The outcome and complications of modern staged repair surgery in newborns with classic bladder exstrophy in different genders: A retrospective study.

INTRODUCTION: Classic bladder exstrophy (CBE) is a rare anterior midline birth defect that remains a challenge for pediatric surgeons. Despite multiple reconstructive methods, outcomes vary widely in various reports. This study aims to compare the success rate and complications of modern staged repair of exstrophy (MSRE) in each gender and compare together. METHODS: This retrospective cross-sectional study included cases of CBE between 2010 and 2020 that underwent MSRE. Short-term follow-up results, including incontinence rate, vesicoureteral reflux (VUR), urinary infections, deformed genitalia, and so on, were measured in each gender, and their differences were reported. RESULTS: Among the 40 newborns with CBE who underwent MSRE, 25 (62.5%) were boys, while the others had non-male genitalia. The rates of incontinence, VUR, dehiscence, and fistulas did not differ significantly between genders. However, chronic urinary tract infections (UTIs) were more frequent in girls, and boys were more likely to have malformed genitalia (p < .05). CONCLUSION: Our findings indicate a similar rate of complications in each gender. However, chronic UTIs and external genitalia deformities were significantly more common in girls and boys, respectively. Further large-sized controlled trials may be needed to corroborate these findings.

The effects of botulinum toxin injection on urodynamic changes in pediatric population with neurospastic bladder: First trial in Iran.

BACKGROUND: Neurogenic bladder is one of the serious, disturbing problems referred to pediatric urologic clinics. The increase in bladder pressure may damage the upper urinary tract. Anticholinergic medications have been used as the first line of complementary treatment. Regardless can be omitted, botulinum toxin (BT) was introduced as an alternative method for increasing bladder compliance. BT is a neurotoxic poison that can interfere with acetylcholine release, leading to reduced external sphincter pressure and detrusor activity. This study was established to assess urodynamic changes following BT injection among Iranian pediatric population, for the first time. METHODS: This clinical trial was conducted at Shahid Beheshti University of Medical Sciences (SBUM), Tehran, Iran, from November 2018 to January 2019 as a medical graduation dissertation. Twenty patients, previously as followings with a neurogenic bladder who met the eligibility criteria, underwent BT injection with general anesthesia using a rigid cystoscope and an endoscopic needle. Demographic data, history of anticholinergic consumption, side effects or intolerance, and the dosage of the injected BT were all recorded. The urodynamic variables during our study included: flow rate in second two, the flow time of diuresis, time of peak flow, average flow, discharged volume, maximum detrusor muscle filling pressure, maximum flow, acceleration, post-void residual volume, compliance, and cystometric bladder capacity. SPSS software version 22 was used to analyze data. The significance level was considered less than 0.05. RESULTS: Twenty patients who did not respond to anticholinergic medications or could not tolerate the side effects were entered the study. The mean age was 7.7 ±â€¯2.02 years (range 5-13), and 13 (65%) of them were male. All patients received anticholinergic medications before BT injection. Discharge volume and maximum detrusor muscle filling pressure showed the most significant changes after injection (p < 0.005). However, there was no significant effect of the baseline characteristics on post-injection improvement in urodynamic results (p > 0.05). CONCLUSION: In this study, maximum detrusor filling pressure and discharge volume were both significantly improved. These findings motivate additional studies towards selecting better indexes for defining the clinical improvement and its relation with specific urodynamic results. LEVEL OF EVIDENCE: Treatment study, level III.

Early pyeloplasty versus conservative management of severe ureteropelvic junction obstruction in asymptomatic infants.

BACKGROUND: Ureteric-pelvic junction obstruction (UPJO) is the most common cause of antenatal and neonatal hydronephrosis and its management remains controversial. While conservative management is advocated for all, this strategy puts a quarter of these patients at risk for possibly irreversible renal damage. AIM: In this study, we compare functional and anatomic outcomes in newborns and infants less than 1 year of age with high-grade unilateral UPJO, following early surgical pyeloplasty (ESP) versus conservative management (CM). MATERIALS AND METHODS: This was a single center prospective interventional study. Infants referred to our tertiary care pediatric surgery clinic between September 2016 and September 2018 with UPJO were considered. To be included patients must have been less than 1 year old, lack of clinical symptoms, suffer from severe hydronephrosis as defined by Society for Fetal Urology (SFU) grades 3 or 4, and have affected kidney Split Renal Function (SRF) above 40%. Patients with bilateral disease, structural anomalies, or an abnormal voiding cystourethrogram (VCUG) were excluded. Anatomical and functional outcomes were measured and compared at 6 and 12 months. RESULTS: Fifty-six patients were assigned to receive either ESP (n = 28) or CM (n = 28). At 6 months Cortical thickness, polar length, and SFU indices were significantly lower in the ESP group, while none of the outcomes were significantly different between the two groups at 12 months. Despite the two groups not being different at 12 months regarding differential renal function (DRF), there was a significant decrease of function in the CM group compared to baseline. CONCLUSION: When considering treatment options for infants with high-grade UPJO, it appears that ESP hastens improvement of anatomic and functional indices, while CM may lead to a significant deterioration in renal function.

An unusual cause of protein losing enteropathy in a 2.5-year-old girl: meso-intestinal fibrosis.

Introduction Protein losing enteropathy is a symptom characterized by loss of protein in intestines resulting in low protein levels in serum and generalized edema. Several causes are reported for this condition. Hereby we report an as yet unreported cause of protein losing enteropathy that we named meso-intestinal fibrosis. Case Report A 2.5-year-old girl referred with features of partial intestinal obstruction and underwent laparotomy. She had history of protein losing enteropathy since 16 months of age with generalized edema and received albumin every other week. Workup of protein losing enteropathy was inconclusive and only a histology report denoted increase in eosinophils in lamina propria of small intestine and hypoallergenic diet was started for her, but no significant response was noted. Laparotomy revealed lace-like white areas in meso of small intestine and intestinal wall was firm in palpation in some areas. Biopsy was taken from these sites and histology revealed severe fibrosis of meso overlying muscularis propria and also patchy fibrosis of intestinal meso led to severe lymphangiectasis in submucosa of small intestine. Discussion Secondary lymphangiectasis due to obstruction of lymphatic flow is mentioned as cause of protein losing enteropathy. Meso-intestinal fibrosis seen in this case that led to secondary lymphangiectasis and also motility disorder has not been reported as yet.

Acute mediastinitis in children: a nine-year experience.

BACKGROUND: Acute mediastinitis is a serious medical condition with a mortality rate of 30 to 40% or even higher. Early diagnosis with prompt and aggressive treatment is essential to prevent its rapid progression. We evaluated acute mediastinitis cases and analyzed the outcomes. MATERIALS AND METHODS: A retrospective chart review was conducted on patients diagnosed with acute mediastinitis who were admitted to Mofid Children's Hospital from January 2001 to January 2010. RESULTS: Seventeen patients aged 1 to 10 yrs. (mean =3.8 yrs) were evaluated including 12 (70%) boys and 5 (30%) girls. The most common symptoms were fever, dyspnea, cyanosis, tachycardia and tachypnea. The etiology of mediastinitis was iatrogenic esophageal perforation (EP), and related to manipulation in 13(77%), and leakage of esophageal anastomosis in 4 cases (33%). The underlying diseases were esophageal atresia in 2(12%), corrosive injury of the esophagus in 13(76%), congenital esophageal stenosis in one (6%), and gastroesophageal reflux esophagitis also in one (6%) patient. Patients with clinical symptoms were evaluated by immediate chest radiography, and gastrografin swallow. After early diagnosis, the patients received wide spectrum antibiotics and immediate mediastinal or thoracic drainage, followed by esophagostomy and gastrostomy. Only one case of endoscopic perforation was managed by NG tube. Fifteen patients (88%) survived successfully. We had 2(12%) cases of mortality in our study (one patient after esophageal substitution, mediastinal abscess and septicemia, and the other one developed esophageal perforation 6 months after early management and died of cardiac arrest during endoscopic dilation). CONCLUSION: Prevention of acute mediastinitis is still a difficult challenge. As the prognosis is not good and patients have high mortality, rapid management is mandatory.

Abdominal inflammatory myofibroblastic tumor: report on four cases and review of literature.

BACKGROUND: The Abdominal Inflammatory Myofibroblastic Tumor (AIMT) is a rare tumor with unknown etiology which usually occurs in children and adolescents. It is composed of myofibroblastic spindle cells intermixed with inflammatory cells. We present four cases of AIMT. CASES PRESENTATION: WE HEREIN PRESENT FOUR CASES OF AIMT IN DIFFERENT AGES (RANGE: 3.5 to 13 years) and in different organs (stomach, periduodenal, mesenteric, and colon). There were two females and two males. The main symptoms were abdominal pain/mass/obstruction, vomiting, and weight loss. In all four patients, diagnosis was made by laparatomy and pathologic examination of excised mass lesion. Three patients underwent complete excision and no residual disease was present, one patient received chemotherapy due to tumor recurrences. The patients were followed up in average for four years. CONCLUSION: As the imaging and laboratory tests are non-specific, the diagnosis of AIMT is rarely made before surgery. AIMT should, therefore, be considered when a mass arises in an unusual location in the pediatric age group. Complete surgical resection should be performed whenever possible and the child should be kept on long-term follow-up.

Thyroid nodules in childhood: a single institute experience.

OBJECTIVE: Thyroid nodules are rare in children. Multiple diagnostic modalities are used to evaluate the thyroid mass. The aim of this study was to determine results of management of thyroid nodules in children with special attention to the role of fine needle aspiration biopsy (FNAB) in diagnosis. METHODS: Thirty-two children who underwent surgery for thyroid nodules in Mofid Children's Hospital within 10 years (1996 to 2005) were retrospectively studied. From clinical records we obtained data about demographic characteristics, clinical manifestations, ultrasonography (USG) findings, and FNAB results, pathological reports, surgical therapy and complications. Data was analyzed statistically for association with thyroid cancer. FINDINGS: Twenty-five patients (78.1%) were girls, and 7 (21.9%) boys. Mean age was 10.9 (range 8 to 14) years. 24 (75%) patients had benign and 8 (25%) malignant tumors. 18 (56.25%) nodules were located in the right lobe. Statistical analysis revealed sensitivity, specificity, accuracy, and positive and negative predictive values as follows: 80%, 65%, 63%, 25%, and 86% for USG; 35%, 41%, 40%, 18%, and 66% for RNS; 91%, 94%, 90%, 74%, and 96% for FNAB respectively. CONCLUSION: Clinical judgment as determined by serial physical findings with USG continues to be the most important factor in the management of thyroid nodules in children. FNAB is the most accurate method of investigation and its accuracy is improved by USG guidance.