picibanil) pleurodesis on preventing the recurrence of PSP in pediatric patients remain unclear. METHODS: It is a retrospective observational study in a single center, between 2014 and 2020, enrolled 48 (8 females) pediatric PSP patients with persistent air leakage at the mean age of 16.3 ± 1.1 years to receive VATS wedge bullectomy and pleural abrasion. Twenty patients received additional intraoperative OK-432 pleurodesis. The clinical characteristics of patients, surgical outcomes, and recurrence rates were analyzed. RESULTS: The OK-432 group had longer operation time (118.6 ± 35.6 vs. 96.5 ± 23.3 min; p < 0.05) and higher proportion of postoperative fever (75.0% vs. 28.5%; p = 0.015) than the standard group. No serious adverse events were noted and other surgical outcomes in the two groups were comparable. After a mean follow-up period of 18.1 ± 19.1 months, the OK-432 group had a lower recurrence rate compared with the standard group (5% vs. 28.6%; p < 0.05, odds ratio 0.13, 95% confidence interval: 0.01-1.15), but it had no significant difference in statistics on the Kaplan-Meier curves (log-rank p = 0.105). CONCLUSION: It was the first study that focused on the addition of intraoperative OK-432 pleurodesis for PSP with persistent air leakage in children and adolescents receiving VATS. It demonstrated the efficacy with a low recurrence rate and short-term safety as a single-center experience. LEVEL OF EVIDENCE: Retrospective review, therapeutic study, Level III.

Laparoscopy versus open surgery for idiopathic intussusception in children.

PURPOSE: This study aims to compare the results of laparoscopy and open surgery for idiopathic intussusception in children as well as evaluate the efficacy of ileopexy. METHODS AND MATERIALS: Between January 2007 and July 2013, children aged <18 years who were operated for intussusception in our institution were reviewed. Patients were classified into two groups, laparoscopy (LAP) and open (OPEN). Both groups were further divided into two subgroups, ileopexy (IP) and non-ileopexy (NIP). Parameters investigated included age, gender, operative indication, surgical procedure, type of intussusception, level of intussusceptum, presence of spontaneously reduced intussusception and pathologic lead points, operative time (OP time), time to oral intake (PO time), length of postoperative hospital stay (LOS), and surgical recurrence. RESULTS: There were 23 and 35 patients in LAP and OPEN group, respectively. No significant difference was found on age, operative indication, surgical procedure, type of intussusception, level of intussusceptum, and presence of spontaneously reduced intussusception between both groups. In LAP group, mean OP time was significantly longer; mean PO time and LOS were significantly shorter. One surgical recurrence occurred in each group (p = 0.76). In comparison of LAP-IP (n = 15) and LAP-NIP (n = 8), OP time, PO time, and LOS were similar in both subgroups. One recurrence was noted in LAP-IP (p = 0.46). The overall conversion rate was 13.0 % (6.8 vs. 25 %, p = 0.21). Compared to patients with intussusceptum to ascending colon, the conversion rate was significantly higher in patients with intussusceptum to transverse and descending colon. With the exclusion of conversion, OP time was significantly shorter in LAP-NIP (p = 0.01). CONCLUSION: Laparoscopy should be considered the primary modality for radiologically irreducible or recurrent idiopathic intussusception in children. Ileopexy provides no benefit on recurrence prevention but contributes to longer OP time.

Coexisting gastrointestinal and hepatobiliary tract anomalies in omphalocele and gastroschisis: A twenty-year experience in a single tertiary medical center.

BACKGROUND: Omphalocele and gastroschisis are the two most common congenital abdominal wall defects; however, no previous study has focused on gastrointestinal and hepatobiliary tract malformations in these two conditions. This study aimed to investigate the demographic characteristics, coexisting congenital gastrointestinal and hepatobiliary tract anomalies, hospital course, and outcomes of patients with gastroschisis and omphalocele. METHODS: This is retrospective chart review of all patients admitted to one tertiary medical center in Taiwan between January 1, 2000 and June 30, 2020 with a diagnosis of gastroschisis or omphalocele. The medical records were reviewed to obtain demographic data regarding coexisting gastrointestinal and hepatobiliary tract anomalies and outcomes. RESULTS: Of the 51 patients included, 21 had gastroschisis and 30 had omphalocele. Gastroschisis was associated with a significantly younger maternal age and a higher incidence of small for gestational age. Of the 30 patients with omphalocele, twelve had associated gastrointestinal and hepatobiliary anomalies. Seven of the 21 patients with gastroschisis had gastrointestinal anomalies, and none had hepatobiliary anomalies. Among the omphalocele patients, three (10%) had documented malrotation, and one developed midgut volvulus. Among gastroschisis patients, four patients (19%) had malrotation, and two developed midgut volvulus. There were no statistically significant differences in postoperative complications or mortality rates between those with and without gastrointestinal/hepatobiliary tract anomalies. CONCLUSION: The diversity of coexisting gastrointestinal and hepatobiliary tract anomalies is higher in the omphalocele than in gastroschisis. In addition, we demonstrate that patients with gastroschisis or omphalocele have a higher rate of intestinal malrotation and midgut volvulus.

Multiseptate gallbladder: A case report and literature review.

RATIONALE: Multiseptate gallbladder (MSG) is a rare congenital gallbladder anomaly. Between 1963 and June 2021, only 56 cases were reported. There is currently no treatment guideline for pediatric or adult cases of MSG. PATIENT CONCERNS: A 14-year-old woman visited our out-patient clinic in September 2020 for epigastric pain that last for 6 months. Honeycomb appearance of the gallbladder was noted under ultrasonography. DIAGNOSIS: The patient was diagnosed with MSG. The diagnosis was confirmed through computed tomography and magnetic resonance cholangiopancreatography. INTERVENTIONS: Cholecystectomy was performed. OUTCOMES: Epigastric pain showed limited improvement after the surgery. Since she was diagnosed with gastritis at the same time, a proton-pump inhibitor was prescribed. Epigastric pain was eventually resolved. LESSONS: MSG cases can undergo cholecystectomy and show good recovery without complications. However, concomitant treatment may be required to resolve in the presence of other symptoms such as epigastric pain.

Importance of early detection of infantile inflammatory bowel disease with defective IL-10 pathway: A case report.

RATIONALE: Infantile inflammatory bowel disease (IBD) is an extremely rare subgroup of IBD that includes patients whose age of onset is younger than 2 years old. These patients can have more surgical interventions, and a severe and refractory disease course with higher rates of conventional treatment failure. Monogenic defects play an important role in this subgroup of IBD, and identification of the underlying defect can guide the therapeutic approach. PATIENT CONCERNS: In 2007, a 4-month-old girl from a nonconsanguineous family presenting with anal fistula, chronic diarrhea, and failure to thrive. She underwent multiple surgical repairs but continued to have persistent colitis and perianal fistulas. DIAGNOSIS: Crohn's disease was confirmed by endoscopic and histologic finding. INTERVENTION: Conventional pediatric IBD therapy including multiple surgical interventions and antitumor necrosis factor alpha agents were applied. OUTCOMES: The patient did not respond to conventional pediatric IBD therapy. Interleukin-10 (IL-10) receptor mutation was discovered by whole-exome sequencing and defective IL-10 signaling was proved by functional test of IL-10 signaling pathway by the age of 12. The patient is currently awaiting hematopoietic stem cell transplantation. LESSONS: Early detection of underlying genetic causes of patients with infantile-IBD is crucial, since it may prevent patients from undergoing unnecessary surgeries and adverse effects from ineffective medical therapies. Moreover, infantile-IBD patients with complex perianal disease, intractable early onset enterocolitis and extraintestinal manifestations including oral ulcers and skin folliculitis, should undergo genetic and functional testing for IL-10 pathway defect.

Spontaneous retroperitoneal hemorrhage in Menkes disease: A rare case report.

RATIONALE: Menkes disease (MD), also known as Menkes kinky hair disease, is a fatal neurodegenerative disease caused by a defect in copper metabolism. The symptoms involve multiple organ systems, such as the brain, lung, gastrointestinal tract, urinary tract, connective tissue, and skin. There is currently no cure for this disease entity, and patients with the classic form of MD usually die from complications between 6 months and 3 years of age. Intracranial hemorrhage secondary to tortuous intracranial arteries is a well-known complication of MD, but spontaneous retroperitoneal hemorrhage, to the best of our knowledge, has never been reported in a patient with MD. Herein, we describe the first case of retroperitoneal hematoma as a complication of MD in a 4-year-old boy. PATIENT CONCERNS: A 4-year-old Taiwanese male patient with MD was referred to the hospital and presented with a palpable epigastric mass. DIAGNOSES: On the basis of the findings of ultrasonography and enhanced computed tomography, the diagnosis was retroperitoneal hematoma. INTERVENTIONS: Interventions included laparotomy with evacuation of the hematoma, manual compression, and suture of the bleeding vessels. OUTCOMES: There were no postoperative complications. LESSONS: This case emphasizes that bleeding in patients with MD is possible at any site in the body owing to the unstable structure of the connective tissues. Timely diagnosis with proper imaging studies can lead to prompt and appropriate management and save patients from this life-threatening condition.

Biliary cysts in children--long-term follow-up in Taiwan.

BACKGROUND: This study analyzed the clinical presentation, postoperative morbidity and mortality and incidence of associated extrahepatic biliary atresia in children with biliary cysts in Taiwan. METHODS: We retrospectively reviewed the records of 158 pediatric patients with biliary cysts seen between June 1981 and July 2004, with follow-up ranging from 12 months to 22 years (mean, 11.2 +/- 6.1 years). Patients were divided into three groups: biliary atresia-associated biliary cyst (BABC, 21 patients), non-biliary atresia-associated choledochal cyst (NBACC) in infancy (37 patients), and late NBACC (> 1 year of age, 100 patients). RESULTS: BABC accounted for 36.2% of the infantile biliary cysts in this study. Extrahepatic cysts in late NBACC had a greater mean diameter than those in infantile NBACC and BABC (21.5 mm vs. 16.0 mm vs. 7.9 mm, p < 0.001). Cholangitis was the most serious complication within 3 months postoperatively in all three groups, resulting in four deaths (two in the infantile NBACC group and one each in the other two groups). Liver cirrhosis developed during long-term follow-up in nine of the 21 patients with BABC, four of whom died. Three of these nine patients underwent liver transplantation and remained well during follow-up. Chronic complications in NBACC occurred mainly in late IVa cases, with persistent intrahepatic dilatation developing in 12 of 35 patients and intrahepatic stones in five. Elevation of serum alanine aminotransferase (ALT) was found preoperatively in 85% of late NBACC and 35% of infantile NBACC cases. Postoperative normalization of ALT occurred after a mean of 152 +/- 23 days and 158 +/- 67 days in late NBACC and infantile NBACC, respectively. Higher ALT levels before operation were associated with a longer period until normalization. CONCLUSION: The possibility of BABC must be included in the differential diagnosis when a small extrahepatic cyst (< 8 mm in diameter) with prolonged jaundice is found in infancy. Postoperative follow-up is essential for patients with NBACC due to their frequently prolonged elevation of serum ALT and possibility of residual intrahepatic dilatation. Cholangitis was the major cause of death within 3 months postoperatively in this study.

Clinical criteria for diagnosing perforated appendix in pediatric patients.

OBJECTIVE: To determine predictive variables associated with a perforated appendix in pediatric patients with acute appendicitis. METHODS: This is a retrospective, cross-sectional, observational study from a medical center with more than 2000 beds (230 beds in Department of Pediatrics and Pediatric Surgery), with a mean admission of more than 12000 cases and 2200 surgeries per year. The outcome variable was perforated appendix, and the predictive variables included demographic and clinical factors. RESULTS: During a 6-year period, appendectomies were performed on 274 patients ranging in age from 1 to 18 years. Perforated appendix was found in 100 children (36.5%). Predictive factors significantly associated with perforated appendix were age younger than 9 years, abdominal pain of more than 2 days' duration, temperature of more than 37.9 degrees C, peritoneal signs, and erythrocyte sedimentation rate of more than 25 mm/h. Abdominal ultrasound was performed in 89 patients (32%). For perforated appendix, the ultrasound had a sensitivity of 35%, specificity of 98%, positive predictive value of 95%, and negative predictive value of 55%. Indications for an abdominal ultrasound were determined from a scoring system using the predictive variables significantly associated with perforated appendix. CONCLUSION: The use of our proposed scoring system to determine the indications to perform an abdominal ultrasound may prove to assist in deciding treatment (medical vs surgical) for children with perforated appendix. Initial antibiotic treatment followed by interval appendectomy would become a more likely treatment option if our study results can be validated in a prospective study.

Recurrent spontaneous subserosal hematoma of ileum causing intestinal obstruction in a patient with menkes disease: A case report.

BACKGROUND: Menkes disease (MD) is a disorder of copper metabolism due to ATP7A gene mutation that leads to severe copper deficiency. Deformed blood vessels can be found in many parts of the body, and intracranial hematoma is generally reported. METHODS: We report a Taiwanese boy with MD who had recurrent spontaneous subserosal hematoma of ileum presenting as intestinal obstruction, with the 2 episodes 23 months apart. The patient returned to the usual physical status after surgical removal of the hematoma. RESULTS: The defective copper metabolism causes dysfunction of a plenty of copper-dependent enzymes, giving rise to unique kinky hair appearance, progressive neurodegeneration, and connective tissue abnormalities. To our knowledge, this is the first report on recurrent subserosal hemorrhage of intestine in MD. CONCLUSION: Owing to the fragile structure of blood vessels, subserosal hematoma should be considered when patients with MD having intestinal obstruction.

Successful treatment of a newborn with congenital hyperinsulinism having a novel heterozygous mutation in the ABCC8 gene using subtotal pancreatectomy.

Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycemia in newborns and infants. CHI is characterized by unregulated secretion of insulin from pancreatic ß: cells. Here, we reported the case of a large-for-gestational-age, full-term newborn that suffered from CHI and developed severe and persistent hypoglycemia at an early stage of life. The infant was nearly unresponsive to medical treatment, which included continuous intravenous glucagon infusion, oral diazoxide, and nifedipine. After medical treatment had failed, an 18-fluoro L-3,4-dihydroxyphenylalanine positron emission tomography scan of the patient showed a focal lesion at the neck of the pancreas. The patient received subtotal pancreatectomy, and shortly after the procedure, the patient's blood sugar returned to the normal range. The patient was confirmed to have a novel heterozygous mutation at position c.2475+1G>A of the ABCC8 gene. This is the first report of a focal form of CHI in a patient in Taiwan, which had preoperatively been confirmed using 18-fluoro L-3,4-dihydroxyphenylalanine positron emission tomography.

VACTERL Association Complicated with Right-sided Congenital Diaphragmatic Hernia.

We describe a neonate with VACTERL association and right-sided congenital diaphragmatic hernia (CDH). Such coexistence is rare. The lack of symptoms during the early neonatal period, the absence of bowel loops herniated into the right thoracic cavity, and an unfinished surgery led to clinical and radiological diagnostic difficulties. Respiratory distress occurred when the patient was 2 months old. Chest radiology plain film revealed typical findings of right-sided CDH. The diagnosis was confirmed after surgical exploration.

Platonin, a cyanine photosensitizing dye, causes attenuation of circulatory shock, hypercoagulable state, and tissue ischemia during heat stroke.

The aim of this study was to investigate the therapeutic effect of platonin, a cyanine photosensitizing dye as well as an inhibitor of proinflammatory cytokines, in an animal model of heat stroke. Anesthetized rats, immediately after the onset of heat stroke, were divided into two major groups and given the following: normal saline (1 mL per kg body weight) intravenously, or platonin (12.5-50 microg/mL per kg body weight) intravenously. They were exposed to ambient temperature of 43 degrees C to induce heat stroke. Another group of rats was exposed to room temperature (26 degrees C) and used as normothermic controls. Their physiologic and biochemical parameters were continuously monitored. When the vehicle-treated rats underwent heat exposure, their survival time values were found to be 18 to 22 min. Resuscitation with intravenous doses of platonin, but not normal saline, immediately at the onset of heat stroke, significantly improved survival during heat stroke (41-147 min). All heat-stressed animals displayed systemic inflammation and activated coagulation, evidenced by increased tumor necrosis factor-alpha, prothrombin time, activated partial thromboplastin time, fibrinogen degradation products, and D-dimer, and decreased platelet count and protein C. Biochemical markers evidenced cellular ischemia and injury/dysfunction: plasma levels of blood urea nitrogen, creatinine, glutamic oxaloacetic transaminase, glutamic pyruvic transaminase, and alkaline phosphatase, and striatal levels of partial pressure of oxygen, local cerebral blood flow, glycerol, glutamate, and lactate/pyruvate were all elevated during heat stroke. The systemic inflammation, hypercoagulable state, and cerebral ischemia and injury during heat stroke were all significantly suppressed by platonin. The data demonstrate that platonin therapy may resuscitate heat stroke victims by reducing circulatory shock, systemic inflammation, hypercoagulable state, and tissue ischemia and injury.

Rapid increase in the height and width of the upper chest in adolescents with primary spontaneous pneumothorax.

BACKGROUND: We determined the chest height in a cohort of patients with primary spontaneous pneumothorax (PSP) who had received chest radiographic examinations prior to the attack. The aim of this study was to determine when their chest height began to change and how this was related to the PSP. METHODS: From June 2009 to February 2012, the chest posteroanterior radiographs of 156 patients with PSP (Group 1) were reviewed. Among another 3134 patients with PSP, we identified 52 patients who had a chest posteroanterior radiograph prior to the attack (Group 2). We also recruited 196 controls for comparison (Group 3). The chest height and chest width at different levels were measured and analyzed. RESULTS: Before 14 years of age, the chest height of patients in Group 2 was no different from that of patients in Group 3. By the age of 14 years, however, the chest height and upper chest width of patients with PSP was significantly higher than that of the normal controls. The difference from normal chest height did not increase at adulthood. CONCLUSION: The rapid increase in chest height and upper chest width is a unique finding in patients with PSP. It might be attributable to the occurrence of PSP. This finding may also help to identify patients who are at risk of PSP.

Diagnosis of Henoch-Schönlein purpura by sonography and radionuclear scanning in a child presenting with bilateral acute scrotum.

Acute scrotum presenting as the only initial manifestation of Henoch-Schönlein purpura (HSP) is so unusual that the diagnosis can easily be missed. We report this condition in a 4-year-old boy admitted with bronchopneumonia. Bilateral painful scrotal swelling with ecchymosis occurred on the second day of hospitalization. Scrotal sonography was performed and a good blood supply was documented. Scrotal nuclear scanning was performed and was consistent with bilateral epididymoorchitis. Multiple purpuric lesions over the lower extremities and perineal region developed on the third day of hospitalization. Intermittent abdominal pain and knee pain developed thereafter. HSP was diagnosed and steroids were prescribed. The symptoms subsided gradually and no complication was noted. This case reminds us that an acute scrotum may be the only initial manifestation of HSP. Sonography and nuclear scanning can help rule out other diseases.

Idiopathic hepatic arterio-portal fistula: report of one case.

Hepatic arterio-portal fistula is a rare cause of portal hypertension in children; it is an abnormal communication of hepatic artery and portal venous system, the most common causes being trauma or malignancy. There were only 11 cases reported in English literature and were not ever reported in Taiwan. We report a 9-year old boy with idiopathic hepatic arterio-portal fistula presented as intractable hematemesis due to esophageal and gastric varices. He had received sclerotherapy twice, and Sugiura operation (resection of the lower part of esophagus, devasculization of the stomach and splenectomy). Idiopathic hepatic arterio-portal fistula was found in angiography examination and the esophageal and gastric varices disappeared after transarterial embolization (TAE). We conclude that angiography is the golden diagnostic method for portal hypertension when the etiology is hepatic arterio-portal fistula and TAE will provide immediately therapy.

Clinical spectrum of alimentary tract duplication in children.

UNLABELLED: Alimentary tract duplication is a rare congenital malformations. Sixteen patients admitted to our pediatric ward were enrolled in a ten-year study over the most recent decade. In this paper, we trace their history and retrospectively analyze sex, age, clinical presentations, duplication size and location, presence of ectopic tissue, complications and additional associated anomalies and prognosis. The duplications were cystic in all cases except one tubular duplication of the colon. The cases included duplication of the stomach in 3 cases (19%); ileum in 12 cases (63%); appendix in 1 case (5%); colon in 3 cases (16%); and rectum in 1 case (5%). The most frequent symptom was intermittent vomiting, half of which occurred before 1 year of age. Other symptoms included abdominal pain (50%), abdominal distension (30%), palpable mass (20%), peritoneal sign (13%), bloody stool (6%), fever (6%), and constipation (6%). The outcome was good, except in 2 patients, 1 of whom died of a perforation of the sigmoid colon duplication, and another who was complicated with multiple congenital anomalies. CONCLUSION: In our series, the average time period from the initial onset of symptoms to the diagnosis was 6.9 days, with only one exception (a 17-year-old girl who had habitual constipation). The constipation in the single exception was not a specific presentation, which may have delayed the diagnosis. Pediatric patients with unexplained gastrointestinal symptoms must be diagnosed for the possibility of alimentary tract duplication, even though it rarely occurs. Ultrasonography is helpful in diagnosing a duplication, as it will identify a three-layered image representing the duplication cyst, common wall, and outer bowel wall. These features have proven useful for establishing the correct diagnosis or ruling out alimentary tract duplication where surgery was indicated anyway.

Congenital webs of the gastrointestinal tract: 20 years of experience from a pediatric care teaching hospital in taiwan.

BACKGROUND: To classify and evaluate the clinical spectrum of congenital webs in the gastrointestinal (GI) tract, including clinical courses and related factors. METHODS: A retrospective chart review was performed on 37 patients with congenital GI webs at a pediatric care teaching hospital in north Taiwan. All of the related parameters were collected and analyzed. RESULTS: Twelve patients had gastric webs, 22 had duodenal webs, and three had jejunal webs. The mean time to diagnosis was 1576 days for gastric webs, 116 days for duodenal and 230 days for jejunal webs. There was a statistically significant difference between the gastric and duodenal groups (p = 0.001). The major symptom was vomiting (78%). Patients with duodenal webs had a high association with congenital anomalies (50%). The major anomalies included cardiac (27%) and GI anomalies (18%). Endoscopy was performed in 10 gastric cases, and all of them were noted to have positive findings, including a fixed nonfolded stenotic ring following a second gastric chamber and a real pylorus. All of the patients received surgery except for three with gastric webs, and no mortality was noted. The mean postoperative days of tolerated feeding was 6 for those with gastric webs, 10 for those with duodenal and 11 for those with jejunal webs. CONCLUSION: The clinical course of gastrointestinal webs may be chronic or obscure. A delay from onset of symptoms to treatment may exist, especially in gastric webs. We suggest that prompt endoscopic confirmation and surgical intervention for these lesions, when suspected due to clinical and radiologic abnormalities, will decrease the morbidity of unexplained recurrent symptoms or signs of GI obstruction in these patients.