Management strategy and outcomes of sacrococcygeal teratoma - an Egyptian multicenter experience.

OBJECTIVES: Nationwide criteria regarding patients with sacrococcygeal teratoma (SCT) are still lacking in Egypt. We aimed to present a multicenter study regarding the management and outcomes of this tumor to evaluate our national treatment strategy. METHODS: A retrospective analysis including all patients with SCT who were managed at four major Egyptian centers between 2013 and 2023. Clinical data, surgical approaches, and short- and long-term outcomes were discussed. RESULTS: The study included 95 patients (74 were females). Antenatal diagnosis was reported in 25% of patients. Seventy-one patients (74.7%) were classified as Altman type I/II. Surgery was performed via a perineal approach in 75 patients, whereas the remaining 20 underwent a combined abdominoperineal approach. Vertical elliptical incision with midline closure was conducted in 51.5% of patients, followed by classic or modified chevron incisions. Benign mature teratoma was detected in 82% of patients. At a median follow-up of 57 months, eight patients (8.5%) had relapsed. The 5-year overall survival (OS) and event-free survival (EFS) of all patients were 94% and 91%, respectively. In the after-care monitoring, 19 patients (20%) had urinary or bowel dysfunctions. Nine of them were managed using medications. Clean intermittent catheterization was practiced in another five patients. The remaining five underwent further surgical interventions. CONCLUSION: Favorable outcomes were achieved in our country during the last decade. Diverse perineal incisions were performed for resection, and vertical elliptical with midline closure was the commonest. During follow-up, 20% of patients developed urological or bowel dysfunctions that required medical and surgical treatment modalities to improve their quality of life.

Congenital aflatoxicosis, mal-detoxification genomics & ontogeny trigger immune-mediated Kotb disease biliary atresia variant: SANRA compliant review.

Biliary atresia (BA) is the most common indication for pediatric liver transplantation. We describe The BA variant: Kotb disease. Liver tissue in the Kotb disease BA is massively damaged by congenital aflatoxicosis resulting in inflammation, adhesions, fibrosis, bile duct proliferation, scarring, cholestasis, focal syncytial giant cell transformation, and typical immune response involving infiltration by CD4+, CD8+, CD68+, CD14+, neutrophil infiltration, neutrophil elastase spill, heavy loads of aflatoxin B1, accelerated cirrhosis, disruption of p53 and GSTPi, and have null glutathione S transferase M1 (GSTM1). All their mothers are heterozygous for GSTM1. This inability to detoxify aflatoxicosis results in progressive inflammatory adhesions and obliterative cholangiopathy early in life. The typical disruption of both p53 and GSTPi causes loss of fidelity of hepatic regeneration. Hence, regeneration in Kotb disease BA typically promotes accelerated cirrhosis. The immune response in Kotb disease BA is for damage control and initiation of regeneration, yet, this friendly fire incurs massive structural collateral damage. The Kotb disease BA is about actual ongoing hepatic entrapment of aflatoxins with lack of ability of safe disposal due to child detoxification-genomics disarray. The Kotb disease BA is a product of the interaction of persistent congenital aflatoxicosis, genetic lack of GSTM1 detoxification, ontogenically impaired activity of other hepatic detoxification, massive neutrophil-elastase, immune-induced damage, and disturbed regeneration. Ante-natal and neonatal screening for aflatoxicosis, avoiding cord milking, and stringent control of aflatoxicosis content of human, poultry and live-stock feeds might prove effective for prevention, prompt diagnosis and management based on our recent understanding of its patho-genomics.

Outcome and complications after surgery for thyroid carcinoma in pediatric age-an evaluation of practice.

OBJECTIVES: To discuss management protocol, surgical complications, and outcomes of thyroid carcinoma in children. METHODS: We performed a retrospective analysis including all pediatric patients with thyroid carcinoma who were managed at our institution between January 2011 and January 2021. Data were analyzed regarding demographics, clinical features, operative details, postoperative complications, and survival data. RESULTS: Thirty-two patients were identified; 26 females (81.25%) and 6 males (18.75%). The median age at operation was 14 years (range: 5-18). Twenty-six (81.25%) patients presented with palpable thyroid swelling. Median tumor size was 3 cm (range: 1-7). Metastatic workup did not detect any pulmonary metastases. Total thyroidectomy was performed in 25 patients (78%), and 16 of them underwent additional bilateral neck dissection (16 had central nodal dissection, and 7 had both central and lateral nodal dissection). Seven patients (22%) underwent hemithyroidectomy, and only one of them had a completion thyroidectomy after 2 weeks. Conservative resection was adopted in six children with similar criteria (tumor size < 1.5 cm in one lobe, no extrathyroid extension, differentiated thyroid carcinoma, no detected lymph nodes). Postoperative complications occurred in eight patients (all had total thyroidectomy) with an overall incidence of 25%. Seven patients had transient morbidities that were managed conservatively (chylous leak n = 1, hypoparathyroidism n = 3, and nerve palsy n = 3). At a median follow-up time of 54 months, four patients had relapsed (all underwent total thyroidectomy). The 5-year OS and EFS were 100% and 87.5%, respectively. CONCLUSION: Operative resection for pediatric thyroid carcinoma can be performed with average short-term complications and achieving excellent outcomes. Total thyroidectomy remains the standard procedure of choice in the majority of those patients. However, conservative surgery can be successfully adopted in a well-selected group of children with favorable long-term results as per our findings.

Laparoscopic management of infantile hydrocele in pediatric age group.

PURPOSE: To evaluate laparoscopic management of hydroceles in pediatrics, with evaluation of the internal inguinal ring (IIR) and the PPV (patent processus vaginalis) in different types of hydroceles, and the incidence of the contralateral PPV. METHODS: The IIR and the type of hydrocele on the same side of 93 patients with 106 infantile hydroceles were evaluated and managed, in addition to contralateral side. RESULTS: The IIR on same side was closed in 8.5% (Type I) and patent in 91.5% (Type II and III) with different shapes. Contralateral IIR was open in 88.7% of cases. The operative time was 30.99 ± 7.23 min, with no intra-operative complication. The vas deferens and testicular vessels were secured and there were no injuries or bleeding. The conversion rate was zero, and all procedures (Type II and II) were completed totally laparoscopic. No post-operative complications except a case of tense hydrocele developed scrotal edema that managed conservatively. CONCLUSION: Laparoscopic hydrocelectomy is safe, applicable and feasible for management of different types of hydroceles in pediatrics. The IIR is patent in nearly all cases with/out communication to the hydrocele. The contralateral IIR can be managed in the same session. Laparoscopic hydrocelectomy with/out hydrocelectomy and IIR closure is essential in preventing recurrence.

The Effect of Antrum Size on Weight Loss, Glucagon-Like Peptide-1 (GLP-1) Levels, and Glycemic Control Following Laparoscopic Sleeve Gastrectomy in Adolescents with Obesity and Type 2 Diabetes.

PURPOSE: The aim of this study was to compare the effect of antral resection versus antral preservation sleeve gastrectomy on the post-operative GLP-1, glycemic control, and weight loss in adolescents suffering from severe obesity and type 2 diabetes (T2D). MATERIALS AND METHODS: This study included 36 adolescents. Patients were randomly divided into 2 groups: group (A) and group (B). Each group included 18 patients who underwent LSG, starting transection at 2 cm or 5 cm from the pyloric ring in group (A) and group (B), respectively. They were followed up at 1, 3, 6, 12, and 24 months post-operatively. The outcomes were the post-operative GLP-1 response, glycemic control, weight loss, and safety. RESULTS: The improvements in the body mass index and the percentage of excess weight loss (%EWL) were statistically significant within each group. The mean GLP-1 levels showed significant increase at the 1, 3, and 6 months but not in the 12 and 24 months in all the studied samples within each group. The mean HbA1c levels and post-prandial serum C-peptide significantly improved within each group (P < 0.05). No statistical differences in the weight loss, %EWL, GLP-1, HbA1c, C-peptide changes, and complication rates were observed between both groups. Diabetic remission was significantly higher (88.9%) in group (A). CONCLUSIONS: LSG resulted in generalized significant GLP-1 initial response that decreased over time. The reduced antrum size did not influence the GLP-1 response, glycemic control, or insulin resistance, but resulted in significantly better T2D remission. Since the study examines a small number of patients, further studies are needed. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04388059.

Management of pediatric benign ovarian tumors in England and Egypt: A comparative study.

AIM OF THE STUDY: We aimed to compare the management of pediatric benign ovarian tumors between an English center and three Egyptian institutions. MATERIALS AND METHODS: This was a retrospective review of all children presenting with benign ovarian tumors between January 2014 and January 2019. A standardized dataset was used to compare between both sides. RESULTS: Eighty-nine patients were included (54 English and 35 Egyptians). Median age at diagnosis in England was 13 years (2-16y), while in Egypt it was 7 years (9m-16y) with P =0.001. Mature teratomas or dermoid cysts were the most common findings in England and Egypt; 75.9% and 82.8% of cases, respectively. The presentation with an acute abdomen represented 27.8% of English and 28.6% of Egyptian patients. Incidentally diagnosed lesions constituted 15% of English patients, whereas none of the Egyptian cases were discovered incidentally. There were variations in diagnostic imaging; England: Ultrasound (USS) (54), magnetic resonance imaging (MRI) (37), and computed tomography (CT) (only one)-Egypt: USS (35), CT (17), and MRI (only one). Minimally invasive surgery (MIS) was performed in 15% of English and 23% of Egyptian patients (P = 0.334). Ovarian-sparing surgery (OSS) was performed in: England 35%, Egypt 37%; P = 0.851. OSS was performed using MIS in 87.5% (7/8) of English patients and 100% (8/8) of Egyptians. Patients presented as emergencies generally had open oophorectomies: England; 86.7% open and 80% oophorectomy-Egypt; 100% open and 90% oophorectomy. Recurrences or metachronous disease occurred in 5.6% of English and 5.7% of Egyptian patients. CONCLUSIONS: There were no significant differences regarding surgical management, tumor pathology, and recurrence or metachronous disease. However, age, incidental diagnosis, and imaging modalities showed notable differences. MIS was correlated with ovarian preservation, whereas emergency surgery generally resulted in open oophorectomy.

Homemade Glove Retrieval Bag in Laparoscopic Complete Excision of Benign Ovarian Teratoma with Ovarian Tissue Preservation in Children: A Case Series.

STUDY OBJECTIVE: To present our experience of laparoscopic resection of pediatric benign ovarian teratomas with gonadal preservation, using a homemade glove retrieval bag. DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: Review of all girls with benign ovarian teratomas who were managed with laparoscopic ovarian-sparing surgery (OSS) at our hospital between January 2013 and December 2018. RESULTS: Eleven patients were included for analysis with a mean age of 6.1 years. Ten patients received elective surgery, whereas 1 patient received emergency surgery because of ovarian torsion. Main indication for OSS was the existence of a dissection plane between tumor margins and healthy ovarian tissue. Postoperative outcome and follow-up were uneventful with a median follow-up of 30.1 months (range; 12-60 months). CONCLUSION: Laparoscopic OSS can be safely performed for these tumors. Laparoscopic magnification with energy devices are excellent tools in such procedures. The homemade glove bag can be used to retrieve the tumor effectively in countries with limited resources.

Laparoscopic Posterolateral Suture Rectopexy for Recurrent Rectal Prolapse in Children.

Introduction: Laparoscopic rectopexy for recurrent rectal prolapse (RP) is more widely used nowadays. Strict indications are needed to get proper outcomes. The advantages rely on the careful dissection of peritoneal sac and fixation of rectum. One of the theories of recurrent RP is hernia-like physiology in front of the rectum. Purpose: The aim of this study is to present our experience of posterolateral laparoscopic suture rectopexy (LSR) in indicated children of recurrent RP. Patients and Methods: Sixteen patients were included with recurrent RP wherein all were subjected to LSR procedure after exclusion of other probable causes. Dissection of peritoneal sac anterior to the rectum was carried out followed by closure of the deep pouch by nonabsorbable sutures then fixation of the right side of rectum and sigmoid to the lateral wall of areolar tissue. Fixation to sacral promontory is done by Ethibond or Prolene sutures when redundancy is obvious. Results: The study included 11 girls and 5 boys with age ranging between 3 and 12 years in the past 5 years. Ten cases were treated earlier with injection therapy and 6 following Thiersh procedure after failure of conservative treatment for 6 months. Operative time ranged between 40 and 100 minutes. Follow-up period ranged between 6 and 36 months with mean of 19.5 months. Postoperative mucosal prolapse reported in 1 case 6 months postsurgery with no full thickness recurrence. Conclusions: LSR is an efficient technique in well-selected children of recurrent RP and could reverse this underlying pathology. Longer follow-up and evidence are needed to standardize the technique.

Algorithm of Laparoscopic Technique in Pediatric Inguinal Hernia: Results from Experience of 10 Years.

BACKGROUND: Many laparoscopic techniques have been evolved along years for pediatric inguinal hernia (PIH) with no standardization of technique. No single technique suits all varieties of hernia. PURPOSE: To propose an algorithm for allocation of PIH to laparoscopic technique based on internal ring (IR) diameter to improve outcomes. PATIENTS AND METHODS: Along 10 years, 459 cases with unilateral PIH were treated in Tanta University Hospital. In the first 5 years (phase I), 214 cases included then an algorithm for stratification was designed and applied in the second 5-year period (phase II), where 245 cases managed. This algorithm included evaluation of the hernia based on IR diameter as measured by the laparoscope from inside by a piece of suture. When the IR diameter is from 4 to 15 mm, complete sac disconnection is used. When IR diameter lies from 15 to 25 mm a purse string is added. When IR diameter is >2.5 cm or recurrent cases, the interrupted muscular arch repair after sac disconnection is used. RESULTS: In phase I, 170 boys and 44 girls from 6 to 180 months of age were treated. All cases managed by laparoscopy were 84 herniotomy, 82 by purse string, and 44 by interrupted muscular arch. In phase II, 180 boys and 65 girls from 3 to 180 months of age were included. Eighty were managed by herniotomy, 137 by purse string, and 25 by interrupted muscular arch. Recurrence rate decreased significantly in phase II. CONCLUSIONS: Application of Tanta algorithm reduces the recurrence rate significantly. The laparoscopic technique should be tailored according to criteria of each group of PIH to get the best outcome and reduced recurrence rate.

Laparoscopic Interrupted Muscular Arch Repair in Recurrent Unilateral Inguinal Hernia Among Children.

PURPOSE: We present a procedure of suturing the transversus abdominis muscular arch to the ileopubic tract laparoscopically in order to repair recurrent unilateral pediatric inguinal hernia (PIH). PATIENTS AND METHODS: Twenty-five children with recurrent unilateral PIH were treated during a 5-year period in a tertiary academic center. All cases were subjected to laparoscopic hernia repair and discharged the next morning. Sutures were placed from the muscular arch to the ileopubic tract, avoiding the spermatic vessels and duct, in an interrupted manner using 2/0-3/0 polypropylene (Prolene®; Ethicon, Somerville, NJ) or polyglactin 910 (Vicryl®; Ethicon) sutures. In 4 cases, a rectangular purse-string-like suture was added to narrow the internal ring defect. Operative findings and postoperative results and complications were assessed. The patients were followed up for a period that ranged between 6 and 60 months. RESULTS: There were 23 boys and 2 girls. Operative age ranged between 18 months and 15 years. Three or four sutures were placed in each case. In 4 cases, an additional rectangular purse-string-like suture was added. Operative time ranged between 35 and 70 minutes, and there was no conversion. Mild scrotal edema was reported in 4 cases and port-site infection in 2 cases; all cases were treated conservatively. One case of recurrence among boys was reported, but there was no case of testicular atrophy. Cosmetic outcomes were excellent. CONCLUSIONS: Laparoscopic interrupted muscular arch repair is a feasible and safe technique in the reconstruction of the inguinal canal in recurrent unilateral PIH. Larger studies and long-term follow-up are needed to support our encouraging results.

The intra-abdominal testis: lessons from the past, and ideas for the future.

The intra-abdominal testis (IAT) has been always an enigma for both diagnosis and treatment. Imaging techniques are known for low sensitivity for localizing the IAT. It has been universally accepted that the gold standard for localizing the IAT is diagnostic laparoscopy. Orchiopexy techniques for IAT are complicated and attended with a higher rate of failure and complication than those for the palpable testis. For the low-lying abdominal testis, a one-stage procedure without interruption of the vessels has a high success rate. The Prentiss maneuver bridges the borders of normal pathway to gain a straighter course to the scrotum. The interruption of the main vascular supply of the testis, depending on collateral circulation, has been used for many years but with questionable effects on the microscopic delicate structure of the testis. Microvascular autotransplantation was intended to avoid this effect, but it is technically demanding and requires special expertise. The principle of traction has been used in the past but was abandoned due to high rate of atrophy. Recently, traction has been revisited with a new approach with very encouraging results. The key to success in any technique for orchiopexy is the complete absence of tension.

Endoscopic dilatation for benign oesophageal strictures in infants and toddlers: experience of an expectant protocol from North African tertiary centre.

PURPOSE: The purpose of this study was to present the safety and effectiveness of an expectant protocol employing Savary-Gilliard dilatation in benign oesophageal stricture in infants and toddlers along a decade of experience. PATIENTS AND METHODS: Thirty eight infants and toddlers with benign oesophageal stricture with age ranged between 5 and 22 months were treated by modified dilatation protocol. Complications and outcomes of the dilatation protocol were reported during a follow-up period of 12 to 100 months. RESULTS: We have 25 cases of corrosive stricture, 4 congenital, 4 post-reflux and 5 post-anastomotic strictures. A total of 654 dilatations in 265 sessions were done. The corrosive subgroup has a mean dysphagia score of 2.6 pre-dilatation that improved at 6 months after end of dilatation to a mean of 0.3. In the non-corrosive subgroup, significant lower number of dilatation and sessions were reported. We have three failures that need surgery. Thirty five cases reach acceptable oesophageal caliber. Mucosal tear and small perforation each reported once. Small diverticulum reported twice. CONCLUSIONS: The expectant dilatation protocol is feasible in managing benign oesophageal stricture in infants and toddlers without increasing the morbidity. It is effective even in long segment or multiple corrosive strictures.

Prospective long-term functional and cosmetic results of ASARP versus PASRP in treatment of intermediate anorectal malformations in girls.

PURPOSE: Prospective randomized comparison of the functional and cosmetic results of anterior sagittal anorectoplasty (ASARP) and posterior sagittal anorectoplasty (PSARP) in the management of intermediate anorectal malformations (ARMs) in girls was performed. METHODS: Thirty-eight girls with intermediate ARMs were randomly allocated to group A treated by ASARP technique and group B treated by PSARP technique: 14 girls from each group passed 30-month age for early functional assessment by Templeton and Holschneider scores, 18 cases passed 55 months for late functional evaluation. Results were compared statistically where P value < or = 0.05 was considered significant. RESULTS: Cosmetic satisfaction was higher in ASARP group with a median score of 3 compared to 2 in PSARP group. Functional results were assessed early at median of 33.5 months post-repair where Templeton score was 4 versus 3.75 and Holschneider score was 11 versus 11. Late functional assessment was done for 8 cases from each group where Templeton score was 4 versus 4, while Holschneider score was 12 versus 11. CONCLUSIONS: ASARP is an optimal technique for treatment of intermediate ARM in girls. Cosmetic results were superior to PSARP. Despite the observations that functional results give higher scores in ASARP group versus PSARP group, no statistical significance could be reached.

Diaphragmatic injuries in children after blunt abdominal trauma.

BACKGROUND: Traumatic rupture of the diaphragm resulting from blunt abdominal trauma remains a challenging clinical entity. Description of such type of injuries in children is scarce in the literature. PURPOSE: The aim of this study was to present this special form of injury in the pediatric age group and compare the different aspects of diaphragmatic injury with that occurring in adults. PATIENTS AND METHODS: Ten cases of diaphragmatic rupture after blunt trauma in children were reported. The presentations, findings, and management were described. RESULTS: This study included 7 boys and 3 girls aged 3 to 16 years. Trauma in 8 cases resulted from motor vehicle or auto-pedestrian accidents and 2 from falling from a height. Chest radiograph shows suggestive signs of diaphragmatic injuries in 7 occasions including intrathoracic visceral herniation in 4 cases and hemothorax in 3 cases. Associated injuries were found in 5 cases in the form of rib fractures in 3 cases and lung contusion in 2 cases. Lung tear, gut perforation, and liver tear were each reported once. Isolated diaphragmatic injury is reported in 5 cases. Three cases were repaired via thoracotomy or laparo-thoracotomy and 7 cases via midline laparotomy. On exploration, we found diaphragmatic avulsion of the costal origin in 5 cases, 3 left and 2 right contrary to tears, of which 4 were left sided and 1 right sided. Primary repair was conducted in 7 cases and an intercostal muscle flap was used in 3 cases. No mortalities were reported. CONCLUSIONS: This series of diaphragmatic rupture in children reveals the following: (1) Avulsion of the costal origin of the diaphragm is a peculiar type of injury described in children (5 of 10). (2) The intercostal muscle flap is a useful tool to bridge diaphragmatic defects. (3) Isolated diaphragmatic injuries do occur in children more frequently than in adults. (4) As in adults, diaphragmatic rupture prevails in the left side, and purposeful surgical diagnosis and early management determine the effectiveness of treatment.

Pulmonary hypertension in human newborns with congenital diaphragmatic hernia is associated with decreased vascular expression of nitric-oxide synthase.

The molecular basis of the pathogenesis of pulmonary hypertension (PH) associated with congenital diaphragmatic hernia (CDH) is poorly understood. Variation in responses to therapeutic strategies such as nitric oxide (NO) inhalation and extracorporeal membrane oxygenation (ECMO) in patients with CDH remains a major problem in pediatric critical care. We investigated the expression pattern of NO-generating enzyme nitric-oxide synthase (NOS) (both endothelial [eNOS] and inducible [iNOS] isoforms) in the lungs of CDH patients with PH and evaluated the influence of ECMO on the expression levels of these genes in an attempt to understand the underlying molecular mechanisms. Lung autopsy specimens from 23 cases of CDH not treated by ECMO and 10 ECMO-treated CDH cases were studied and compared with 11 age-matched controls. Expression of iNOS and eNOS was assessed by immunohistochemistry and video-image analysis. Expression of iNOS in the endothelium of small pulmonary arteries (external diameter < or =200 Mum) was significantly lower in CDH cases that had not received ECMO treatment (p = 0.04). ECMO-treated CDH cases did not differ from controls in iNOS expression. Alveolar macrophages (CD68+ cells), of which the number also was increased, showed significantly enhanced staining for iNOS in CDH cases (p = 0.03) compared with controls. The observed decrease in pulmonary expression of iNOS in patients with CDH suggests a potential role in the pathogenesis of pulmonary hypertension in newborns with CDH. ECMO treatment was correlated with induction of this enzyme, which may result in NO-mediated vasodilatation and thereby transiently reduce the pulmonary hypertension in CDH.