Colocolic intussusception due to lipoma in an adolescent with refractory anal fissure; case report and literature review.

INTRODUCTION AND IMPORTANCE: Intussusception is uncommon in older patients, making its diagnosis challenging and necessitating a high level of clinical suspicion. While pediatric intussusception typically presents with a triad of symptoms including abdominal pain, bloody diarrhea, and an abdominal mass, the majority of adult patients experience chronic abdominal pain and partial obstruction. Consequently, the diagnosis of adult intussusception may be delayed due to the similarity in presentation with other conditions. CASE PRESENTATION: In this article, we have presented a 13-year-old boy with chronic and refractory anal fissure. The patients also complained of constipation for a year, intermittent abdominal pain, and bloating. Although he was treated with conservative laxative medications, the constipation was not relieved. Incidentally, a colocolic intussusception was found through an MRI. CLINICAL DISCUSSION: We have provided a comprehensive description of an unexpected intussusception at an uncommon age which was found incidental. Medical literature was reviewed for better optimal planning in surgery. CONCLUSION: Intussusception in a teenager is unexpected, and this case shows the importance of considering it even in the presence of nonspecific symptoms. This case serves as a reminder to healthcare professionals to consider intussusception as a potential diagnosis in similar cases.

A mobile application for postoperative education of caregivers of children with congenital hypospadias: Requirement analysis.

Background and Aims: Hypospadias, a congenital anomaly, can have long-term effects on sexual, urinary, and reproductive functions, making proper postoperative care essential for desirable outcomes, which could be facilitated through a mobile application for diseases with long-term complications. The aim of this study was to investigate the data and functional requirements or minimum data set of a postoperative education mobile application for caregivers of children with hypospadias. Methods: A literature review of papers published until April 2023 using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement was conducted to determine the data and functional requirements of a mobile application that provides postoperative education to caregivers of children with congenital hypospadias. Based on the results, a questionnaire was prepared, and its content validity and reliability were evaluated by CVI and CVR. Additionally, data was examined by 30 residents, specialists, and subspecialists in pediatric surgery using the Delphi approach. Results: The study identified 28 data elements in three main categories: demographic data, clinical data, and application function. Functional requirements of the mobile application were suggested for use in designing the application. Also, the most critical data elements included the definition of disease, the importance of treatment, surgical preparation, bandage, hygiene, symptoms and infection, bleeding, and emergency condition. Conclusion: The study will pave the way for developing postoperative educational applications for caregivers of children with congenital hypospadias. M-Health app developers and clinician specialists can utilize these findings to design practical applications that assist caregivers in managing the care of hypospadias patients.

Dumble cystic lymphangioma as an underlying cause of vague abdominal complaints in a 2-year-old girl: case report.

Lymphatic malformations (LM) refer to very rare hamartomatous benign lymphatic ectasias with an incidence of 1:250 000. They almost involve children more than adults, and among them, infants under one age are mostly affected. Macrocytic LM is found to be more than 2 cm in diameter or 2×2 cm2 in volume. The proper treatment for mesenteric LM is complete surgical excision unless there is vital structure involvement. Case presentation: The authors report a mesenteric macrocytic LM in a 2-year-old girl complaining of vague abdominal discomfort and persistent vomiting in which ultrasonography revealed a cystic masse with seromucous components. She then underwent exploratory laparotomy. The operation and the follow-up duration were uneventful. Discussion: LMs are rare benign lesions of vascular origin with lymphatic differentiation, according to the latest International Society for the Study of Vascular Anomalies (ISSVA 2018). Under light microscopy, these malformations are characterized by their thin-walled endothelium and lymphatic tissue.These mobile lesions are incidentally found or appear with intestinal obstruction or acute abdomen scenarios. Conclusion: Although benign, the LMs have the potential for invasion and recurrence. Thus, the examiner physician must keep such intra-abdominal lesions in mind.

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.

Exacerbation of Congenital Hydronephrosis as the First Presentation of COVID-19 Infection in Children.

Background: Congenital hydronephrosis is one of the most common abnormalities of the upper urinary tract, which can be exacerbated by a variety of intrinsic or extrinsic triggers. The urinary tract system is one of the major organs complicated by COVID-19 infection. Case Presentations. Here, we report five patients with an established diagnosis of congenital hydronephrosis, who presented with acute abdominal pain and fever and an abrupt increase in the anteroposterior pelvic diameter (APD). Patients had a previous stable course and were under regular follow-up with serial ultrasonographic studies. They underwent surgery or supportive treatment due to the later exacerbation of hydronephrosis. Based on the clinical and imaging findings, no plausible etiologies for these exacerbation episodes, including infection, nephrolithiasis, or abdominal masses, could be postulated. The common aspect in all these patients was the evidence of a COVID-19 infection. Conclusions: Infection with COVID-19 in children with antenatal hydronephrosis may exacerbate the degree of hydronephrosis and renal APD in ultrasonography, which itself may be mediated by the increase in inflammatory mediators.

Electrochemical measuring of reactive oxygen species levels in the blood to detect ratio of high-density neutrophils, suitable to alarm presence of cancer in suspicious cases.

Here for the first time, a real-time electrochemical assay on unprocessed blood was designed to detect the presence of cancer in patients. The system has been based on the recently approved pathway, which indicates that the abundance of immature and mature low-density neutrophils (LDNs) with reduced ROS production in peripheral blood is increased with the presence of active cancer tumors. Reduced ROS/H2O2 released from LDNs play the main role in determining the ROS/H2O2 levels of peripheral blood. In contrast, HDNs with increased levels of released ROS/H2O2 have higher concentrations than LDNs in normal cases. Hence, the reduced level of ROS species in peripheral blood recorded by our carbon nanostructure decorated sensor in less than 30 seconds showed a great pre-warning about the presence of non-treated cancer in patients with suspicious mass who have been sent for further evaluations.

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.

Combination of different clinical reasoning tests in a national exam.

INTRODUCTION: Clinical reasoning as a critical and high level of clinical competency should be acquired during medical education, and medical educators should attempt to assess this ability in medical students. Nowadays, there are several ways to evaluate medical students' clinical reasoning ability in different countries worldwide. There are some well-known clinical reasoning tests such as Key Feature (KF), Clinical Reasoning Problem (CRP), Script Concordance Test (SCT), and Comprehensive Integrative Puzzle (CIP). Each of these tests has its advantages and disadvantages. In this study, we evaluated the reliability of combination of clinical reasoning tests SCT, KF, CIP, and CRP in one national exam and the correlation between the subtest scores of these tests together with the total score of the exam. METHODS: In this cross sectional study, a total number of 339 high ranked medical students from 60 medical schools in Iran participated in a national exam named "Medical Olympiad". The ninth Medical Olympiad was held in Shahid Beheshti University of Medical Sciences, Tehran, Iran, under the direct supervision of the Ministry of Health and Medical Education in summer 2017. The expert group designed a combination of four types of clinical reasoning tests to assess both analytical and non-analytical clinical reasoning. Mean scores of SCT, CRP, KF, and CIP were measured using descriptive statistics. Reliability was calculated for each test and the combination of tests using Cronbach's alpha. Spearman's correlation coefficient was used to evaluate the correlation between the score of each subtest and the total score. SPSS version 21 was used for data analysis and the level of significance was considered <0.05. RESULTS: The reliability of the combination of tests was 0.815. The reliability of KF was 0.81 and 0.76, 0.80, and 0.92 for SCT, CRP, and CIP, respectively. The mean total score was 169.921±41.54 from 240. All correlations between each clinical reasoning test and total score were significant (P<0.001). The highest correlation (0.887) was seen between CIP score and total score. CONCLUSION: The study showed that combining different clinical reasoning tests can be a reliable way of measuring this ability.

Thoracotomy versus Video-Assisted Thoracoscopy in Pediatric Empyema.

BACKGROUND: To compare the outcomes of video-assisted thoracoscopic surgery (VATS) in comparison to open thoracic surgery in pediatric patients suffering from empyema. METHODS: A prospective study was carried out in 80 patients referred to the Department of Pediatric Surgery between 2015 and 2018. The patients were randomly divided into thoracotomy and VATS groups (groups I and II, respectively). Forty patients were in the thoracotomy group (16 males [40%], 24 females [60%]; average age, 5.77±4.08 years) and 40 patients were in the VATS group (18 males [45%], 22 females [55%]; average age, 6.27±3.67 years). There were no significant differences in age (p=0.61) or sex (p=0.26). Routine preliminary workups for all patients were ordered, and the patients were followed up for 90 days at regular intervals. RESULTS: The average length of hospital stay (16.28±7.83 days vs. 15.83±9.44 days, p=0.04) and the duration of treatment needed for pain relief (10 days vs. 5 days, p=0.004) were longer in the thoracotomy group than in the VATS group. Thoracotomy patients had surgical wound infections in 27.3% of cases, whereas no cases of infection were reported in the VATS group (p=0.04). CONCLUSION: Our results indicate that VATS was not only less invasive than thoracotomy, but also showed promising results, such as an earlier discharge from the hospital and fewer postoperative complications.

Abdominal Mass Secondary to Human Toxocariasis.

Toxocariasis is an extensive helminthic infection that leads to visceral larva migrans in humans. A 2.5-year-old girl referred for abdominal mass. She had history of pharyngitis for two weeks. There were no other symptoms. Abdominal examination revealed an irregular solid mass in right lower quadrant (RLQ). Abdominal ultrasonography revealed an echohetrogenic large mass in RLQ, liver, and retroperitoneal area. Abdominal CT scan showed a huge mass. At laparotomy a large retroperitoneal mass that involved right liver lobe, bladder, ileocecal valve, small and large intestines was found. At histopathology diagnosis of toxocariasis was made.

Complete Urethral Duplication in Children: A Case Report.

INTRODUCTION: Urethral duplication (UD) is a rare congenital anomaly with multiple anatomical variants. CASE PRESENTATION: In this article we present a four year-old child with complete UD. The patient was admitted for hypospadias repair, in evaluation we found type IIA1 UD according to Effmann classification. Patient underwent hypospadias repair saving complete UD. CONCLUSIONS: After one year follow-up he has normal and continent urination.

Evaluation of Onlay Island Flap Technique in Shallow Urethral Plate Hypospadiasis.

BACKGROUND: Hypospadias is one of the most common congenital genital anomalies in males that necessitates to be operated early in infancy (when 6 to 9 months old). On the other hand, hypospadias is a challenging field of pediatric urology with multiple reconstruction techniques. A perfect hypospadias repair is supposed to return urethral continuity with sufficient caliber, eradicate phallus curvature, and supply an acceptable appearance with low complications. OBJECTIVES: This study aimed to evaluate the outcomes of using onlay island flap technique in the repair of hypospadias with shallow urethral plate. PATIENTS AND METHODS: In this prospective study within June 2012 to December 2013, we performed onlay island flap procedure to repair hypospadias with shallow urethral plate measuring less than 6 millimeter. This technique was selected for all types of hypospadiasis except subcoronal type. Nesbit's dorsal plication procedure was established for chordee. In cases with very small glans, urethroplasty was performed without glansplasty. RESULTS: Twenty three patients with mean age of 30 (range 10 - 60) months underwent onlay island flap repair; all had a shallow urethral plate < 6 mm, 3 had a very small glans, and 18 had chordee. Meatus was located in distal shaft in 5 cases, mid shaft in 8, proximal in 6 and penoscrotal type in 4 patients. Chordee was corrected with Nesbit's dorsal plication in 16 cases. Complications were: meatal stenosis in 2 cases and urethrocutaneous fistula in 2 patients, all of which were repaired surgically. Mean follow up time was 13 (3 - 20) months. All cases that had glansplasty have excellent esthetic appearance. CONCLUSIONS: This technique offers acceptable results regarding meatal stenosis, urethrocutaneous fistula and esthetic outcome.

Ileocecal patch -low rectal anastomosis in total colectomy: New idea for the prevention of fecal incontinence.

BACKGROUND: Total colectomy is used in children with total colonic aganglionosis, Ulcerative colitis (UC) and familial adenomatous polyposis (FAP). The purpose of this study was to maintain ileocecal valve and rectal-sparing surgery for the prevention of fecal incontinence in these children. METHODS: From1990 to 2011, 14 children with diagnosis of UC, FAP and Hirschsprung's disease were operated. Total colectomy was done with the preservation of patch of cecum with ileocecal valve and half of the rectum with ileocecorectal anastomosis. Distal ileum designed as S shape pouch and ileocecal valve were preserved. In Hirschsprung's disease, posterior rectal myotomy was established. The data were collected and analyzed. RESULTS: The mean age of the patients was 54 months (ranged from 2 months to 18 years). Ten patients were male. Among 14 patients, Hirschsprung's disease, ulcerative colitis and FAP were seen in 10, 3, and one case, respectively. They were followed up annually. Clinical and endoscopic examinations were performed to evaluate the function of ileocecorectal anastomosis. They followed from 2 to 24 years. At first year, the patients experienced four to six bowel movements during the day and one at night. This frequency decreased over time. The main postoperative complications included recurrent enterocolitis (n=2), perianal fistula (n=2). Only 2 patients were suffering from some degree of fecal soiling. CONCLUSION: The results show that the Ileocecal patch- low rectal anastomosis in total colectomy leads to low complications and prevent fecal frequency and incontinence. It also increases absorptive function of ileum in children.

Pediatric Germ Cell Tumors; A 10-year Experience.

OBJECTIVE: The aim of this study was to evaluate the outcome of germ cell tumors in patients admitted to our center during a ten year period. METHODS: In a retrospective descriptive study, patients with the pathological diagnosis of germ cell tumor (GCT) were included. All records were evaluated and patients followed by personal visit in clinic or phone call. Data regarding age, sex, tumor site, bio-chemical assay, pathology, treatment and outcomes were gathered. For qualitative variables we computed frequency and percentage and for quantitative variables, mean and standard deviation. Survival analysis was performed using Kaplan-Meier. All statistical analyses were performed by SPSS version16.0. Findings : Forty four patients consisted of 32 girls (72.7%) and 12 boys (27.3%). Their median age was 23 months. The most common pathological tumor types were 18 (40.9%) mature teratomas and 14 (31.8%) yolk sac tumors. Extra gonadal tumors were more prevalent (32 cases) and consisted of 21 (47.7%) sacrcoccygeal, 7 (15.9%) retroperitoneal, 2 (4.4%) mediastinal and 2 (4.4%) cervical tumors. In gonadal tumors 9 patients had ovarian and 3 patients testicular involvement. Staging at the time of diagnosis revealed stage one in 23 (52.3%) cases. All patients were treated surgically and the most common procedure was total resection in 41 (93.2%) patients. Fifteen (34.1%) patients received chemotherapy. In follow-up 31 (77.5%) patients were in complete remission, 9 (22.5%) had died, and 4 cases did not appear to follow-up visits. The median survival was 16 months (IQR 4-49 months). The highest mortality rate was found in patients with yolk sac tumors (8 of 13 cases). CONCLUSION: The patients with extra-gonadal GCT and a high AFP level have the worst prognosis and lower survival rate. Combination of surgery and chemotherapy can lead to a better prognosis.

State's pull-through for total colonic aganglionosis and GI dismotility.

BACKGROUND: Total colonic aganglionosis (TCA) is present in 4% - 5% of the patients with Hirschsprung's disease and has a high surgical mortality rate between 13% - 23%. Diagnosis and treatment of TCA is still a major challenge for pediatric surgeons. Many techniques with several advantages and disadvantages were established for its treatment. We have performed State's pull-through as total colectomy and ileo-proctostomy with long posterior rectal myotomy in TCA and severe dysmotility disorders. METHODS: In this retrospective study, 13 patients with TCA were evaluated from 1992 through 2012 in two pediatric surgery centers, which consisted of 10 TCA, one intestinal neuronal dysplasia (IND), and two chronic intestinal pseudo- obstruction syndrome (CIP). All patients underwent total colectomy, resection of part of the involved small intestine, and rectal anastomosis in one layer with 4/0 vicryl with long posterior rectal myotomy. All patients had preoperative barium enema and rectal biopsy for diagnosis. Leveling ileostomy was performed in 12 patients and mid-jejunostomy in one patient. In two of 13 children, proximal diverting loop ileostomy was established after definitive operation. In three patients, trans-rectal myotomy was needed two weeks after the initial operation. RESULTS: All patients were females with the age ranging from six months to six years. State's pull-through was carried out for all (the mean age of definitive procedure was 5.1 months). Rectal biopsy of 10 patients reported no ganglion cells of which one had extended aganglionosis to mid- jejunum, one had IND, and two had ganglionicbowel bowel with clinical presentation of CIP. The follow- up period was from six months to 10 years (the mean follow- up period was 10 months). There were no significant complications in 12 patients except episodes of diarrhea and severe dehydration which needed hospitalization. One patient with mid- jejunum aganglionosis had severe failure to thrive and needed repeated hospitalization for parenteral nutrition. All patients had acceptable bowel function following operation (two to six times a day). Now, five of the 13 patients are above the age of toilet training, having voluntary bowel movement with little or no medication (Leopromid). CONCLUSION: State's pull-through is recommended in all cases of TCA and severe dysmotility problems of the colon. This technique is less difficult to perform, and avoids the complications and disadvantages of removal of the rectum and has satisfactory results.

Protective temporary vesicostomy for upper urinary tract problems in children: a five-year experience.

OBJECTIVE: Temporary vesicostomy is a urinary diversion procedure for patients with upper urinary tract (UUT) dilatation, secondary to bladder outlet obstruction or dysfunction. The aim of this study was to evaluate our experience in children undergoing such diversion, analyzing its efficacy to prevent urinary tract infection (UTI), improve or resolve hydronephrosis, stabilize or improve kidney function and restore the health of UUT. METHODS: In this retrospective study, patients who had vesicostomy by Blocksom technique due to bladder outlet obstruction or dysfunction were evaluated in Mofid Children's Hospital (in Tehran) from March 2007 to March 2012. The reason for applying this procedure was failure in clinical treatment. Data regarding gender, age, diagnosis, time of any surgical intervention, associated anomalies, primary/secondary complications and mortality were collected using a questionnaire, and evaluated by giving a grade that ranged from 0 (worst) to 10 (best) based on Lickert's scale. FINDINGS: From a total number of 53 patients, (88.7% male and 11.3% female) with a mean age of 225 days, 66% had posterior urethral valve and 16 (30%) neurogenic bladder. UTI was present in all cases, hydronephrosis in 52 (98.1%), and vesico-ureteral reflux only in 45 (84.9%) patients. Valve ablation was performed in 17 cases, and clean intermittent catheterization in14 patients which were unsuccessful. We performed vesicostomy in all patients. Mortality rate was 7.5%. Vesicostomy was closed in 35 patients. Cure rate was 85% in UTI, 82.7% in hydronephrosis, 80% in VUR, and 86.5% in kidney function. CONCLUSION: Vesicostomy is a simple procedure that protects upper urinary tract, decreases hydronephrosis, and improves kidney function. The procedure is well tolerated and reversible, with less complication and should be considered in children in whom conservative and medical treatment has failed.

Can We Rely on Frozen Sections of a Rectal Biopsy for One-stage Trans-anal Pull-through Operation in Hirschsprung's Disease?

OBJECTIVE: Successful results after one-stage trans-anal pull-through (OSTAPT) operation for Hirschsprung's disease (HD) depend on accurate identification of the aganglionic segment in intra-operative frozen section (FS). Misinterpretation of the findings of the rectal biopsy is an anxiety-evoking pitfall for the surgeon. This study aims to describe our experiences in comparing results of FS and permanent Section (PS) rectal biopsies in children with HD who were candidates for OSTAPT in a single-step operation. METHODS: Subjects under the age of 14 years, admitted from March 2000 to July 2008 in a university-affiliated children's hospital for open rectal biopsy to diagnose HD were included in the study. All biopsies were taken 2-3 cm above the dentate line. 210 specimens of full-thickness rectal biopsy were obtained for both frozen section and permanent biopsy from all patients, examined by two well experienced pediatric pathologists for ascertaining the presence of ganglion cells, and the results were compared. Analysis was performed by SPSS Software version 11.5. FINDINGS: Two-hundred one infants and children underwent FS rectal biopsy to exclude HD. Positive results were seen in 63.8% of the specimens examined as PS and in 58.3% of FS samples. 93.9% of positive results in FS studies were confirmed by PS studies. 6.1% of FS reports were false positive and 21.7% were false negative (P<0.001). The sensitivity of FS was 85.8% and specificity 90.2%. Positive predictive value (PPV) was 93.9% and negative predictive value (NPV) was 78.3% in FS studies (P<0.001). The accuracy of FS was 80.4%. CONCLUSION: Although FS of the rectal biopsy is useful in defining the aganglionic segment during operation, according to this study, it cannot be used as the sole base for performing primary pull-through operation before the results of the permanent section are on hand.

Diphallus with imperforate anus and complete duplication of recto-sigmoid colon and lower urinary tract.

BACKGROUND: Diphallus is a rare anomaly and accompanying anomalies vary from bifid scrotum, bladder exstrophy, imperforate anus and colo-rectal anomaly such as duplication, and other associated anomalies. CASE PRESENTATION: A 2-day old infant is reported with imperforate anus and complete duplication of recto-sigmoid colon, rectal pouch, doubling of the genitalia with completely formed penis (diphallus), double bladder, urethra and hypospadias. No family history of abnormalities was noted. The patient underwent several operations: laparatory and colostomy at 3rd day of life, and after clinical and paraclinical investigations, cystoplasty, ureteral reimplantation and resection of left phallus were carried out when 4 months old. At the age of 1 year, after colostogram and total colon evaluation, laparatomy, resection of duplicated recto-sigmoid colon, and pull-through was carried out; 3 months later colostomy closure was performed and the patient discharged without complications. CONCLUSION: The patients with diphallus have to be examined carefully because of the high incidence of other systemic anomalies. Treatment of diphallus usually includes excision of the duplicated penile structure, its urethra, and repair of associated anomalies.