Laparoscopic Cholecystectomy Operative Time and Hospital Stay Differences Between Sicklers and Non-sicklers: A Five-Year Comparative Cross-Sectional Study at King Abdulaziz Medical City, Jeddah.

Background Sickle cell disease (SCD) is one of the most important hemoglobinopathies that result in the formation of pigment gallstones. Laparoscopic cholecystectomy (LC) is a safe surgical procedure for gallstones in SCD. Currently, there is no standard or guidelines for the preoperative preparation of these patients. This study aims to estimate the difference in pre-operative length of stay, operative time, postoperative length of stay, and total hospitalization length of stay among patients with and without SCD in a tertiary hospital in Jeddah, Saudi Arabia. Such knowledge would aid in establishing a standard for the preoperative preparation of SCD patients for LC. Methods Data from all patients undergoing laparoscopic cholecystectomy between January 2013 and December 2018 were collected retrospectively with a comparative cross-sectional study design. Data included age, sex, BMI, clinical presentation, mode of admission (elective or emergency), preoperative length of stay, operative time, postoperative length of stay, total hospitalization length of stay, and type of surgery (day or inpatient). JMP 15.2.1 was used for statistical analysis. Results From 2013 to 2018, 793 patients underwent LC, and of those, 16 (2.018%) were SCD patients. The results showed significant differences in preoperative (p<0.001), postoperative (p<0.001), and total hospitalization stay time (p<0.001) between the SCD patients and non-SCD patients. However, the data show no significant difference in the operative time of LC between the two studied groups. Conclusion SCD is the most common hemoglobinopathy-causing gallstone. Challenges in these patients are mainly in peri-operative management. Further prospective cohort studies are needed to create a standardized approach for peri-operative management of SCD patients to facilitate delivery of the same level of care and shorten total hospitalization time.

Gastrointestinal Basidiobolomycosis in pediatric patients: A diagnostic dilemma and management challenge.

INTRODUCTION: Basidiobolomycosis is a rare fungal disease, lately appearing in the gastrointestinal system of pediatric patients. Their clinical presentation resembles that of lymphoma or granulomatous inflammations. This non-specific presentation makes Gastrointestinal Basidiobolomycosis (GIB) a diagnostic challenge. METHODS: We are reporting the largest series of pediatric GIB, from Saudi Arabia. 12 patients were diagnosed between January 2012 and December 2019, between the ages of 16 months and 8 years. RESULTS: The most common symptoms were fever and abdominal pain. Further examination revealed an abdominal mass. Biopsy of the mass was the mainstay of diagnosis, with histological findings of typical filamentous fungal hyphae and zygospores, surrounded by eosinophils. CONCLUSION: Role of surgery was limited to establishing the diagnosis and dealing with complications. Antifungal medication was the cornerstone of treatment in all our patients. Three of our patients were exceptional with complications such as entero-cutaneal fistula, entero-enteric fistula and short bowel syndrome. These complications have not been previously reported. We have discussed the challenges related to their management.The diagnosis of GIB in pediatric patients with abdominal mass, needs a high index of suspicion. We believe outcome depends on the severity of disease, involvement of surrounding tissues and presence of complications at the time of diagnosis.

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.

Biliary atresia with an unusual abdominal orientation: A case report.

INTRODUCTION: Biliary atresia (BA) is a rare condition where the hepatic or common bile ducts get obliterated by fibrous tissue for unknown causes. It is diagnosed in 5-10 per 100,000 live births worldwide in whom liver injury and death are definite if untreated. PRESENTATION: We report a case of BA with associated malrotation and situs ambiguous discovered incidentally during Kasai procedure. The small intestine was found to be malrotated with the duodenojejunal junction to the right of the vertebral column. The liver, stomach, and spleen were in the center, right side, and left side of the abdomen respectively. The malrotation was corrected by performing a Ladd's procedure in addition to the Kasai. She had an uneventful postoperative course. At follow-up on the second, sixth and 12th weeks postoperatively, she had normal stool color and liver function. DISCUSSION: BA patients with associated structural anomalies might have a worse outcome after the Kasai procedure. Several studies demonstrated that the coexistence of malrotation with BA to be in the range of 0.3%-9.4% among BA patients. Also, the presence of situs abnormalities with BA has been reported in some studies to be present in 0.01-0.045% of BA cases. However, the presentation of BA with both situs inversus and intestinal malrotation has seldom been reported. CONCLUSION: The diagnosis of BA should prompt further investigation for other anatomical abnormalities as the presence of which might affect the management plan.

Laparoscopic cholecystectomy for cholelithiasis in children with sickle cell disease.

Patients with sickle cell disease (SCD) suffer from an increased incidence of gallstone formation due to hemolysis of sickled red blood cells; this leads to an increased level of bilirubin in secreted bile that becomes a nidus for pigment stone formation. Laparoscopic cholecystectomy (LC) is considered a standard operative procedure for gallstone disease mainly due to lower postoperative wound complaints, faster recovery, better postoperative cosmetic results, shorter hospital stay, and earlier return to work. Although numerous studies have been published addressing both the advantages and complications of LC in acute calcular cholecystitis, there is still limited evidence concerning the safety and efficacy of LC for the management of cholelithiasis in pediatric patients with SCD, and controversies remain unresolved. In this review, we aim to comprehensively study the available literature and propose evidence-based practice recommendations for the optimal management of gallstones in pediatric SCD patients. The current practice differs greatly depending on the prevalence of SCD in a particular geographic area. We acknowledge the limited number of patients reported, the lack of randomized control trials addressing the practice of specific recommendations, and the need for further evidence-based studies.

Anaplastic Sarcoma of the Kidney: A Rare Unique Renal Neoplasm.

Anaplastic sarcoma of the kidney (ASK) is an extremely rare tumor, which usually presents as a large renal mass. Microscopically, the tumor is composed of pleomorphic mesenchymal spindle cells with marked atypia, associated with chondroid differentiation and focal round primitive mesenchymal cells. Herein, we present a case of anaplastic sarcoma of the kidney in a 3-year-old female, who presented with a large abdominal mass. To the best of our knowledge, less than 25 cases are reported in the literature. In addition, this is the first case reported from the Middle East.

Unusual presentation of a retained foreign body in a child.

A 4-year-old boy, not known to have any medical illness, presented to several medical facilities reporting right upper quadrant and right flank pain, intermittent fever and chills for 3 months. A CT of the abdomen showed that a swallowed 'bobby pin' had pierced through the right kidney. This finding explained the boy's symptoms. The pin was removed by laparotomy without any subsequent complication. The patient had an uneventful postoperative recovery.

IgG4-related sclerosing mesenteritis in a 7-year-old Saudi girl.

Sclerosing mesenteritis (SM) is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently maintained on 5 mg prednisone daily and no recurrence of symptoms was noted during the 24-month follow-up period. We emphasize, therefore, that SM can present clinical challenges and the presence of SM should cue clinicians to search for other coexisting autoimmune disorders that can have various outcomes.

Abdominal compartment syndrome secondary to chronic constipation.

Abdominal compartment syndrome (ACS) is defined as an elevated intraabdominal pressure with evidence of organ dysfunction. The majority of published reports of ACS are in neonates with abdominal wall defects and in adults following trauma or burns, but it is poorly described in children. We describe the unusual presentation of an 11-year-old boy with a long history of chronic constipation who developed acute ACS requiring resuscitative measures and emergent disimpaction. He presented with a 2-week history of increasing abdominal pain, nausea, diminished appetite and longstanding encopresis. On exam, he was emaciated with a massively distended abdomen with a palpable fecaloma. Abdominal XR confirmed these findings. Within 24 hours of presentation, he became tachycardic and oliguric with orthostatic hypotension. Following two enemas, he acutely deteriorated with severe hypotension, marked tachycardia, acute respiratory distress, and a declining mental status. Endotracheal intubation, fluid boluses, and vasopressors were commenced, followed by emergent surgical fecal disimpaction. This resulted in rapid improvement in vital signs. He has been thoroughly investigated and no other condition apart from functional constipation has been identified. Although ACS secondary to constipation is extremely unusual, this case illustrates the need to actively treat constipation and what can happen if it is not.

Investigating the use of preoperative nasogastric tubes and postoperative outcomes for infants with pyloric stenosis: a retrospective cohort study.

PURPOSE: Literature is lacking regarding the role of nasogastric tubes in patients with pyloric stenosis. There is also no consensus among surgeons. Some believe that pyloric stenosis is a form of gastric outlet obstruction, and the stomach should be drained until the obstruction is relieved. Others claim that infants can handle their secretions, and draining the stomach may further exacerbate the alkalosis. This chart review examines the use of preoperative nasogastric tubes in a single pediatric institution and its effect on vomiting rates and length of stay. METHODS: After research ethics board approval, a retrospective review was performed on 109 patients admitted between January 1, 2007, and December 31, 2008, with pyloric stenosis who underwent pyloromyotomy. Data were collected on presence of a preoperative nasogastric tube, preoperative electrolyte levels, ultrasound characteristics, episodes of postoperative vomiting, and length of stay. RESULTS: One hundred six patients were used in the final analysis. A nasogastric tube was placed in 77 patients (73%). Patients with a preoperative nasogastric tube had significantly higher episodes of postoperative vomiting (P = .015; 95% confidence interval [CI] 0.29-2.63) and length of stay (P = .017; 95% CI, 2.49-25.01). Bicarbonate levels were also significantly higher in patients with a nasogastric tube. There was no difference in the duration of symptoms, ultrasound characteristics, or type of operation between the 2 cohorts. CONCLUSION: The data strongly suggest that preoperative nasogastric tube placement adversely affects postoperative vomiting and consequently increases length of stay. The lack of consensus about the use of preoperative nasogastric tubes coupled with these findings indicates the need to evaluate this practice with a prospective randomized controlled trial.

Laparoscopic excision of infarcted accessory spleen.

An accessory spleen is present in about 10-30% of the population and, usually, does not cause symptoms. We present a case report of an unusual presentation of accessory spleen infarction, with a literature review. A 12-year old male presented with acute left-upper quadrant pain that slowly resolved. An ultrasound and computed tomography scan showed a 3.5 x 2.5 x 2 cm solid mass abutting and displacing the splenic flexure of the colon, with surrounding inflammatory changes. This was interpreted as a colonic duplication cyst, and the boy was treated with antibiotics and underwent elective laparoscopic exploration. It was removed laparoscopically without complication and, on pathologic examination, proved to be consistent with an infarcted accessory spleen. Less than two dozen similar cases of accessory spleen infarction have been reported in the literature, most presenting with acute abdominal pain. Preoperative diagnoses included appendicitis, ovarian torsion, neoplasm, and, in our case, colonic duplication. The natural course of infarcted accessory spleen would be to atrophy, but, even with advanced imaging techniques, it may be impossible to diagnose infarcted accessory spleen with enough confidence to avoid surgery.