A Rare Case of Prostatic Utricle with Crossover Vas Deferens in Children.

Background: A prostatic utricle (PU) is an unusual pathology with most patients being asymptomatic. However, approximately 29% of patients may show lower urinary tract symptoms, recurrent urinary tract infections (UTI), postvoid dribbling, urethral discharge, epididymo-orchitis, stones, and secondary incontinence caused by urine trapping in the pouch and urinary retention. The standard treatment is through surgical resection, but it is only offered to patients with symptoms. Case summary: We report a case involving a six-year-old boy with congenital hypothyroidism and penoscrotal hypospadias who had previously undergone onlay urethroplasty for the proximal shaft, chordee release, orchidopexy for bilateral undescended testis, and laparoscopic herniorrhaphy for left inguinal hernia. However, the patient later evolved the repetition of UTI and right epididymo-orchitis. Cyclic voiding cystourethrography confirmed the presence of a cystic lesion communicating with the prostatic urethra from the utricle. The PU was then excised laparoscopically. The utricle was identified posterior to the bladder, and insertions of the vas deferens crossover into the utricle were detected by laparoscopy. The post-procedure course was uneventful. Conclusions: Laparoscopic resection of PUs offers a better exposure field, improved wound appearance, complete resection, and reduces the incidence of complications. During laparoscopy, the PU was clearly distinguished from the bladder or other pelvic organs. An incidental finding of vas deferens crossover has rarely been reported. A combined cystoscopy and laparoscopy for PU resection is executable, safe, and valid in this patient population.

A nationwide survey of incidence rates and risk factors of inguinal hernia in preterm children.

PURPOSE: Clinical observations showed a higher incidence rate of inguinal hernia (IH) in preterm infants. In this study, we calculated the incidence rate of preterm IH from the National Health Insurance Research Database (NHIRD) of Taiwan. METHODS: From NHIRD, 92,308 subjects born in the year of 1997-2005 were randomly selected as the study cohort. The medical claims of these subjects from birth to 6th year of age were analyzed. Preterm births were defined using ICD code 765.1*. Risk factors such as birth weight, lung disorders, and ventilator supports before IH repairs were investigated. The risk of incarceration and bowel resection were also evaluated. RESULTS: From 92,308 subjects, 2560 preterm births were identified. IH was repaired in 231 preterm (9.02%) and 3650 term subjects (4.07%). Male (preterm 13.3% and non-preterm 6.3%) had more hernia repairs than female (preterm 3.8% and non-preterm 1.6%). The incidence rate of IH is 13.7% for those under 1500 g, 8.2% for those 1500-1999 g, 7.7% for those 2000-2499 g, and 6.3% for those above 2500 g. The incidence rate of IH in preterms with past history of lung disorders and ventilation supports is 8.7 and 13.6%, respectively. There was no significant difference in the incidences of incarceration and bowel resection between preterms and non-preterms. CONCLUSIONS: Birth weight under 2500 g is a significant risk factor for IH repairs. Other risk factors are male gender, past history of lung diseases, and ventilator supports.

A newly designed total implantable venous access device in rats for research with high efficiency and low cost.

BACKGROUND: In this study, we introduced a newly designed totally implantable device for long-term vascular access in rats and compared its efficacy, related complications, and cost-effectiveness with conventional exteriorized jugular vein catheters. METHODS: Forty adult male Sprague-Dawley rats, weighing 250-300 g, were equally divided into two groups (I and II) and all underwent jugular vein catheterization surgery. The totally implanted device was used in group I and conventional exteriorized catheters were used in group II. The functionality of each catheter was checked every 3 d and evaluation included vascular accessibility, patency, and infection. The weight of the animal and microbial culture from the wound and tube were also monitored. We analyzed the cause of vascular access failure and complications, both mechanical and infectious, and compared related variables. RESULTS: The proportions of 9-d patency and 30-d patency in group I were 90% (18/20) and 75% (15/20), respectively, and in group II 80% (16/20) and 35% (7/20), respectively. There was a statistically significant difference in 30-d patency. The rats in group II were more liable to involve vascular access failure because of catheter dislodgment and had a higher infection rate (P = 0.001). Daily body weight gain was also greater in group I than in group II (2.46 ± 0.59 g/d versus 1.84 ± 0.96 g/d; P = 0.02). CONCLUSIONS: This newly designed and totally implanted device substantially increases the success rate of long-term venous access compared with conventional methods. It reinforces the merits of the subcutaneous port and a tethered swivel system and overall has better performance and reliability. Furthermore, given its low cost and the high level of effectiveness offered, this technology could be a powerful tool to be used in future translational medicine research, especially in cases of long-term intravascular administration.

Long-term complications and outcomes of augmentation cystoplasty in children with neurogenic bladder.

Augmentation cystoplasty (AC) is an effective surgical procedure for patients with neurogenic bladder whenever conservative treatments have failed. The present study aimed to determine the risks of metabolic complications, malignancy, long-term outcomes and histopathologic changes of native bladder and the augmented intestine after AC in children with neurogenic bladder. Pediatric patients < 18 years who underwent AC between 2000 and 2020 were enrolled. Early postoperative complications, long-term outcomes and histopathologic changes in mucosal biopsies of native bladder and the augmented intestine after AC were reviewed. Twenty-two patients with a mean age of 7.6 ± 4.4 years were included. The ileum was used in 19 patients and the sigmoid colon in 3 patients. The length of hospital stay was 14.8 ± 6.8 days. Post-operatively, the urinary continence rate improved from 22.7 to 81.8% (p < 0.001). Hydronephrosis resolved in 17 of 19 patients. Vesicoureteral reflux resolved in 16 (64.0%) of the refluxing ureter units and was downgraded in 7 (28.0%). Grades of hydronephrosis and reflux significantly improved following AC (p < 0.001). The estimated glomerular filtration rate also significantly increased (p = 0.012). Formation of urinary tract stones was the most frequent late complication (in 8 patients, 36.4%). Life-threatening spontaneous bladder perforation occurred in 1 patient. After a mean follow-up of 13.4 ± 5.9 years, there were no cases of mortality, new-onset symptomatic metabolic acidosis, or changes in serum electrolytes. Of the 17 patients who were followed for > 10 years, no cases of malignancy or metaplastic changes were identified in the native bladder or augmented bowel epithelium. AC is a safe and effective procedure with low surgical and metabolic complication rates. In addition, AC provides a satisfactory continence rate and long-term protection of renal function, increases functional capacity, and regresses reflux and hydronephrosis. Individualized surveillance is recommended for the early identification of urolithiasis and metabolic disturbances.

Comparing Outcomes of Single-Incision Laparoscopic Herniorrhaphy in Newborns and Infants.

BACKGROUND: As surgical techniques progress, laparoscopic herniorrhaphy is now performed more often in premature babies. The aim of this study was to analyze the outcomes of newborns and infants who underwent single-incision laparoscopic herniorrhaphy (SILH) at our center. METHODS: We retrospectively reviewed patients younger than 12 months old who received SILH at our department from 2016 to 2020. SILH involved a 5 mm 30-degree scope and 3 mm instruments with a 3-0 Silk purse-string intracorporeal suture for closure of the internal ring. At the time of surgery, Group 1 newborns, whose corrected age was 2 months and below, were compared to the Group 2 infants, whose age was above 2 months. We assessed the patients' characteristics, anesthesia, surgical data, and complications. RESULTS: A total of 197 patients were included (114 newborns in Group 1 and 83 infants in Group 2). The mean age and body weight in Group 1 were 1.2 months and 3.8 kg, respectively, whereas in Group 2, they were 3.2 months and 6.7 kg, respectively. There were no significant differences in operative time (Group 1 = 34.1 min vs. Group 2 = 32.3 min, p = 0.26), anesthetic time (Group 1 = 80.0 min vs. Group 2 = 76.3 min, p = 0.07), length of hospitalization (Group 1 = 2.3 days vs. Group 2 = 2.4 days, p = 0.88), postoperative complications including omphalitis (Group 1 = 5.3% vs. Group 2 = 1.2%, p = 0.13), wound infection (Group 1 = 0.9% vs. Group 2 = 1.2%, p = 0.81), and hydrocele (Group 1 = 0.35% vs. Group 2 = 8.4%, p = 0.14). No recurrence, testicular ascent or atrophy, or mortality was observed in either group during the 2-year follow-up period. CONCLUSIONS: Single-incision laparoscopic herniorrhaphy is a safe and effective operation for inguinal hernia repair in infants, even those with prematurity, lower body weight at the time of surgery, or cardiac and/or pulmonary comorbidities. Comparable results revealed no significant differences in perioperative complications despite younger ages and lower body weights.

Clinical characteristics, nocturnal antidiuretic hormone levels, and responsiveness to DDAVP of school children with primary nocturnal enuresis.

PURPOSE: Decreased nocturnal antidiuretic hormone (ADH) excretion has been suggested to be a causative factor for PNE in children. We investigate the demographic characteristics and nocturnal ADH levels of children with PNE who attended a tertiary referral center and to determine their response to treatment with desamino-D-arginine vasopressin (DDAVP). METHODS: We performed a retrospective study in 90 PNE children aged 6-12 years. We recorded the gender, height, weight, number of children per family, and psychosocial problems and compared these findings with the corresponding data obtained from a national survey. We also measured the nocturnal ADH levels and evaluated the response rate to DDAVP. RESULTS: The number of PNE patients decreased with an increase in age. Enuresis was significantly associated with male gender (P = 0.044) and more number of children per family (P = 0.043). The rates of comorbidity with defecation problems, obesity, attention-deficit hyperactivity disorder (ADHD), and overweight were 36.7, 17.8, 12.2, and 10%, respectively. Although the prevalence of obesity and ADHD was higher among children with PNE, there was no significant difference between PNE patients and their prevalence in the community. The ADH levels at 2 a.m. and 8 a.m. were 0.87 ± 0.75 and 0.89 ± 0.76 pg/ml, respectively. In the 50 (55.5%) patients who received DDAVP treatment, the complete- and partial response rates were 86 and 14%, respectively. CONCLUSIONS: Our data confirmed that PNE was predominant in boys and larger family, and similar to the findings for disease prevalence, the number of children seeking treatment tended to decrease with increasing age. Low ADH levels were recognized as a possible cause of PNE, thereby explaining the good response to DDAVP treatment in Taiwanese children with PNE.

Splenic Torsion in Heterotaxy Syndrome with Left Isomerism: A Case Report and Literature Review.

Splenic torsion is an unusual condition that results in congenital abnormality, especially in the visceral abnormal arrangement. We report the case of an 8.5-year-old boy with features in the right upper quadrant. Radiological investigations revealed heterotaxy syndrome with polysplenia and a hypodense tumor in the right upper quadrant adjacent to several spleens. We initially treated it as an intra-abdominal tumor. Laparoscopy was performed to check the tumor condition and revealed a congestive tumor located in the abdomen of the right upper quadrant below the central liver, which was suspected to be a torsion spleen without attaching ligaments. Laparoscopic splenectomy was successfully carried out without complications. The pathological report shows splenic tissue with hemorrhagic infarction. Physicians should be vigilant of the differential diagnosis of the acute abdomen in adolescents.

Analysis of the RET gene in subjects with sporadic Hirschsprung's disease.

BACKGROUND: Hirschsprung's disease (HSCR), or aganglionic megacolon, is a hereditable disease of the enteric nervous system. It is an embryonic developmental disorder characterized by the absence of ganglion cells in the lower enteric plexus. Gut motility is compromised in HSCR, with consequent risk of intestinal obstruction. METHODS: We sequenced the RET gene and characterized the clinical manifestations in 15 unrelated Chinese patients (9 males, 6 females; age range, 2-21 years) with sporadic HSCR. Genomic DNA extraction, PCR and DNA sequence analysis were performed according to standard procedures. RESULTS: We identified heterozygous RET gene mutations in 2 patients. The mutations included a missense mutation in exon 2 (CGC --> CAC) resulting in a substitution of arginine by histidine at codon 67 (patient 1), and a missense mutation in exon 3 (TAC --> AAC) resulting in a substitution of tyrosine by asparagine at codon 146 (patient 2). The pathological findings disclosed short-segment HSCR in patient 1 and long-segment HSCR in patient 2, respectively. CONCLUSION: We identified RET gene mutations in 2 of 15 patients with HSCR in Taiwan. The Y146N mutation we identified was novel.

Severe necrotizing fasciitis of the abdominal wall secondary to colon perforation in a child.

We discuss the successful saving of an 18-month-old boy with necrotizing fasciitis of the abdominal wall secondary to colon perforation and peritonitis. The patient underwent emergency surgery with exploratory laparotomy, repeated procedures of debridement, and reconstructive abdominal wall surgery with skin graft. He recovered 6 months after admission. We also address the issue of the closure of large abdominal wall defect and the importance of alimentation in this patient group. We discuss the need to recognize necrotizing fasciitis as a potential complication of intra-abdominal disease, as once necrotizing fasciitis occurs, mortality is more likely. We conclude that recognition and aggressive surgical debridement is mandatory.

Prepubertal testicular germ cell tumors: 25-year experience in Taipei Veterans General Hospital.

BACKGROUND: Due to the rarity of testicular tumors in the prepubertal population, adequate information about their biological course is difficult to document well in a single institution. The purpose of this study was to focus on prepubertal males in an attempt to evaluate clinical features and optimal management among various testicular germ cell tumors with long-term follow-up. METHODS: We retrospectively reviewed the records of children younger than 12 years of age with primary testicular germ cell tumors between February 1981 and December 2005 at Taipei Veterans General Hospital. Thirty-four children were diagnosed with adequate clinical and pathologic data. The stage of the disease was determined according to the staging system used by the Children's Oncology Group. Mean follow-up time was 139 months (range, 2-283 months). RESULTS: All of the 34 prepubertal patients were diagnosed initially with a painless scrotal mass. The mean age of the patients at diagnosis ranged from 6 months to 84 months (mean, 20.5 months). All patients underwent radical orchiectomy as an initial treatment. Twenty-nine (85.3%) patients had yolk sac tumors, and 5 (14.7%) had mature teratomas. Of the 29 patients with yolk sac tumor, 26 (89.7%) were diagnosed as stage I, 1 (3.4%) as stage III, and 2 (7.0%) as stage IV. Five (19.2%) of the 26 stage I yolk sac tumors progressed to metastasis after radical orchiectomy, and all of these 5 patients later received chemotherapy. One patient initially with stage III yolk sac tumor and 2 patients with stage IV yolk sac tumor were also treated with chemotherapy. Eventually, 1 patient with stage IV yolk sac tumor died due to tumor progression; the remaining 28 patients with yolk sac tumor all survived without tumor relapse after appropriate treatment. In the 5 patients with teratomas, there was no tumor relapse after radical orchiectomy with a mean follow-up time of 139.1 months. The 5-year survival rates for yolk sac tumor and teratomas were 96.5% and 100%, respectively. CONCLUSION: The most common prepubertal malignant testicular tumor is yolk sac tumor, and the most common benign testicular tumor is teratoma. Children with testicular germ cell tumors have excellent long-term survival rates after appropriate treatment.

Renal cell carcinoma in children and young adults.

Renal cell carcinoma (RCC) is a relatively uncommon tumor in childhood. Its biologic behavior and prognostic factors have rarely been documented. We report treatment and survival of 4 children (aged 8, 9, 11, and 14 years) who had RCC, along with a review of the literature to analyze the frequency of major symptoms, clinical stage, and prognostic factors based on 130 published cases of RCC in individuals younger than 20 years of age. Two of our cases had renal tumors detected by ultrasound screening, and all 4 cases were followed for a considerable length of time and were alive and free of disease after treatment. An analysis of these 130 published cases of pediatric RCC showed tumor staging and cell type to be the factors that affected patient survival. Tumors composed of granular cells or mixed cells, or at advanced stages, had a poor prognosis. Age, sex, tumor size, symptom duration, and cellular pattern were not related to patient prognosis. Children older than 10 years of age, who have an abdominal mass, flank pain, and/or hematuria should alert clinicians to consider the possibility of RCC. The importance of early diagnosis of renal tumors, using ultrasound as a tool of screening, is emphasized, since surgical treatment leads to a favorable prognosis only in the early stage of RCC.

Esophageal atresia with tracheoesophageal fistula: ten years of experience in an institute.

BACKGROUND: Esophageal atresia (EA), tracheoesophageal fistula (TEF), or both is a complicated problem. The purpose of this study was to evaluate the outcomes and postoperative complications in patients with EA/TEF who were admitted to our hospital. METHODS: In total, 15 patients were enrolled from 1994 to 2003, including 8 males and 7 females. Patient demographics, associated anomalies, and outcomes were analyzed. RESULTS: The most common variant was EA with a distal TEF (type C), which occurred in 12 patients (80%). The latter had associated congenital anomalies, and cardiac anomalies were the most frequent, occurring in 8 patients (53.3%). Of the 6 cases who had life-threatening anomalies, 4 (66.7%) died, and of the 9 cases who had no life-threatening-anomalies, 2 (22.2%) died. Tracheomalacia and/or stenosis were diagnosed in 8 patients (66.7%) postoperatively. Though 3 of the 4 cases who suffered from dying spell received intratracheal stent implantation, 2 cases still died. CONCLUSION: The survival rate of the patients with EA/TEF is influenced mainly by associated life-threatening anomalies. TMS combined with a history of dying spell may be the major fatal complication.

Surgical treatment of chylothorax caused by cardiothoracic surgery in children.

Four pediatric cases of chylothorax after cardiothoracic surgery, which were managed surgically, are reviewed retrospectively. All patients underwent right thoracotomy and mass ligation of the right thoracic duct without detecting the true site of leakage. Although 1 patient died from heart failure the day after operation, the other 3 recovered quickly without sequelae. Based on our limited experience, we suggest that right thoracotomy with mass ligation of the right thoracic duct can successfully cure chylothorax on either side, particularly if identification of the site of leakage is considered too risky because of severe adhesion from previous cardiothoracic surgery.

Modified prepuce unfurling for buried penis: a report of 12 years of experience.

BACKGROUND/OBJECTIVE: About 10 years ago, we started to correct buried penis using the technique of modified prepuce unfurling. We have made modifications in the years since our preliminary results were reported in 2002. METHODS: One hundred and thirty-four patients received modified prepuce unfurling since 2000, with ages ranging from 2 months to 33 years. The surgical procedures included the removal of the narrowest part of the prepuce, dissection of the fibrotic tissue from the Buck's fascia, and unfurling the inner prepuce to cover the penis. Most patients had their procedures in day care service. No urinary catheter was needed. All patients were followed up for at least 2 months. RESULTS: Most patients had satisfactory results. All patients had the glans exposed after surgery, although one patient needed reoperation for prolonged edema and two patients had wound infections. CONCLUSION: Modified prepuce unfurling is a safe and effective method to correct buried penis.

Second hernia repairs in children-a nationwide study.

BACKGROUND: Second inguinal hernia repairs may be needed either owing to contralateral metachronous hernia (MH) or ipsilateral recurrent hernia (RH). In this study, we estimated the incidence rates of MH and RH from a large nationwide database. METHODS: The information was obtained from the National Health Insurance Database (with 23 million insurants). Subjects with hernia repairs were identified, and information such as age, gender, unilateral/bilateral procedures in the first and second hernia repairs were analyzed. RESULTS: Among the 92,308 newborns observed from their births to the end of 6th year, 3881 had first hernia repairs. Among the 3068 subjects with first unilateral repairs, 307 had second repairs, and among the 813 with first bilateral repairs, 15 had second repairs. The incidence of second repairs was 10.85% (13.71% if <1year old) after first unilateral repairs and was 1.23% (0.63% if <1year old) after first bilateral repairs. CONCLUSIONS: The incidence of RH (estimated from second hernia repairs after first bilateral repairs) was 1.23%. The incidence of MH (from second repairs after first unilateral repairs) was 9.62%. These incidence rates are consistent with other published reports.

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.

Pediatric lung abscess: a retrospective review of 23 cases.

Pulmonary abscess is a rare but critical problem in childhood. We did a retrospective review of 23 children with documented lung abscess who had been admitted and treated at the Taipei Veterans General Hospital over a 20-year period from April 1982 to April 2002. Among the 23 children, 11 cases were primary lung abscess, and 12 were secondary lung abscess. The pathogens were isolated in 16 patients (69.6%), and blood cultures yielded in only 3 patients (13.0%). The most common microorganism isolated in this series was Streptococcus pneumoniae. The 2 patients (8.7%) that died in our series had secondary lung abscess. We herein report the presenting symptoms, bacteriology, clinical management, and outcome of these 23 cases.

Hepatoblastoma and hepatocellular carcinoma in children.

BACKGROUND: After nation-wide hepatitis B vaccination in Taiwan, the authors reviewed retrospectively their experience to investigate how the histopathology type of pediatric malignant hepatic tumors changed and whether the survival rate was improved with current surgical technique and adjuvant chemotherapy. METHODS: Cases of hepatoblastoma and hepatocellular carcinoma were retrospectively analyzed and divided into 2 groups by time of diagnosis. Group I was those who received treatments during 1978 to 1990. Group II was those treated during 1991 to 2001. RESULTS: There were 20 cases of hepatoblastoma and 15 cases of hepatocellular carcinoma. Male predominance was noted in both groups (male:female, 12:8 and 9:6, respectively). The patients with hepatoblastoma were younger than those with hepatocellular carcinoma (mean/range, 9/2-36 months and 10/0.7-15 years, respectively). Cases of hepatocellular carcinoma were all hepatitis B carriers. The ratio of hepatoblastoma:hepatocellular carcinoma increased from 11:12 in group I to 9:3 in group II. Three-year survival rate of hepatoblastoma was zero in group I and 55% (5/9) in group II. Three-year survival rate of hepatocellular carcinoma was zero in both groups. Operative mortality was 9% (2/23) in group I and zero in group II. CONCLUSIONS: Since the institution of national program of universal hepatitis B vaccination, the incidence of hepatocellular carcinoma in children have declined in Taiwan. The improved survival of hepatoblastoma resulted from better surgical technique and chemotherapy.

Surgical outcomes of total colonic aganglionosis in children: a 26-year experience in a single institute.

BACKGROUND: There is a lack of consensus regarding the treatment of total colonic aganglionosis (TCA) with respect to perioperative morbidity, mortality, complications, and functional outcomes. The aim of this study was to review the results of surgical TCA treatment over a 26-year period and characterize the outcomes. METHODS: We retrospectively reviewed the clinical characteristics, surgical courses, and outcomes of TCA patients who underwent definitive pull-through operations from 1986 to 2012. Follow-up data were collected by chart reviews and telephone interviews using a standardized questionnaire. RESULTS: We identified nine infants with TCA (8.6%) from among 105 infants with Hirschsprung's disease treated during the 26-year period. Neither sex predominated (male/female ratio = 4:5). All infants underwent laparotomies and simultaneous enterostomies. All patients eventually underwent modified Duhamel pull-through procedures at a mean age of 179 days (range, 47-352 days). Two infants died of complications after surgery including heart failure and sepsis. The remaining infants recovered smoothly with antilaxative medications, and all but one was weaned off these medications. Although the surviving patients did not catch up on growth, they and their families were satisfied with the surgical results. CONCLUSION: Infants with TCA had satisfactory outcomes after the modified Duhamel pull-through operation. Based on our experience, we suggest that the pull-through operation could be performed earlier, even when there are loose stools from the enterostomy.