Shehata technique versus Fowler-Stephens orchidopexy in intra-abdominal testis: A meta-analysis.

INTRODUCTION: The management of intra-abdominal testis (IAT) represents a significant clinical challenge, necessitating the transposition of the testis from the abdominal cavity to the scrotum. This procedure is rendered complex by the abbreviated length of the testicular vessels. OBJECTIVE: Our purpose in this study was to conduct a systematic review and meta-analysis comparing Shehata technique (ST) versus Fowler Stephens technique (FST) in treating patients with IAT. STUDY DESIGN: We conducted a comprehensive literature search using several databases, including Ovid Medline, Cochrane, PubMed, Google Scholar, Web of Sciences, EMBASE, and SCOPUS until February 2024. This study included research that compared ST and FST for managing intra-abdominal testis. We evaluated the rates of atrophy and retraction, as well as the overall success rates, for both techniques. RESULTS: Six studies were identified as appropriate for meta-analysis, comparing orchidopexy performed using the ST with 169 patients, against the FST involving 162 patients. The comparison showed no statistically significant age difference at the time of surgery between the groups (I2 = 0%) (WMD 0.05, 95% CI - 1.24 to 1.34; p = 0.94). Operative time in first the stage was lower in the FST group than ST group (I2 = 95%) (WMD 10.90, 95% CI 1.94 to 19.87; p = 0.02). Operative time in the second stage was lower in the ST group than FST group (I2 = 83%) (WMD - 6.15, 95% CI - 12.21 to -0.10; p = 0.05). Our analysis showed that ST had a similar atrophy rate (I2 = 0%) (OR: 0.45, 95% CI: 0.20 to 1.01; p = 0.05). No difference was found between techniques in terms of retraction rate (I2 = 0%) (OR: 0.64, 95% CI: 0.17 to 2.47; p = 0.52). The ST demonstrated a notably higher overall success rate compared to FST (I2 = 1%) (RR: 1.14, 95% CI: 1.03 to 1.27; p = 0.009). Overall success rate in ST and FST were 87% and 74%, respectively. Overall atrophy rate in ST and FST were 5% and 12%, respectively. Overall retraction rate in ST and FST were 5% and 10%, respectively. DISCUSSION: The ST, renowned for its pioneering two-stage laparoscopic approach that leverages mechanical traction to lengthen the testicular vessels, is gaining popularity due to its recognized safety and efficacy. Conversely, the Fowler-Stephens technique, a traditional method that relies on collateral blood supply for testicular mobilization, has come under examination for its potential link to an increased risk of testicular atrophy. CONCLUSION: This meta-analysis reveals that the Shehata technique has similar or better outcomes compared to the Fowler-Stephens technique in IAT management. Further prospective multicentric randomized controlled trials are warranted.

Spectrum of Genital and Extragenital Anomalies in Malformation Syndromes Associated With 46, XY Disorders of Sex Development: A Single Center Experience.

OBJECTIVES: This study aimed at integrating the clinical and phenotypic characteristics, hormonal profile and genetic diagnosis of children with malformation syndromes associated with XY disorders of sex development (DSD) in a single-center in Egypt. METHODS: This retrospective study included patients with syndromic XY DSD recruited from the Pediatric Endocrinology and Surgery units at Alexandria University Children's Hospital (AUCH), Alexandria, Egypt, between 2018 and 2023. All patients included in the study underwent a detailed clinical and laboratory evaluation, ultrasonography (and laparoscopy if needed). RESULTS: The study included 30 children with syndromic XY DSD; most of these children were diagnosed at birth. The most common extragenital malformations included skeletal anomalies (70%), facial dysmorphism (46.7%), cerebral malformations (30%) and congenital heart disease (23.3%). Ventricular septal defect was the most common congenital heart disease. CONCLUSION: Integration between clinical, laboratory and genetic data is the cornerstone in the management of XY DSD patients for appropriate decision making of surgical intervention and sex assignment, in addition to screening for other associated features of each mutation.

Spectrum of Genital and Extragenital Anomalies in Malformation Syndromes Associated With 46, XY Disorders of Sex Development: A Single Center Experience.

OBJECTIVE: This study aimed at integrating the clinical and phenotypic characteristics, hormonal profile and genetic diagnosis of children with malformation syndromes associated with XY disorders of sex development (DSD) in a single-center in Egypt. METHODS: This retrospective study included patients with syndromic XY DSD recruited from the Pediatric Endocrinology and Surgery units at Alexandria University Children's hospital (AUCH), Alexandria, Egypt, during the period between 2018 and 2023. All patients included in the study underwent a detailed clinical and laboratory evaluation, ultrasonography (and laparoscopy if needed); and decision making was done accordingly. RESULTS: The study included 30 children with syndromic XY DSD; most of these children were diagnosed at birth. The most common extragenital malformations included skeletal anomalies (70%), facial dysmorphism (46.7%), cerebral malformations and congenital heart disease (23.3%). Ventricular septal defect was the most common congenital heart disease. CONCLUSION: Integration between clinical, laboratory and genetic data is the cornerstone in the management of XY DSD patients for appropriate decision making of surgical intervention and sex assignment, in addition to screening for other associated features of each mutation.

Does the contralateral testicular volume decide the need for diagnostic laparoscopy in cases of unilateral impalpable undescended testis?

BACKGROUND: This study aimed the evaluation of the value of the calculated volume of a normal testis to predict the status of its contralateral impalpable side and hence decide the importance of laparoscopic exploration. METHODS: Patients with unilateral impalpable undescended testis - as confirmed by clinical and sonographic examination- were enrolled in our prospective interventional study between November 2018 and August 2022 at Elshatby University Hospital, Faculty of Medicine, Alexandria University. The volume and three-dimensional diameter of the normal contralateral testis were measured by the pre-operative US using the formula: Volume = L x W x H x π/6, where L is the length, W is the width, H is the height, and was correlated with the intra-operative laparoscopic findings. RESULTS: Seventy-six patients were included in our study. The age of the studied patients ranged between 6 months and 4 years with a mean of 2.17 ± 1.30 years; most of them were between one and three years old. Forty-six patients (60.5%) have left-sided impalpable testis and 30 patients (39.5%) have right-sided impalpable testis. The calculated volume of the contralateral normal testis was significantly larger in those patients who had both blind ending vas and vessels (0.89 ± 0.16) and in those who had an atrophic testis passing through the deep inguinal ring (DIR) -which was excised through the inguinal region- (0.83 ± 0.20) than in those patients who had their testes intra-abdominal (0.53 ± 0.18) or passing through the DIR to the inguinal region (0.80 ± 0.19). (Kruskal Wallis test; p < 0.001*). CONCLUSIONS: The calculated sonographic volume of a normal testis can predict the status of its contralateral impalpable side significantly with sensitivity & specificity of 75.0% & 88.89% respectively and a cut-off point of ≤ 0.674; hence, helps in parent counselling preoperatively. TRIAL REGISTRATION: Name of the registry: Clinicaltrials.gov PRS. TRIAL REGISTRATION NUMBER: NCT05933811. Date of registration: 10-7-2023 (retrospectively registered). URL of trial registry record: https://clinicaltrials.gov .

Splenogonadal fusion: a case report.

BACKGROUND: Accessory splenic tissue is a commonly encountered phenomenon in medical literature. Typically, these accessory spleens are found in close proximity to the main spleen, either in the hilum or within the surrounding ligaments. Nevertheless, it is noteworthy that they can also be located in unusual sites such as the jejunum wall, mesentery, pelvis, and, exceptionally rarely, the scrotum. The first documented case of accessory splenic tissue in the scrotum was reported by Sneath in 1913 and is associated with a rare congenital anomaly called splenogonadal fusion. This report describes an infant who presented with a scrotal mass noted by his mother and after examination, investigations, and surgical exploration, it was revealed to be splenogonadal fusion. CASE DESCRIPTION: An 8-month-old Caucasian male patient presented with a mass in the left testicle and bluish discoloration of the scrotum, which had been incidentally noticed in the previous 2 months. The general physical examination was unremarkable. Other than a palpable scrotal mass that was related to the upper pole of the testis, the rest of examination was unremarkable. Imaging revealed that this mass originated from the tail of the epididymis without infiltrating the testis and tumor markers were normal. On inguinal exploration, a reddish brown 2 × 2 cm mass was found attached to the upper pole and was completely excised without causing any harm to the testis, vessels, or epididymis. Histopathological evaluation confirmed the presence of intratesticular ectopic splenic tissue. CONCLUSION: Although uncommon, splenogonadal fusion can be included in the differential diagnosis of a testicular swelling. Accurate diagnosis allows for appropriate treatment planning which helps to avoid unnecessary radical orchiectomy, which can have a significant impact on the patient's reproductive and psychological wellbeing.

Is cystourethroscopy a crucial preoperative step in severe and complex types of hypospadias?

Background and objectives: Proximal hypospadias is considered the most severe subtype of the hypospadias spectrum and represents approximately one-fifth of the total cases. It is well-evidenced by many studies that the rate of postoperative complications following the repair of this complex subtype is significantly higher when compared to the distal variants. Few reports described the proximal hypospadias from the other perspective which is the preoperative one. Most pediatric surgeons notice an unexplained incidence of lower urinary tract infection and occasional difficulty of urinary catheterization in those children. This sometimes requires the use of additional measures such as the use of urethral sounds, filiforms and followers, and even catheterization under anesthesia. The aim of the work is to evaluate the role of preoperative cystourethroscopy in detecting associated anomalies in cases of proximal and severe hypospadias. Materials and methods: This prospective study was conducted in the pediatric surgery unit at Alexandria Faculty of Medicine between July 2020 and December 2021 and included all children with severe grades of hypospadias. After thorough evaluation, all children underwent cystourethroscopy just before the procedure. Any abnormalities in the urethra, urinary bladder, or ureteric openings were recorded if present. Finally, the definitive operation was performed as per schedule. Results: Fifty-two patients (41 fresh and 11 redo patients) with a median (range) age at presentation of 5 (1-16) years were enrolled in this study. The intraoperative cystourethroscopy was done in all of the patients. Significant abnormal findings were recorded in 32 patients (61.5%), while the other 20 patients (38.5%) were revealed to be normal. The most common abnormal findings were dilated prostatic utricle opening and hypertrophied verumontanum (in 23 and 16 cases, respectively). Conclusion: Although most of the associated anomalies with proximal hypospadias are asymptomatic, cystourethroscopy is better used owing to a high incidence of these anomalies. This can facilitate an early diagnosis as well as early detection and intervention at the time of repair.

Ectopic opening of vasa deferentia into a prostatic utricle cyst.

Prostatic utricle cysts result from incomplete regression of the Müllerian duct remnant or decreased androgenic stimulation of the urogenital sinus. Children usually present with urinary tract infections (UTIs), irritative symptoms and urine retention, although some cases are asymptomatic. These cysts sometimes can cause a palpable abdominal mass, urethral discharge, terminal hematuria and recurrent epididymitis. A high incidence of such cysts is seen in boys with hypospadias, especially the more proximal types.

Evaluation of the safety of using harmonic scalpel during laparoscopic cholecystectomy in children: A preliminary report.

Background and objective: In spite of being one of the most common surgical procedures performed in adults, laparoscopic cholecystectomy (LC) is relatively uncommon in the pediatric age group. Most surgeons prefer to dissect the cystic duct using a monopolar electrosurgical hook and occlude it with simple metal clips. Although the safety of using the ultrasonically-activated shears, e.g., harmonic scalpel for dissection of the gallbladder is confirmed in many studies, its efficacy in the closure of the cystic artery and duct in adults is still debatable. Furthermore, very few reports studied its safety in children during LC. The aim of our work is to study the safety and efficacy of ultrasonic shears in controlling the cystic duct and artery during LC in children. Materials and methods: A prospective study was conducted from May 2017 to April 2020, where all children having symptomatic gallbladder stone disease were included in the study. HS was used as a sole instrument in gallbladder dissection as well as in controlling cystic duct and artery. No metal clips or sutures were used throughout the procedure. Results: A total of forty-two children having symptomatic gallstone disease were included in the study. The main indication for LC was hemolytic anemia. Their age ranged from 3 to 13 years with a mean of 8.4 ± 3.25 years. All operations were completed laparoscopically, i.e., no conversion to open surgery was needed. The mean operative time was 40 ± 10.42 min. There were no intraoperative complications apart from gall bladder perforation in two cases during dissection from the liver bed while the postoperative recovery was smooth in all patients. Patients started oral feeding after 11.30 ± 3.01 h. The mean time for discharge was 25.47 ± 7.49 h, ranging from 14 to 48 h. Postoperative ultrasound for all cases showed no evidence of minor or major bile leaks or CBD injuries. Conclusion: This is the first report to evaluate the use of HS as a sole instrument during LC in the pediatric age group. HS is a safe and efficient instrument that can be used alone in gallbladder dissection as well as in controlling cystic duct and artery during LC in children.

Intussusception in preterm neonates: A systematic review of a rare condition.

BACKGROUND: While necrotizing enterocolitis (NEC) is a prevalent condition in preterm neonates admitted to neonatal intensive care unit (NICU), intussusception is exceedingly uncommon and often overlooked. This is due to the fact that they share many clinical characteristics. The initial misdiagnosis of intussusception in preterm neonates (IPN) especially has led to a delay in their management, which increases the risk of developing compromised bowel. Additionally, it is difficult to reach a diagnosis as neonatal intussusception does not have any classical radiological signs even when contrast enema is used. This systematic review is based on the published literature including case reports and case series to review the clinical features of IPN and how to differentiate it from NEC in order to shed the light on this rare disease and how having a high index of suspicion would help practitioners to make an early and accurate diagnosis METHODS: A systematic literature search to report all cases of relevant articles that reported IPN till date. All cases that were born before 37 weeks gestational age, presented within the neonatal period and having well established documentation were included in the study. Any case that did not have these criteria was excluded from our study. RESULTS: Only 52 cases met these criteria during the period from 1963 till date. An average of 10 days had elapsed before the cases were confirmed to have IPN either clinically or intraoperatively. The most frequent manifestations were abdominal distension and bilious gastric residuals, occurring in 85% and 77% of the cases respectively, followed by bloody stools in 43% of cases. However, this triad was present only in approximately one-third of the cases. Only 13 cases were diagnosed as having intussusception preoperatively. About two thirds of the intussusception were located in the ileum. Pathological lead point was present in 7 cases only; 4 of them were due to Meckel's diverticulum. Nine cases only out of the 52 cases with IPN died. CONCLUSION: It is crucial to detect the clues for diagnosis of intussusception because in contrast to NEC, it is unresponsive to conservative management, affects the viability of the bowel and surgery is essential.

Thoracoscopic Repair of Neonatal Congenital Diaphragmatic Hernia: Minimizing Open Repair in a Low-Income Country.

Aim: To assess the severity of persistent pulmonary hypertension (PPH) in congenital diaphragmatic hernia (CDH) neonates solely using oxygenation index (OI). Study Design: A prospective study was carried out from April 2016 to March 2019, where all confirmed CDH neonates were evaluated for the possibility repair through thoracoscopic approach. The severity of PPH was assessed using OI. It is calculated using the equation: mean airway pressure (MAP) × FiO2 × 100 ÷ PaO2. Neonates having OI <5 were considered to have a mild degree of pulmonary hypertension; hence, thoracoscopic repair was offered for them. Results: Thirty-nine CDH cases met the selection criteria; therefore, they underwent thoracoscopic repair. Primary diaphragmatic repair was successfully accomplished thoracoscopically in all neonates without any perioperative complications. Conversion from thoracoscopy to open method occurred in five cases. The causes were due to difficulties encountered during repair and none was due to a pure anesthetic problem or general deterioration during thoracoscopy. Recurrence had occurred in two cases only. Conclusion: OI is a reliable subjective parameter that could be used as an adjuvant to the usually used cardiovascular and pulmonary parameters for thoracoscopic repair decision. With increasing surgical experience, a wider range of neonates may be considered for thoracoscopic CDH repair.

Huge ovarian cyst in a neonate with classical 21-hydroxylase deficiency.

Congenital adrenal hyperplasia is the most common cause of ambiguous genitalia worldwide, with an incidence of 1 in 15,000 live births. The most frequently-occurring subtype, 21-hydroxylase deficiency, results in diminished production of aldosterone and cortisol as well as increased androgen secretion. Previous studies have reported a relationship between ovarian cyst formation and adrenal androgen excess; nevertheless, neonatal large ovarian cysts have rarely been reported in newborns with congenital adrenal hyperplasia. Herein, we present the unique case of a neonate with classical 21-hydroxylase deficiency who underwent surgery for a huge unilateral solitary ovarian follicular cyst on the seventh postnatal day. Possible mechanisms by which androgen excess may cause ovarian cyst formation are also discussed.

Gastroduodenal Intussusception Due to Gastric Mucosal Prolapse Polyp in a 2-Year-Old Child.

BACKGROUND: Gastroduodenal intussusception is an invagination of a portion of the stomach into the duodenum. It predominately occurs in adults. Case Report: We present a gastroduodenal intussusception in an hypochromic microcytic anemic 2-year-old girl. A large filling defect in the second and third parts of the duodenum, indenting the pyloric antrum, was due to a gastroduodenal intussusception secondary to a cauliflower-like gastric mucosal prolapse polyp, a type of gastric hyperplastic polyp. Conclusion: Anemia may accompany a gastric mucosal prolapse polyp.

Hemiscrotal agenesis: a novel phenotype of a rare malformation.

BACKGROUND: Hemiscrotal agenesis (HSA) is an exceedingly rare congenital anomaly in scrotal development. It is characterized by unilateral absence of scrotal skin with intact midline raphe. In the English literature, only seven patients were diagnosed with HSA. Herein, we report a 14-month-old boy with HSA, unilateral cryptorchidism and a perineal skin tag. Additionally, the patient had a monodactylous limb, unilateral cerebellar hypoplasia, and a cardiac septal defect. CASE PRESENTATION: A 14-month-old boy presented with right HSA and ectopic scrotal skin in the right perineal region. Extra-genital examination showed right monodactylous lower limb, without dysmorphic facial features or any other skeletal anomalies. His karyotype was 46, XY, while his hormonal profile showed prepubertal LH and FSH. Skeletal survey showed right monodactylous lower limb (with only a big toe which had 2 phalanges) and normal spine alignment. A previous echocardiography was done and showed a small muscular ventricular septal defect (VSD) that closed on follow-up. Magnetic resonance imaging of the brain showed posterior fossa malformation. The patient had his right testis fixed in the right scrotum. The pathological examination of the perineal lesion showed fibro-epithelial polyp (skin tag), with no testicular tissue or atypia. CONCLUSION: We believe that this is the first case to be reported with hemiscrotal agenesis and ipsilateral cryptorchidism, associated with a perineal skin tag, unilateral monodactylous lower limb on the same side, unilateral cerebellar hypoplasia, and VSD. Interestingly, further genetic analysis is required to reach a final diagnosis. However, regrettably, advanced molecular diagnostic studies for this patient is not available in our country.

Congenital short bowel syndrome: a rare cause of neonatal intestinal obstruction.

Congenital short bowel syndrome (CSBS) is an uncommon gastrointestinal disorder in which an unclear aetiology causes considerable intrauterine reduction in small bowel length. As a result of reduced absorptive intestinal length, chronic diarrhoea, vomiting, and consequently, failure to thrive are likely. We report a case of CSBS in a 26-day-old girl who had malrotation and a short bowel with a length of bowel from the pylorus to the ileocecal junction of approximately 40 cm. The patient underwent Ladd's procedure, but she is still dependent on parenteral nutrition.

Neonatal Gastrointestinal Stromal Tumor of the Sigmoid Colon: A Case Report and Review of Literature.

ABSTACTBackground: Gastrointestinal stromal tumors (GIST) are the most common mesenchymal neoplasms of the digestive tract. They are believed to originate from the interstitial cells of Cajal or their precursors. Case Report: A 10-day-old boy presented with intestinal obstruction. He was operated upon and a mass was found at the sigmoid colon, which was resected and divided colostomy was performed. The diagnosis of neonatal GIST was confirmed by histologic and immunohistochemical studies. Conclusion: This is the ninth case of neonatal GIST that arose from the intestine and the first reported to arise from the sigmoid colon. Colonic GIST can occur in the neonatal age group.

Pseudopancreatic Cyst Extending into the Mediastinum in a 7-Year-Old Child.

Mediastinal pseudopancreatic cyst (MPP) is the extension of a pancreatic pseudocyst through esophageal or aortic hiatus into the posterior mediastinum. It can produce a range of manifestations caused by compression by the cyst, for instance, odynophagia, dysphagia, pericardial, or pleural effusion. Here we report a case of MPP in a 7-year-old child who was presented with repeated chest infections and left pleural effusion. It was successfully drained by cystogastrostomy.

Gallbladder Papilloma in a Child Unmasking Metachromatic Leukodystrophy: A Case Report With Review of Literature.

Background: Metachromatic leukodystrophy (MLD) is a lipid storage disease characterized the accumulation of sulfatides in different viscera including the gallbladder. Case report: A 2-year-old girl had upper right quadrant lesion that was preoperatively thought to be a biliary cystadenoma. Histologically, the gallbladder lesion was a tubulo-villous papilloma with multiple foci of papillary mucosal hyperplasia. Many storage histiocytes containing metachromatic granules, characteristic of MLD, were present in the tips of the papillae. MLD was later confirmed by enzyme studies. Conclusion: Gallbladder papilloma can be the presenting feature of MLD.