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Abstract Background Pleuropulmonary Blastoma (PPB) is an extremely uncommon, highly aggressive tumor that arises from either the lungs or pleura. According to Dehner, PPB was classified into three groups: type I (cystic), type II (mixed), and type III (solid). Type I tends to occur more commonly in infants and has a more favorable prognosis compared to types II and III. This tumor is very rare in pediatric age group; hence, there is no consensus on the optimal treatment regimen for it to date. Type I tumors, which resemble congenital lung cysts, can eventually progress to more aggressive type II and type III tumors. This article aims to increase general awareness of this pathology, clinical presentation, and differential diagnosis in order to identify this rare entity early in its course. By presenting 4 such cases, we highlight that PPB can be missed early in diagnosis and it is important to be alert when putting this rare tumor in differential diagnosis of cystic lung lesions. Methods A retrospective study was conducted between 2015 and 2020 involving patients who had a definitive diagnosis of PPB with emphasis on clinical presentation, preoperative imaging studies, intra-operative findings, pathological reports, ancillary treatment, and outcomes. All patients were followed up every 6 months to monitor local recurrence and distant metastasis by undergoing physical exam and non-contrast enhanced CT of the chest. The primary outcome is to identify the mortality and morbidity (recurrence and distant metastasis) of PPB for cases admitted in our institute. Results Four children were diagnosed with PPB during the study period. Clinically, patients presented with manifestations ranging from respiratory distress, fever to obstructive shock and radiologically, 2 cases were presented with mediastinal mass and the other 2 presented with pneumothorax. Regrettably, none of the cases were diagnosed pre-operatively. One lesion proved to be type I, 2 were type II and one was type III. All cases underwent chemotherapy using the combination of vincristine, Adriamycin and cyclophosphamide (VAC regimen). Recurrence was detected in a type II case, around 2 years after operation, and the other type II case developed brain metastasis that was discovered 3 years after operation. Type I case showed no local or distant metastasis. Conclusion A prompt preoperative diagnosis and workup of cases of PPB is crucial to enable optimal intervention intraoperatively and early postoperative treatment. Though it is uncommon, PPB should be considered in the differential diagnosis of cystic lung lesions.

Abstract Background Progressive familial intrahepatic cholestasis is an autosomal recessive genetic disorder that manifests primarily with jaundice and pruritus and can progresses from persistent cholestasis to cirrhosis and late childhood liver failure. Classically, progressive familial intrahepatic cholestasis is classified into three subtypes: 1, 2, and 3 and results from a defect in a biliary protein responsible for bile formation and circulation in the liver. In the last decade and with the increased use of genetic testing, more types have been known. Case presentation A 6-month-old Afrocentric boy presented with progressive jaundice and pruritus that started since the age of 2 months. He was thoroughly investigated to be finally diagnosed as progressive familial intrahepatic cholestasis type 4. A low-fat diet, ursodeoxycholic acid, fat-soluble vitamins, and cholestyramine were started. He showed initial improvement then had refractory pruritus and impaired quality of life. He underwent surgical biliary diversion at the age of 1 year with marked improvement of manifestations. Conclusion Owing to the increased technology of genetic testing, more clinical subtypes of progressive familial intrahepatic cholestasis were diagnosed other than the classical three types. Surgical management using biliary diversion could be beneficial and delays or may even obviate the need for liver transplantation.

Abstract 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

Abstract 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.

Background: Ureteral atresia is a rare disease that is usually associated with a non-functioning dysplastic kidney. The condition may be unilateral or bilateral, short, or long, and may involve any part of the ureter. Case description: A 4-year-old boy presented with a gradually developing right-sided abdominal distension. Abdominal examination revealed a right lumbar mas of approximately 20 × 15 cm. Abdominal ultrasound showed a large cystic mass that extended from the hilum of the right kidney and crossed the midline to reach the left side of the abdomen. The left kidney was absent. Voiding cystourethrogram (VCUG) was unremarkable. Magnetic resonance urography (MRU) confirmed the presence of a thin-walled cystic lesion extending from the right renal hilum, crossing midline, reaching the left lumbar region, and the absence of the left kidney. Renal nuclear scan showed a functioning right kidney and an absent left kidney. Exploratory laparotomy via a Pfannenstiel incision revealed a normal right kidney and a large extraperitoneal abdominal cyst. This cyst contained about 200 cc of brownish fluid. The cystic mass was in fact a crossed fused left kidney with a proximal ureteral atresia. Excision of the cyst along with the left ureter was done. The postoperative course was uneventful. Conclusion: Ureteral atresia is a rare condition. It can result in a dysplastic kidney in the form of a large retroperitoneal abdominal cystic mass.

Background: Double appendix, also known as vermiform appendix duplex, is a rare congenital anomaly. It is estimated to occur in 1 in 25,000 people with fewer than hundred cases reported worldwide. Case description: A 10-year-old boy presented to our clinic with fecal incontinence. He had a history of an anorectal malformation (recto = bladder neck fistula), for which he had undergone a neonatal anorectoplasty with an abdominal approach. The patient was not on any bowel management program. A plan of daily enemas, toilet training, and dietary measures including a high fiber and low carbohydrate diet was started. Despite following this program, his fecal incontinence persisted. The patient subsequently underwent a laparoscopic-assisted Malone appendicostomy without complications. During the operation, two cecal appendices were found. One appendix was removed, while the other one was used for the Malone appendicostomy. His postoperative course was uneventful. The patient currently does antegrade enema twice daily and his incontinence has completely resolved. Conclusion: Double appendix, although very uncommon, can be found in patients with anorectal malformations.

Abstract Introduction Rectal atresia is a rare subtype of anorectal malformations in which the patients are born with a normal anal canal but have complete atresia located few centimeters proximal to the dentate line. We present the transanal end-to-end rectoanal anastomosis as a surgical technique for the management of these patients, highlight the outcomes, and emphasize on some clinical tips. Methods Four patients were diagnosed as having rectal atresia on clinical and radiological basis. All of them underwent single loop low sigmoid colostomy in the first 24 h. After 6 months, transanal end-to-end rectoanal anastomosis was performed followed by closure of the stoma 3 months later. Results The 2 cases that are older than 3 years demonstrated normal continence and are clean between bowel movements, while the other two showed good anal tone and passing stools between 1–3 times daily, being dry in between. Conclusion Transanal recto-anal anastomosis allows a safer route of anatomical reconstruction of the anorectum, therefore avoiding the potential complications associated with the other more invasive approaches.

Background and objectivesProximal 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 methodsThis 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.ResultsFifty-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).ConclusionAlthough 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.

IntroductionConcealed penis, which is the congenital type of buried penis, is a condition in which a normal-sized penis is totally or partially hidden by pubic, scrotal, or thigh skin. Several procedures had been described for its correction including phallopexy, that is, fixation of penile Buck's fascia to the sub-dermis.Aim of the workOur work aims to study the difference in outcome between performing phallopexy at one level and at two levels.Material and methodsOur study included 180 uncircumcised patients who had a concealed penis while having an average length of an outstretched penis. These patients were divided into two groups: the first one was treated with one level of phallopexy at the 3 and 9 o'clock points, while the second group was treated with the same procedure in addition to another level of stitches at the mid-penile level. The follow-up was carried out for one post-operative year regarding penile skin edema, infection, congestion, necrosis, and/or re-retraction.ResultsThe overall success rate was 96.1% for a normally-looking penis without post-operative re-retraction. Re-retraction developed in two patients (2.2%) of those who had one-level phallopexy and in five patients (5.6%) of those who had two-level phallopexy without statistical significance (FEp = .444). Penile skin edema developed in 76 patients (42.2%) being significantly lower in patients with lower body weight (p = .030*).ConclusionPhallopexy could be performed safely in the case of the concealed penis with satisfactory results. Two levels of phallopexy did not add any advantage to the post-operative results besides the fact that this may be demanding, time-consuming, and may require higher resources, so we recommend the easier one-level phallopexy in the treatment of such conditions with satisfactory results.

Abstract 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.

Background and objectiveAlthough complex atresias, such as apple-peel and multiple atresias, comprise a smaller percentage, they are usually associated with a higher incidence of postoperative complications and mortality rate. Contrary to simple atresias where the surgical technique of choice usually entails bowel resection and anastomosis with or without enteroplasty, managing apple-peel atresia remains more sophisticated. Decompressive and functionalizing stomas are sometimes mandatory to overcome problems such as increased wall thickness and the wide disparity among the anastomotic ends. Few reports discussed using tube enterostomy in the management of apple-peel atresia; nonetheless, no previous prospective studies were conducted to discuss its efficacy on a larger population. In this study, we are describing our experience using this technique on 12 patients suffering from apple-peel atresia in our center.MethodsA prospective study was conducted from June 2015 to May 2020, where all children who were found to have apple-peel atresia were included in the study. T-tube was placed through an enterotomy through the dilated proximal bowel, around 10 cm before the anastomotic line, and was kept in place using a double suture (Stamm technique) before closing the anterior face of the anastomosis. The short distal limb of the T-tube was oriented toward the anastomotic line, while the long proximal limb was directed proximally. After finishing the anastomosis, the T-tube was delivered outside the abdominal wall, anchoring the enterostomy along with the proximal dilated jejunum against the anterior abdominal wall.ResultsA total of 12 cases were encountered throughout the period of study. The mean age at operation was 4 days and the mean birth weight was 2700 g. The mean time for starting oral feeding postoperatively and T-tube removal was 8 and 10.5 days, respectively. Cases were discharged after a mean of 22 days. As regards morbidity and mortality, a single case developed skin excoriations at the site of tube insertion and was managed conservatively using topical ointments and another case died from overwhelming sepsis 3 days after the operation.ConclusionT-tube enteroplasty is a safe and feasible option in the surgical management of apple-peel atresia. The main strength of our study is its prospective nature and that it includes apple-peel atresia cases only. However, the main limitation is that a larger sample is needed.

Scrotoschisis, also known as extracorporeal testicular ectopia, is an exceedingly rare congenital abnormality that affects the wall of the scrotum. In this condition, the testis exteriorizes through an opening in the anterior scrotal wall. The exact etiology for scrotoschisis is still unknown; however, many theories arose to explain its occurrence. Herein, we report 19th case in the literature in a 2-day-old boy who presented with an eviscerated right testis. We highlighted the possible etiologic hypotheses and management considerations as well.

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.

Background and objectiveIn 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 methodsA 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.ResultsA 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.ConclusionThis 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.

Background and ObjectiveThe optimal treatment protocol of intraabdominal testis is still a matter of debate and until now there are a lot of areas of controversy as regards this challenging subtype. The aim of this report is to document current practice patterns among surgeons from different continents through an online Redcap survey supervised the World Federation of the Association of Pediatric Surgeons (WOFAPS).MethodsA 16-question-survey related to the management of intraabdominal testis was created and administered via RedCap. The WOFAPS headquarters sent an email to all members inviting voluntary survey participation. Data were entered using Microsoft EXCEL spreadsheet and analyzed. Descriptive statistics were performed for each survey item.ResultsThere were 436 WOFAPS members who participated in this study with a response rate of 29%, and the vast majority were pediatric surgeons. Only 13% tried to use hormone therapy aiming to induce testicular descent or to improve future fertility. The choices of various surgical techniques were noted. During laparoscopy, if vessels and cord structure were seen entering the ipsilateral internal inguinal ring, most respondents chose to explore the groin. On the other hand, should there was an absent or atrophic testis, the respondents were split on whether to perform a contralateral orchiopexy.ConclusionThis survey describes the current practices of a sample of pediatric surgeons and urologists in the management of intraabdominal testis. The use of hormonal treatment, timing of fixation and management in case of passing through vas and vessels through DIR were undisputable. However, management of low-lying and peeing testis together with the management of contralateral testis were still debatable.

Abstract Purpose The current study aimed to report on the safety and efficacy of utilizing a modified WALANT (mWALANT) technique during open surgical carpal tunnel release (CTR), where we used undiluted epinephrine compared to the originally described WALANT technique. Methods From January 2015 till the end of June 2021, 200 patients (175 (87.5%) were females) who presented with carpal tunnel syndrome, either bilateral (108 (54%) patients) or unilateral (92 (46%)) were included, formulating a total of 308 procedures. Open surgical CTR was performed as a daycare procedure by the same surgeon. The mWALANT injectable mixture was prepared by mixing 8 CC of 2% lidocaine HCl + 1 CC of 0.25 mg/1 ml epinephrine without dilution (2.5 times the concentration used in the original WALANT technique). The injection was performed before draping. Results The patients’ average age at surgery was 42.88 ± 13.03 years old; they were followed up for an average of 31 ± 17.17 months. The average operative time was 9.5 ± 1.87 min. None (0.0%) of the patients needed top-up of local anesthesia or shift into general anesthesia, and no (0.0%) patients needed postoperative hospital stay. The average VAS during the surgical procedure was 2.5 ± 2.1, mainly reported during infiltration of the local anesthesia; no patients reported discomfort during the surgical procedure itself. 180 (90%) patients reported a full return to their usual preoperative ADL after an average of 4.7 ± 1.2 weeks. No (0.0%) postoperative fingers ischemic or temperature changes. Two (1%) patients experienced an adrenaline rush in the form of tachycardia that needed sedation and close monitoring by the anesthesiologist; they were discharged on the same day. One (0.5%) patient (who had uncontrolled diabetes mellitus) showed a superficial wound infection which resolved after conservative management. Conclusions Using undiluted epinephrine during the mWALANT technique is safe and effective. There is no need to wait until the drugs fully function, and no epinephrine-related complications were encountered apart from occasional adrenaline rush symptoms.

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.