The Co-Existence of Patent Omphalomesenteric Duct and Omphalocele in Patau's Syndrome in Saudi Arabia: A Case Report.

The pathophysiology of Patau's syndrome involves the triplication of chromosomes, leading to multiple comorbidities. An omphalocele is characterized by a protrusion of abdominal contents from the base of the umbilical cord through the peritoneum. An omphalomesenteric duct remnant occurs when there is a failure of duct closure that results in a diverticulum extending from the fetal midgut to the yolk sac. While congenital defects rarely occur simultaneously in patients with Patau's syndrome, this case report describes a newborn with Patau syndrome who presented with both an omphalocele and an omphalomesenteric duct remnant. The newborn exhibited various congenital abnormalities such as coloboma, microphthalmia, broad nasal bridge, cleft lip, cleft palate, low-set ears, systolic murmur, omphalocele, intestinal umbilical fistula (omphalomesenteric continuous vitelointestinal duct remnant), polydactyly, rocker-bottom feet, left-sided clubbed foot, and ruptured myelomeningocele. Imaging revealed additional complications such as a large patent ductus arteriosus, hypoplastic distal arch, markedly dilated right atrium and left ventricle, and cerebellar hypoplasia. Chromosomal analysis confirmed the diagnosis of Patau's syndrome. Given the untreatable medical condition, the patient was placed under "Do Not Resuscitate," and palliative care was initiated. The simultaneous appearance of an omphalocele and an omphalomesenteric continuous vitelointestinal duct is rare, and surgical intervention is the standard of care if the patient is deemed suitable for surgery. However, in cases where surgery is not feasible, palliative care is initiated. Regardless of the outcome, genetic counseling is essential and should include a discussion on paternal autonomy, understanding the disorder, suggesting alternative management methods, and making crucial decisions concerning future family care and planning.

Non-operative treatment for simple acute appendicitis (NOTA) in children during the COVID-19 era: new lessons from the pandemic.

Coronavirus (COVID-19) was a pandemic disease that was affecting our medical and surgical daily practice badly. The surgical management of acute appendicitis was the gold standard, but new studies suggest the safety of antibiotic treatment alone. Non-operative treatment for simple acute appendicitis (NOTA) avoids surgery, the risks of general anesthesia, and long hospital stays. It also decreases the risk of exposure to coronavirus. We aimed to study the cost-effectiveness and outcome of NOTA during the COVID-19 pandemic and compared it to single-incision pediatric endo-surgery appendectomy (SIPESA). A prospective cohort study for NOTA of patients from 6 to 12 years old in the COVID-19 pandemic period from April 1st, 2020, to April 30th, 2021, patients were divided into two groups: Group S was managed by SIPESA, and Group N was managed by NOTA. Family education and assurance with detailed explanation were done for early detection of any complications, and we continue monitoring the patients until their complete recovery. Group S had 24 cases (40%), mean age 9.3 years. Group N had 36 cases (60%), mean age 9.1 years. Six cases (17%) in group N were converted to surgical management in the first 6 months of the study. The mean cost dropped from $2736/day to $400/day. The mean psychological stress for the children improved from 4.4 in April to 2 in September. The mean follow-up was 3.5 months. NOTA is a feasible, cost-effective approach, and we recommend it, as we have learned this lesson during the COVID-19 pandemic days.

Overview of complications associated with epidermolysis bullosa: A multicenter retrospective clinical analysis of 152 cases.

BACKGROUND/PURPOSE: Epidermolysis bullosa (EB) is a rare disease of skin and mucosa which may causes surgical complications. We review these in a large patient cohort from Saudi Arabia. METHODS: A retrospective study was conducted at 21 centers between 2003 and 2020. Demographic data and information on EB type [Simplex (EBA), Dystrophic (DEB) and Junctional (JEB)]. The dataset included clinical features, operations, surgical complications, and treatment. RESULTS: There were 152 (63 male) children with EB [EBS n = 93 (61.2%); DEB n = 30 (19.7%); JEB n = 25 (16.4%), and Kindler syndrome n = 4, (2.6%)]. Children with JEB and DEB tended to have a higher frequency of skin and musculoskeletal system complications (skin cancer, pseudosyndactyly and recurrent skin infection). Esophageal strictures were mostly seen in DEB (n = 19, 63%) and to a lesser extent in EBS (n = 20, 21%) and JEB (n = 4, 16%). Pyloric atresia was uncommon (n = 4) and limited to those with JEB. Percutaneous gastrostomy for feeding support was used in all types. Ankyloglossia was common but often recurred (76%) after division. Circumcision was usually safe and complication-free in male children except in those with severe JEB. Phimosis was reported in 10% of uncircumcised patients. CONCLUSIONS: Our series showed that surgeons play a key role in the management of some complications associated with EB. It is also important to be aware of the particular sub-type as this can predict the natural history and likely response to treatment. LEVEL OF EVIDENCE: 2.

The Outcome of Pediatric Esophageal Strictures Managed with Endoscopic Balloon Dilation in Saudi Arabia.

Background: Esophageal strictures can affect nutrition of infants and children impairing their weight gain. To our knowledge, this is the first article evaluating and comparing between the two methods of dilation in terms of outcome and one of few, if any, to assess both weight and height to evaluate the nutritional outcomes following dilation. To determine the safety, efficacy, and long-term effects of endoscopic dilation in managing pediatric esophageal strictures by assessing the clinical and nutritional outcomes. Methods: A retrospective study of 137 patients with esophageal strictures who underwent either endoscopic balloon dilatation or Savary dilatation, or both. Outcome parameters measured include the number of dilatations, nutritional status, and if symptoms had been relieved. Results: The most frequent cause of esophageal strictures was post-tracheoesophageal fistula repair (n = 51, 37.2%), and the majority were lower third strictures (n = 47, 34.3%). However, 8 cases (5.8%) had failed the dilation procedure. Savary dilatation had the highest number of complications. Overall, success rate was 79.6%. Higher success rate was for cases dilated by endoscopic balloon dilation (EBD) (n = 47, 90.4%). There was a statistically significant correlation between the success rate and the method of dilatation (P = .042). Statistically significant increment of weight was recorded for lower strictures (P = .001). Conclusion: EBD was associated with the highest success rate. Endoscopic dilatations are safe and efficient in managing pediatric esophageal strictures with improvement in both clinical and nutritional outcomes.

Single-Incision Pediatric Endosurgery Cholecystectomy Performed by Residents in Children with Sickle Cell Disease.

Introduction: Few centers worldwide have advanced single-incision pediatric endosurgery (SIPES) for pediatric age group. Up to our knowledge this is the first study assessing the safety of SIPES cholecystectomy in children with sickle cell disease (SCD) done by surgical residents. Aims: To determine the feasibility, safety, and expediency of SIPES cholecystectomy in children with SCD performed by surgical residents. Materials and Methods: Retrospective study of all SIPES cholecystectomies performed in our unit from April 1, 2011 to March 31, 2018. We compared the outcome of SIPES cholecystectomy done by fellows and residents. SIPES Covidien 5-12 mm port was inserted through umbilicus. Long 50 cm laparoscope, straight regular instruments, and transabdominal gallbladder traction suture were used in all patients. The cystic duct and artery were identified and divided. The gallbladder is then dissected off the liver and extracted from the abdomen through the port. Results: Forty-three SCD patients underwent SIPES cholecystectomy for 7 years. Mean HbS was 37.56%. Mean age was 10 years. Twenty-four cases (56%) and 19 (44%) were performed by 8 fellows and 10 residents, respectively. Nine other procedures were done simultaneously with cholecystectomy and were excluded from the mean operative time (MOT). The MOT for all cases was 85 minutes, 78 minutes for fellows and 94 minutes for residents (P value is <.001). One extra port was inserted in 2 patients at the beginning of the series. Conclusion: SIPES cholecystectomy in children with SCD is safe and feasible if done by residents under supervision. Stepwise training is essential in this challenging surgery. Involving residents with other SIPES procedures and hands-on minimally invasive surgery single port workshops help them in improving their skills.

Is It Safe for Trainees to Perform Single-Incision Pediatric Endosurgery Splenectomy?

INTRODUCTION: Few centers worldwide have advanced single-incision pediatric endosurgery (SIPES) splenectomy. The safety and feasibility of SIPES performed by trainees were not assessed before. SIPES splenectomy is a demanding technique that needs high level of skills. This is the largest series of 39 SIPES splenectomies performed by 14 trainees in one single center. AIMS: To assess the safety, feasibility, and technical challenges of SIPES splenectomy performed by trainees and to compare it with other published series. MATERIALS AND METHODS: Retrospective study of all patients operated with SIPES splenectomy for 7 years. SIPES port was inserted through 1.5 cm umbilical incision. One type of port and straight regular instruments were used in all cases. RESULTS: Forty-nine patients underwent SIPES splenectomy. Eighty percent was done by our trainees. Six cholecystectomies were done simultaneously. Forty-five patients with sickle cell disease, two with thalassemia, one spherocytosis, and one Fanconi's anemia. Mean operative time (MOT) for splenectomy was 182 minutes (130-190) and 251 minutes for splenectomy with cholecystectomy (230-270) depending on severity of adhesions and size of the spleen; P value <.001. Two conversions have to be opened due to bleeding. There is neither wound infection nor incisional hernia up to date. CONCLUSION: SIPES splenectomy is safe and feasible when performed by surgical trainees without adding any morbidity to the patient. Learning curve can quickly improve with more exposure of trainee to different SIPES procedures and hands-on workshops. More than one procedure could be done at the same time. It has excellent cosmesis and almost invisible scar.