Investigating the effect of sudden occlusion of the testicular vessels on the testicular tissue in rat models.

One of the ways to treat undescended testicles is to use orchiopexy surgery, which is modified into the two-stage Fowler-Stephens technique in cases of short or immobile testes. The disadvantage of using this technique is the probability of testicular ischaemia following the sudden closure of the testicular artery; although the collateral arteries prevent atrophy, the testis loses its germ cells during this stress and may no longer be functional. Therefore, this study aims to examine the changes in testicular tissue regarding necrosis and infarction after the occlusion of the vessels. Methods and materials: In this experimental study 15 male rats weighing 200-250 g were prepared and first, one of the rats was sacrificed and testicles on both sides were used for pathology control. After general anaesthesia vascular ligature was performed with the left testes undergoing both venous and arterial occlusion and the right testes only arterial occlusion. After 1 month, all specimens were killed and the testes were completely removed and sent for histopathological evaluation. Results: A total of 14 rats and 28 testes were studied in two equal groups of case and control. There was no significant difference between the case and control groups in terms of testicular volume, consistency, and viability. Microscopic findings revealed that necrosis, infarction, and state of inflammation were significantly higher in the case group than in the control group. Conclusion: The results of this study show that abrupt closure of the testicular artery in rats is associated with necrosis and infarction, decreased spermatogenesis, and more inflammation. However, no significant differences were found in terms of macroscopic findings including volume, consistency, and viability.

Evaluating the utility of cystoscopy, distal colostography, and sonography for locating the fistula in patients with anorectal malformation: a case series.

Anorectal malformations are congenital conditions ranging from a simple perianal fistula to a complex cloacal malformation. Since the precise determination of the location of the fistula is the central pillar in choosing the type of surgery, this study aims to evaluate and compare the efficacy of three techniques, transperineal ultrasound, distal colostography, and cystoscopy. Materials and methods: This study was performed on patients with anorectal abnormalities who had undergone decompressive colostomy and were planned for anorectoplasty in the period from September 2017 to March 2019 in a pediatric surgical center. To answer our question, all three mentioned methods were conducted before the surgery and were compared with the intraoperative findings. Results: Sonography, distal colostography, and the second cystoscopy findings were similar to intraoperative conclusions concerning the presence of a fistula in patients, whereas blind cystoscopy had 30% accuracy and similarity. Regarding the type of fistula sonography, distal colostography, and second cystoscopy each had 50, 37.5, and 10 inconsistency with the intraoperative findings. In all cases where a fistula was detected in blind cystoscopy, the location of the fistula was correctly determined by this modality. Data analysis on the pouch to perineum distance measurements obtained from sonography and colostography were significantly different from that of surgery. Conclusion: The results of this study emphasize the need to perform several diagnostic modalities to determine the location and type of fistula to improve diagnostic accuracy.

Ileal atresia and total colonic hirschsprung disease in a 36-week neonate: A case report.

Intestinal atresia and hirschsprung disease are two common causes of bowel obstruction in neonates; simultaneous occurrence is rare. This report delineates a 36-week newborn with ileal atresia and total colonic hirschsprung who was referred to our unit due to failure of meconium passage during the first 48 h after birth.

A crying liver: a scan pattern mimicking spontaneous perforation of the biliary ducts.

A 2-month-old infant was referred for hepatobiliary scintigraphy due to ascites of unknown cause. The top differential diagnosis was spontaneous perforation of the biliary ducts. Delayed images up to 4 hours were against this diagnosis showing normal distribution of the radiotracer throughout the bowel. However, on delayed images, the scan showed mild tracer retention in the ascites confirmed by SPECT/CT images. Surprisingly, the exploratory abdominal surgery revealed an intact hepatobiliary system, pointing toward other possible etiologies. Second-review surgery was performed due to uncontrolled progressive ascites showing congestive hepatopathy and biliary leak from the hepatic surface suggestive of the "crying liver".

Sentinel lymph node biopsy in pediatric Wilms tumor.

INTRODUCTION: Although the sentinel lymph node Biopsy (SLNB) is well stablished in solid tumors among adults but the experience on SLNB in pediatrics is still limited. In this article we report our experience of sentinel lymph node detection that is applied on pediatric solid renal tumors. MATERIAL AND METHODS: Twenty 1-16 year old children with non-metastatic primary Wilms tumor regarding the radiological studies were enrolled. Radio tracer injection was carried out after renal vein, artery and ureter ligation, at the time of radical nephrectomy. Sentinel node detection and sampling was performed in every location with radiotracer count of 3 times more than background. Finally lymph node sampling was completed following the standard current discipline in Wilms tumor surgery. RESULTS: A single SLN was detected in 16 patients. 4 patients had more than one SLN. The most common site of SLN was inter aortocaval space. Histopathologic studies revealed tumor involvement in 3 sentinel nodes (15%). All other lymph node samples were also studied histologically and LN involvement was not detected in any of the cases with tumor free sentinel lymph node (no false negative case). Multiple LN involvement was reported in two patients with positive SLN in which, other involved lymph nodes were removed with the tumor during radical nephrectomy. CONCLUSION: Intraoperative SLNB is a safe and feasible tool to improve the accuracy of staging in pediatric Wilms' tumor. We suggest to ligate renal artery and vein prior to radiotracer injection to diminish the background confounding effect. LEVEL OF EVIDENCE: Level II (development of diagnostic criteria in a consecutive series of patients and a universally applied "gold standard") TYPE OF STUDY: Study of Diagnostic Test.

Novel Deleterious Mutation in Steroid-5α-Reductase-2 in 46, XY Disorders of Sex Development: Case Report Study.

Background: Steroid-5α-reductase-2 (SRD5A2) and 17ß-hydroxysteroid dehydrogenase type 3 (17ß-HSD3) enzyme deficiencies are frequent causes of 46, XY disorder of sex development (46, XY DSD), where an infant with 46, XY has a female phenotype. We assessed the hydroxy-steroid-17ß-dehydrogenase-3 (HSD17B3)and SRD5A2 genes in twenty Iranian phenotypic females with 46,XY DSD. Materials and methods: All exons in HSD17B3 and SRD5A2 genes were subjected to PCR amplification followed by sequencing. Results: Of 20 identified 46, XY DSD patients, one had a homozygous missense 17ß-HSD3 mutation Ser65Leu (c.194C > T). We found 1 SRD5A2 novel homozygous missense mutation of Tyr242Asp (c.891T > G) in exon 5, which in-silico analyses revealed that this mutation may have deleterious impact on ligand binding site of SRD5A2 protein. Three other individuals harbored 17ß-HSD3 deficiencies without identified mutations. Conclusions: SRD5A2 and 17ß-HSD3 mutations are found in 10% of 46, XY DSD Iranian patients.

Bioavailability of rectal acetaminophen in children following anorectal surgery.

BACKGROUND: Acetaminophen is widely used as an analgesic and antipyretic agent in pediatrics. Although bioavailability of rectal acetaminophen is unpredictable, rectal route is a usual and acceptable method of prescription. Major anorectal surgery may alter the normal structure of the surgical site, especially the vascular elements and the normal connections between port and systemic vessels. As a result the pharmacokinetics of rectal medications might also be altered. Based on this hypothesis, we decided to study acetaminophen plasma concentration among children who underwent these types of surgeries to determine the pharmacokinetic of absorption, plasma concentration, safety, and efficacy of rectal acetaminophen. MATERIALS AND METHODS: The study included 20 cases with previous history of pull-through procedure owing to Hirschsprung's disease (HD), 20 cases with imperforate anus (IA) reconstructive surgeries who were admitted for colostomy closure, and 20 otherwise healthy cases of inguinal herniotomy. Venus blood sampling was done 4, 8 and 12 hrs after a single loading dose of rectal acetaminophen (40 mg/kg), and plasma acetaminophen concentration was compared between groups. RESULTS: Mean serum acetaminophen levels of the HD group were significantly higher than those of the herniotomy group (36.3 ±â€¯6.79, 27.4 ±â€¯8.42, 16.8 ±â€¯7.62 versus 25.9 ±â€¯9.12, 16.7 ±â€¯6.74, 8.1 ±â€¯5.79 (µg/ml) at 4, 8 and 12 hrs after drug administration and P < 0.05). The IA group had higher concentrations of plasma acetaminophen compared to the herniotomy group; however, the p values were not statistically significant. (31.4 ±â€¯10.39, 21.5 ±â€¯9.12, 13.3 ±â€¯6.79 versus 25.9 ±â€¯9.12, 16.7 ±â€¯6.74, 8.1 ±â€¯5.79 (µg/ml) at 4, 8 and 12 hrs after drug administration). Serum concentrations of acetaminophen in IA and HD patients were above the therapeutic range four hours after administering the loading dose (31.4 ±â€¯10.39 and 36.3 ±â€¯6.79 versus 5-20 µg/ml). CONCLUSION: Bioavailability of rectal acetaminophen might get altered after major anorectal surgery in children. Rectal acetaminophen should be administered with special caution among infants with history of anorectal operations. Repeated dose of rectal acetaminophen may cause the drug blood concentration to reach toxic levels in these patients. TYPE OF STUDY: Prospective comparative study. LEVEL OF EVIDENCE: Level II.

Pre-operative hormone stimulation in hypospadias repair: A facilitator or a confounder.

INTRODUCTION: Preoperative hormone stimulation (PHS) is popular in hypospadias reconstructive surgery. While still controversial, it is performed to have a more developed gross anatomy of the glans and penis for improvement of the surgical condition and outcome. OBJECTIVE: In this study we assessed morphometric and histologic effects of pre-operative testosterone therapy on the penis and prepuce in patients with hypospadias. STUDY DESIGN: 18 patients with hypospadias who received 3 monthly doses of 25 mg testosterone injections were compared with 23 patients with hypospadias who were managed without pre-operative androgen therapy. Penile morphometry and hormone side effects were assessed in monthly pre-operative visits. Intra operative observations and preputial histopathology were also compared between the groups. RESULTS: Glans diameter enlarged significantly after the first dose of testosterone. (P < 0.001) while morphologic changes were not significant by further injections. 72.2% showed one or more hormone related side effects that were mostly mild. Severe side effects such as thick pubic hair growth, frequent erections or considerable penis hyper-sensation were observed in 27.8%. Glans to corpus ratio was 0.2 ± 0.75 in PHS group compare to 0.3 ± 0.17 in control group. (P < 0.001). Hemorrhage needing tourniquet placement was reported in 44.4% of the PHS patients compared to 26.1% among the control group. Histopathologic studies revealed increased vascularity and less inflammation of the prepuce in PHS group compared to controls. DISCUSSION: The glans and penis enlargement wasn't significant in 22.2% of patients who underwent PHS. We identified the first dose of testosterone as the most effective one. This finding supports the idea of close clinical monitoring to stop PHS when a significant response is observed and the expected goals are reached. Androgen side effects were not uncommon among our patients although they were mainly mild. Intraoperative assessments showed a decreased glans wing thickness to corporal body ratio in PHS group compared to controls. This finding caused more complex glanuloplasty while wrapping the glans wings over neo urethra and enlarged corporal bodies. CONCLUSION: We suggest limiting PHS to carefully selected cases considering the observed anatomical and histological changes and the side effects. Monthly monitoring during PHS is recommended to stop androgen therapy as soon as the minimum defined targets are reached. This may decrease the rate of androgen side effects while providing better surgical conditions.

Pediatric Vaginal Leech Infestation with Severe Bleeding: A Case Report and Review Article.

BACKGROUND: Vaginal leech infestation is a rare event with vaginal bleeding being its prominent sign. Few cases have been reported in young children. CASE: In this article, we present a case of vaginal leech infestation in a 2-year-old girl who presented with significant vaginal bleeding that caused severe acute anemia, prompting transfusion. On examination, she appeared anemic, but healthy, without any signs of pubertal development. The leech was seen during examination with the patient under anesthesia and was removed. With removal of the leech, bleeding decreased significantly and stopped completely after 2 hours. She was discharged the next day in good condition. SUMMARY AND CONCLUSION: The important concern in vaginal leech infestation is early diagnosis to prevent severe acute anemia and shock.

Childhood Sex-Typed Behavior and Gender Change in Individuals with 46,XY and 46,XX Disorders of Sex Development: An Iranian Multicenter Study.

Disorders of sex development (DSD) are congenital conditions in which the typical genetic and hormonal profiles are affected and thereby the usual process of sexual differentiation. Most of these studies, however, have been conducted in Western countries. In the present study, preschool sex-typed activities of Iranian individuals with DSD and their age-matched non-affected male and female relatives were assessed using the Pre-School Activities Inventory (PSAI) modified for retrospective self-report. A total of 192 individuals participated in our study, including 33 46,XX individuals with congenital adrenal hyperplasia (CAH; M age = 10.36, SD = 5.52), 15 46,XY individuals with complete androgen insensitivity syndrome (CAIS; M age = 19.8, SD = 7.14), and 16 46,XY individuals with 5-alpha reductase deficiency type-2 (5α-RD-2; M age = 17.31, SD = 7.28), as well as one age-matched non-affected male and female relative for each patient. With regard to PSAI scores, male-identifying participants with 5α-RD-2 and male controls reported similar levels of male-typical childhood play. Female-identifying participants with 5α-RD-2 and CAH showed comparable scores: significantly less masculine and more feminine than male controls, but significantly more masculine and less feminine than females with CAIS and female controls. These findings support the role of androgens in the development of sex-typical childhood play behavior, with those being exposed to higher levels of fetal functional androgens expressing more masculine behavior at preschool ages.

Prenatal testosterone and theory of mind development: Findings from disorders of sex development.

Women on average perform better than men on the "Reading the Mind in the Eyes" test (RMET) which is a measure of Theory of Mind (ToM). The aim of this study was to assess whether these sex differences are influenced by differences in prenatal testosterone levels through a study on individuals with Disorders of Sex Development and matched controls. ToM performance was examined using the RMET in female-assigned-at-birth individuals with increased prenatal testosterone exposure (Congenital Adrenal Hyperplasia (CAH) and 5-alpha Reductase type-2 Deficiency (5α-RD-2)), female-assigned-at-birth individuals with testosterone insensitivity (Complete Androgen Insensitivity Syndrome (CAIS)), and their age-matched unaffected male and female relatives. A total number of 158 individuals participated in the study; 19 with 5α-RD-2, 17 with CAH, 18 women with CAIS, 52 matched unaffected men and 52 matched unaffected women. All subgroups were around 20 years of age. Women with CAH scored significantly lower on RMET than control women and CAIS individuals. CAIS individuals scored significantly higher than control men and participants with 5α-RD. Statistically, CAIS individuals' performance on RMET was similar to control women's, women with CAH did not differ significantly from control men and 5α-RD-2 individuals scored significantly lower than control men. These results, which are in line with previous theories, illustrate that performance on the RMET, as an index of ToM, may be influenced by variations in prenatal androgens levels.

Mental Health and Disorders of Sex Development/Intersex Conditions in Iranian Culture: Congenital Adrenal Hyperplasia, 5-α Reductase Deficiency-Type 2, and Complete Androgen Insensitivity Syndrome.

Sixty-one patients (22 patients with congenital adrenal hyperplasia [CAH] with a mean age of 14.86 years [range, 5-23], 20 patients with 5-α reductase deficiency type 2 [5α-RD-2] with a mean age of 19.5 years [range, 5-29], and 19 patients with complete androgen insensitivity syndrome [CAIS] with a mean age of 18.26 years [range, 5-28]) were evaluated using the Kiddie Schedule for Affective Disorders and Schizophrenia, the Structured Clinical Interview for DSM-IV Axis I, Axis II, and the Global Assessment Functioning Scale. All participants were female-assigned at birth. Ten patients (16.4%) transitioned to the male gender. Overall, 68% of patients had one or more lifetime Axis I disorders, including 63.6% of the CAH participants, 90% of 5α-RD-2 participants, and 52.6% of the CAIS participants. The most commonly observed were affective disorders (27.9%), gender identity disorder (27.9%), and anxiety (16.4%). Our study demonstrates that mental health of Iranian patients with DSD is at risk. This might be due to the fact that patients with DSD conditions are mostly treated medically and their mental health is often superficially addressed in developing countries such as Iran, at least in the past. We argue that it is important to pay attention to the mental health issues of patients with DSD and focus on specific issues, which may vary cross-culturally.

Speech intelligibility after repair of cleft lip and palate.

Background: Intelligibility refers to understandability of speech; and lack of it can negatively affect children's overall communication effectiveness. Children with repaired cleft lip and/or cleft palate (CL/P) may experience poor speech intelligibility. This study aimed at evaluating speech intelligibility in children with repaired CL/P who had not been referred to speech-language pathology clinics for early intervention. Methods: Sixty-four monolingual Persian-speaking children, 32 children with repaired CL/P, and 32 controls aged 3 to 5 years participated in this survey. Their speech intelligibility was evaluated through the Persian Speech Intelligibility Test and was normalized on children 3 to 5 years. Each speech sample was heard by a speech and language pathologist (SLP), as well as 2 nonprofessional listeners. Two objective measures of speech intelligibility including Percentage of Consonants Correct (PCC) and Percentage of Intelligible Words (PIW) were used in this research. Results: Children with CL/P were significantly outperformed by their peers in PCC (p= 0.0001) and PIW (p= 0.0001). More than half of the case group had compensatory errors and 40.6% had obligatory errors. The PCC and the PIW were statistically different in children with different rates of hyper nasality (p= 0.001). Conclusion: Speech intelligibility of children with CL/P is impaired due to their articulation disorders (obligatory and compensatory errors). This survey documents the necessity for speech therapy for increasing speech intelligibility in this population.

Sexual orientation and medical history among Iranian people with Complete Androgen Insensitivity Syndrome and Congenital Adrenal Hyperplasia.

OBJECTIVE: To report sexual orientation, relationship status and medical history of Iranian people with Differences of Sex Development (DSD) who were raised female. METHODS: Our participants consisted of nineteen 46,XY individuals with Complete Androgen Insensitivity Syndrome (CAIS) and eighteen 46,XX individuals with Congenital Adrenal Hyperplasia (CAH) who were raised as females and older than 13years. As well as their relationship status and detailed medical history, an expert psychiatrist assessed their sexual orientation by a semi-structured psychiatric interview with them and, where applicable, their parents. RESULTS: Five percent of CAH participants and 42% of CAIS participants were in a relationship, which was significantly different. All CAH individuals had been diagnosed at birth; 89% of CAIS had been diagnosed after puberty and due to primary amenorrhea and 11% were diagnosed in childhood due to inguinal hernia. Genital reconstructive surgery had been performed in 100% of CAH participants and 37% of CAIS. Regarding sexual contact experiences and sexual fantasies (androphilic, gynephilic or both), no significant differences were found. However, CAH females had significantly more gynephilic dreams (P=0.045). CONCLUSION: This study, notable as one of the rare from a non-western culture, described sexual, medical and socioeconomic status of 46,XX CAH and 46,XY CAIS individuals living in Iran. Although broadly in line with previous findings from Western cultures, Iranian CAH individuals had fewer romantic relationships, but in contrast to previous studies their sexual orientation was only different from CAIS in the contents of sexual dreams.

Psychosexual Outcome Among Iranian Individuals With 5α-Reductase Deficiency Type 2 and Its Relationship With Parental Sexism.

INTRODUCTION: Few studies exist on the psychosexual outcome of homogeneous groups of individuals with 5α-reductase deficiency type 2 (5α-RD-2) and the relation between gender changes and parental hostile and benevolent sexism, which are two components of ambivalent sexism that assume a stereotypical approach toward women in an overtly negative way or a chivalrous, seemingly positive way. AIM: To report on the psychosexual outcome of individuals with 5α-RD-2 and to investigate its relation to the level of parental sexism in a relatively large sample of Iranians with 5α-RD-2. METHODS: Twenty participants (mean age = 19.5 years, SD = 6.345) with a molecularly confirmed diagnosis of 5α-RD-2 who were assigned the female gender at birth and raised as female were included in the study. Participants and their parents were interviewed and their medical records were assessed. Parents also completed the Ambivalent Sexism Inventory (ASI), which includes hostile and benevolent sexism subscales. MAIN OUTCOME MEASURES: Psychosexual outcome and parental hostile and benevolent sexism measurements. RESULTS: Twelve of 20 participants (60%) were diagnosed with gender identity disorder not otherwise specified (Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision). Ten of these transitioned to the male gender. The other 10 participants (50%), including the two diagnosed with gender identity disorder not otherwise specified, continued living in a female gender role. When comparing the ASI subscale scores between families of participants who changed their gender and those who did not, no significant difference was found for ASI total and hostile sexism scores, but there was a difference for benevolent sexism (P = .049): those whose daughters had changed their gender had higher benevolent sexism scores. CONCLUSION: The high prevalence of gender change and gender dysphoria reported in the literature was confirmed in this relatively large and homogeneous sample of Iranians with 5-α-RD-2 raised as female. Prenatal exposure to testosterone is hypothesized to play a role in the development of gender identity and sexual orientation, but parental attitudes also might be important. Although gender change in individuals with 5-α-RD-2 is often attributed to high levels of hostile sexism in some cultures, our findings show this to be associated with benevolent sexism.

Thoracoscopic Esophageal Atresia with Tracheoesophageal Fistula Repair: The First Iranian Group Report, Passing the Learning Curve.

BACKGROUND: Thoracoscopic treatment of esophageal atresia and tracheoesophageal fistula (EA+TEF) is accepted as a superior technique at least in cosmetic point of view but it is considered as an advance endoscopic procedure that needs a learning curve to be performed perfectly. This is the first report of Iranian group pediatric surgeons in thoracoscopic approach to EA. METHODS AND MATERIALS: Since 2010, twenty four cases with EA+TEF underwent thoracoscopic approach in Sarvar Children Hospital (Mashhad -Iran). During the first 6 months, thoracoscopic approach to 6 cases of EA+TEF was converted to open procedure because of technical and instrumental problems. The first case of successful thoracoscopic EA repair was accomplished in 2010 and since then, 10 cases of EA+ TEF among 18 patients were treated successfully with thoracoscopic approach RESULTS: Overall conversion rate was 58.3% but conversion rate after the primary learning curve period, was 35.7%. The main conversion causes include difficulties in esophageal anastomosis, limited exposure and deteriorating the patient's condition. Anastomotic leak and stenosis were observed in 20% and 40% respectively. Overall mortality rate was 4.2%. CONCLUSION: Thoracoscopic repair of esophageal atresia seems feasible and safe with considerable superiorities to the conventional method although acceptable results needs a prolonged learning curve and advanced endoscopic surgical skill. Clear judgment about the best surgical intervention for EA according to all cosmetic and functional outcomes needs further studies.

Two-stage endoscopic repair of congenital diaphragmatic hernia.

Minimally invasive surgery (MIS) in congenital diaphragmatic hernia (CDH) repair has recently been gaining popularity and is the first choice in most departments. Loss of space in an undeveloped abdomen may cause serious problems such as difficult visceral reduction and need for conversion or postoperative complications such as abdominal compartment syndrome. A 4-month-old boy with delayed diagnosis of CDH underwent MIS repair via thoracoscopy, but the abdominal space was not large enough to accept the herniated viscera so a wide transverse fasciotomy was performed via laparoscopy and an iatrogenic ventral hernia induced. The abdomen was distended by pneumoperitoneum, which was retained for 2 days. Finally thoracoscopic CDH repair was completed successfully and the diaphragmatic defect reconstructed by prosthetic patch. Laparoscopic transverse abdominal fasciotomy is suggested as the first step in MIS repair of CDH in an undeveloped abdomen.

Two staged modified substitution urethroplasty using appendix-free flap.

Reconstruction of long posterior urethra defect is technically challenging. Substitution urethroplasty is used in long, complex, recurrent posterior urethral strictures. This article presents a modified technique and the clinical outcome of two-stage substitution urethroplasty with appendix free flap and microvascular anastomosis. A three-year-old boy with a 5 cm iatrogenic posterior urethral defect was managed by urethral substitution using the appendix. An appendix-free flap was used according to anatomic limitations, employing the transposed inferior epigastric artery and saphenous vein to maintain conduit blood supply. The conduit was buried in the scrotum for 3 weeks and its viability monitored until the final reconstructive stage. Two-year follow-up with ultrasound and cystoscopy revealed satisfactory results. A well-vascularised bed and flap are the mainstays of substitution urethroplasty, so we suggest inferior epigastric artery perineal transposition and staged reconstruction as alternatives that may improve the blood supply of the neourethra.