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.

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.

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.

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.

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.

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.

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.

Constipation is a major complication after posterior sagittal anorectoplasty for anorectal malformations in children.

BACKGROUND: To assess whether constipation or fecal incontinence is a major late complication after posterior sagittal anorectoplasty in patients with anorectal malformation (ARM). METHODS: We retrospectively enrolled 188 children, 85 low-type ARM (L-ARM) and 103 high-type ARM (H-ARM), who had complete medical records of bowel habits and medication histories after posterior sagittal anorectoplasty for anorectal malformation in Mackay Memorial Hospital. Stool characteristics as well as physical and medication history were evaluated. The symptom severity (SS) scoring system was used to assess changes in bowel habits. RESULTS: During a mean follow-up period of 4.3 years, constipation was found to be the most common late complication in both groups of patients (64.5% in the L-ARM group and 78.6% in the H-ARM group). Compared to constipation, stool incontinence was much less frequent, with 4.7% in L-ARM and 3.9% in H-ARM. There was no significant difference in mean SS scores between the two groups. CONCLUSION: Constipation was the most common late sequela in children after correction of ARM in our study.

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.

Cystic lymphatic malformation of the pelvis mimicking seminal vesicle cysts.

A 6-year-old boy presented with fever of 2 days of duration and had blood tests demonstrating leukocytosis and impaired liver function. Abdominal sonogram incidentally revealed 2 cystic lesions within the pelvis. Further contrast-enhanced abdominal computed tomography showed a symmetrically bilobed cystic mass between the urinary bladder and rectum in the location of the seminal vesicles. The boy underwent surgical excision, and the histopathology confirmed the mass as a cystic lymphatic malformation originating from the mesentery of the sigmoid colon.

Sonogram before and after pyloromyotomy: the pyloric ratio in infantile hypertrophic pyloric stenosis.

BACKGROUND: Sonography is used to diagnose infantile hypertrophic pyloric stenosis, but Little information is available about the appearance of postoperative sonographs. The purpose of this study was to evaluate the morphology of the pylorus in association with an obstruction before and after pyloromyotomy. METHODS: Pyloric length, diameter, muscle thickness and intermuscular space were measured sonographically at diagnosis and daily after pyloromyotomy until discharge in 12 infants with infantile hypertrophic pyloric stenosis. The ratios of pyloric wall thickness and intermuscular space to the entire pyloric diameter were measured. RESULTS: The pylorus still appeared hypertrophied after pyloromyotomy on the sonograms. The traditional measurement of linear dimensions of the pylorus was not significantly changed from the preoperative values by the time of discharge, except for muscle thickness. The intermuscular space increased from 4.8+/-0.8 mm preoperatively to 7.3+/-2.1 mm by postoperative day 3 (p=0.10). Lowe's pyloric ratio at diagnosis was a mean of 0.32, decreasing to 0.29 on postoperative day 3 and 0.29 on the day of discharge (p=0.82). The alternative pyloric ratio increased significantly by postoperative day 2 (0.24+/-0.09 on day 2 vs. 0.11+/-0.07 preoperatively, p=0.02). CONCLUSION: The pyloric ratio appears to be a reliable parameter in evaluating the regression of pyloric stenosis after pyloromyotomy, and also aids in the diagnosis of pyloric stenosis.

Neonatal gastric perforation: report of 15 cases and review of the literature.

BACKGROUND: Gastric perforation among neonates is a rare but frequently fatal condition of uncertain etiology. The aim of this study was to review the clinical course of neonatal gastric perforation and to evaluate possible prognostic factors. METHODS: We retrospectively analyzed the medical records of 15 patients with neonatal gastric perforation over a 19-year period. Another 97 patients described in the medical literature, for whom the gestational ages and birth weights were clearly stated, were also reviewed. RESULTS: In our series, there were three girls and 12 boys, nine of whom were full-term infants and six preterm infants. The most common initial manifestations were poor activity, abdominal distension, and respiratory distress. The overall mortality was 47% (7/15). Prematurity was the only statistically significant risk factor; 83% (5/6) of premature infants died compared with 22% (2/9) of term babies (p < 0.05). Combining our series with the patients reported in the literature, there were a total of 50 premature infants and 62 term infants. Gastric perforation occurred on postnatal days 2-7 and presented with nonspecific manifestations. The mortality was significantly higher in premature than in term infants (31/50, 62% vs. 16/62, 26%; p < 0.001). A trend towards higher mortality in infants with lower birth weights was observed (>2500 g, 28%; 1501-2500 g, 52%; 1000-1500 g, 60%; <1000 g, 100%). Infants with birth weights <2500 g had a significantly higher mortality than infants with birth weights >2500 g (32/58, 55% vs. 15/54, 28%; p<0.05). CONCLUSION: Neonatal gastric perforation is associated with high mortality, particularly in premature infants. There is also a trend towards higher mortality in lower-birth-weight infants.

Infected congenital splenic cyst associated with duodenal duplication cyst and malrotation--a case report.

Congenital splenic cyst is a rare entity. A potential complication of this anomaly is infection. We report on an 18-month-old boy who had Salmonella enterocolitis and 2 weeks later was found to have an infected splenic cyst. At surgery, a duodenal duplication cyst and malrotation of the gut were incidentally found. Infected splenic cysts usually occur in older children or adults. To the best of our knowledge, congenital splenic cyst has not previously been reported in association with other malformations.

Incomplete duplication of trachea with bronchogenic cyst: a case report.

Tracheal duplication is a very rare infant congenital airway anomaly. We report a bronchogenic cyst communicating with a tracheal duplication. This is the first reported case of pathologically confirmed duplication of trachea communicating with a bronchogenic cyst.