Crusted Scabies in a Pediatric Liver Transplant Recipient on Immunosuppression.

BACKGROUND Crusted scabies is a severe skin infection resulting from hyper-infestation with the obligate parasite Sarcoptes scabiei var. hominis. In contrast to classic scabies, crusted scabies may involve as many as hundreds to millions of mites. Importantly, this condition is associated with a mortality rate of 60% in 5 years, and is more likely to develop in immunosuppressed hosts, presumably due to an impaired T-cell response against the mite. CASE REPORT We present a case of crusted scabies in a 13-year-old girl during her early post-liver transplant period receiving immunosuppression successfully treated with topical 5% permethrin. She had pruritic erythematous papules, confirmed as scabies through skin scraping. The challenge of misdiagnosis during the initial presentation is noteworthy, given atypical manifestations and more common differentials. To our knowledge, only 2 cases of crusted scabies in pediatric solid-organ transplant recipients have been reported. Despite the absence of guidelines for pediatric solid-organ transplant recipients, urgent treatment is required due to the high associated mortality rates. In our case, early treatment proved successful without any secondary bacterial infections or clinical evidence of relapse during a 6-month follow-up. CONCLUSIONS We report a case of scabies in a pediatric liver transplant recipient that was successfully treated. It is crucial to consider the diagnosis of scabies given the associated morbidity and the risk of secondary bacterial infections among other more common differentials. Notably, the response to standard anti-scabies treatment may lead to resolution without relapse in pediatric solid-organ transplant recipients.

Navigating Inconclusive Upper-Gastrointestinal Series in Infantile Bilious Vomiting: A Case Series on Intestinal Malrotation.

BACKGROUND Bilious vomiting in a child potentially portends the dire emergency of intestinal malrotation with volvulus, necessitating prompt surgical management, with differentials including small-bowel atresia, duodenal stenosis, annular pancreas, and intussusception. Although the upper-gastrointestinal series (UGI) is the diagnostic investigation of choice, up to 15% of the studies are inconclusive, thereby posing a diagnostic challenge. CASE REPORT We report a case series of 3 children referred for bilious vomiting, whose initial UGI was inconclusive and who were eventually confirmed to have intestinal malrotation at surgery. The first child was a female born at 37 weeks with antenatally diagnosed situs inversus and levocardia, who developed bilious vomiting on day 1 of life. The duodenojejunal flexure (DJ) could not be visualized on the UGI because of faint opacification on first pass of the contrast and subsequent overlap with the proximal jejunal loops. The second child was a male born at 36 weeks, presenting at age 4 months with bilious vomiting of 2 days duration. The third child was a female born at 29 weeks, presenting with bilious aspirates on day 3 of life. UGI for all 3 showed persistent hold-up of contrast at the proximal duodenum with no opacification of the distal duodenum or small bowel.Adjunctive techniques during the UGI and ultrasound examination helped achieve a preoperative diagnosis of malrotation in these children. CONCLUSIONS Application of diagnostic adjuncts to an inconclusive initial UGI may help elucidate a preoperative diagnosis of intestinal malrotation in infantile bilious vomiting.

An Intelligent Customer-Driven Digital Solution to Improve Perioperative Health Outcomes Among Children Undergoing Circumcision and Their Parents: Development and Evaluation.

BACKGROUND: Circumcision as a common elective pediatric surgery worldwide is a stressful and anxiety-inducing experience for parents and children. Although current perioperative interventions proved effective, such as reducing preoperative anxiety, there are limited holistic solutions using mobile apps. OBJECTIVE: This paper aims to describe the development and primary evaluation of an intelligent customer-driven smartphone-based app program (ICory-Circumcision) to enhance health outcomes among children undergoing circumcision and their family caregivers. METHODS: Based on the review of the literature and previous studies, Bandura's self-efficacy theory was adopted as the conceptual framework. A multidisciplinary team was built to identify the content and develop the apps. Semistructured interviews were conducted to evaluate the ICory-Circumcision. RESULTS: The ICory-Circumcision study was carried out from March 2019 to January 2020 and comprised 2 mobile apps, BuddyCare app and Triumf Health mobile game app. The former provides a day-by-day perioperative guide for parents whose children are undergoing circumcision, while the latter provides emotional support and distraction to children. In total, 6 participants were recruited to use the apps and interviewed to evaluate the program. In total, 4 main categories and 10 subcategories were generated from content analysis. CONCLUSIONS: ICory-Circumcision seemed to lean toward being useful. Revisions to ICory-Circumcision are necessary to enhance its contents and features before advancing to the randomized controlled trial. TRIAL REGISTRATION: ClinicalTrials.gov NCT04174404; https://clinicaltrials.gov/ct2/show/NCT04174404.

Percutaneous transhepatic interventional therapy of portal vein stenosis in paediatric liver transplantation: A systematic review of efficacy and safety.

PURPOSE OF THE SYSTEMATIC REVIEW: To determine the efficacy and safety of percutaneous trans-hepatic balloon and/or stent angioplasty (PTA) in the management of portal vein (PV) stenosis following paediatric liver transplantation. METHODS: Articles were included from a systematic search of Medline, Embase, Cochrane CENTRAL, ClinicalTrials.gov and International Clinical Trials Registry from inception to the 29th of August 2020. RESULTS: There were 213 paediatric liver recipients who underwent PTA for PV stenosis in 19 included studies published between 1991 and 2019. Balloon angioplasty was the initial treatment in the majority (n = 153). Primary stent placement (n = 34) was performed for elastic recoil, intimal tears and PV kinks and rescue stent placement (n = 14) for recurrent PV stenosis following primary balloon angioplasty. The technical success was 97.6%-100% overall, 97.6%-100% for balloon-angioplasty-only and 100% for primary stenting. The clinical success was 50%-100% overall, 50%-100% for balloon-angioplasty-only and 100% for primary stenting. Long-term PV patency was 50%-100% overall, 37.5%-100% for balloon-angioplasty-only and 100% for primary stenting. Primary balloon angioplasty was successful in 78% of the cases. Of the recurrent PV stenoses, 9% resolved with stent placement and one required a meso-Rex shunt. There was one re-transplantation without stenting. The complication rate was 2.6% for balloon-angioplasty-only (bleeding, liver abscess, 2 PV thromboses) and 5.9% for primary stenting (bleeding, stent-fracture). There was no procedure-related mortality. CONCLUSION: Percutaneous transhepatic balloon angioplasty may be the initial management of portal vein stenosis in paediatric liver recipients. Stent placement may be a primary option in selected cases and a reliable rescue option for recurrent portal vein stenosis following balloon-angioplasty-only.

Laparoscopic versus open appendectomy in pediatric patients with complicated appendicitis: a meta-analysis.

BACKGROUND: Acute appendicitis is a common condition in the pediatric population. In patients with uncomplicated appendicitis, laparoscopic appendectomy (LA) is preferred as compared to open appendectomy (OA). However, in patients with complicated appendicitis (CA), as defined as suppurative, gangrenous or perforated appendicitis, or appendicitis with periappendicular abscess formation, the decision to perform OA or LA remains unclear. METHODS: The PRISMA guidelines were adhered to. An electronic database search from 1997 to 2017 was performed using the Cochrane, Medline, PubMed, Scopus, Ovid, Embase, and Web of Knowledge databases. Data analysis, including subgroup analysis of randomized-control trials, was performed using RevMan 5.3. Assessment of methodological and statistical heterogeneity, as well as publication bias of the included studies, was performed. RESULTS: Six randomized-control trials (296 LA versus 373 OA) and 33 case-control trials (3106 LA versus 4149 OA) were analyzed. Compared to OA, LA has a shorter length of stay (WMD = - 0.96, 95% CI - 1.47 to - 0.45) and a lower rate of surgical site infection (OR 0.37, 95% CI 0.25-0.54), although the rates of intraabdominal abscess formation were similar (OR 1.01, 95% CI 0.71-1.43). LA was also shown to have lower readmission rates, lower incidences of postoperative ileus or intestinal obstruction, lower incidence of reoperation, as well as a shorter time taken to oral intake. Operative time for OA was shorter than LA (WMD = 12.44, 95% CI 2.00-22.87). CONCLUSION: While studies in the past have associated LA with higher rates of intraabdominal abscess in patients with CA, our meta-analysis has shown that they were similar. Considering this, together with other improved postoperative outcomes, LA should be the procedure of choice in pediatric patients presenting with CA.

Portal vein reconstruction in pediatric living donor liver transplantation for patients younger than 1 year with biliary atresia.

BACKGROUND/PURPOSE: Infants with biliary atresia undergoing living donor liver transplantation (LDLT) are at increased risk of portal vein (PV) complications because of their smaller vascular caliber and sclerosis because of previous Kasai portoenterostomy and recurrent cholangitis. METHOD: Of 154 children who underwent transplantation between November 2005 and January 2011, 34 with biliary atresia received a transplant while younger than 1 year. Six patients underwent PV reconstruction with an interposition vein graft, and the others underwent the branch patch technique. The clinical characteristics of those who underwent the interposition reconstruction or the branch patch technique were compared, and the PV complications were assessed. RESULTS: Portal vein complications occurred in 5 patients (14.7%) in the branch patch group. There were 4 patient deaths, and all of them had received branch patch reconstruction. The branch patch reconstruction cases with a sclerotic small caliber (<4 mm) determined by using preoperative ultrasonography showed a significantly high mortality rate (44.4%). All patients with interposition vein graft reconstruction are still alive with excellent graft function without anticoagulation therapy. CONCLUSION: The interposition vein graft appears to be a feasible option with better graft survival and less PV complications when performing LDLT for biliary atresia in infants younger than 1.

Type 1 Bland Sutton colonic atresia complicated by fetalis hydrops in a premature neonate.

Colonic atresia (CA) is an unusual cause of neonatal intestinal obstruction where a section of the colon has failed to form, leading to blockage or absence. A premature baby was delivered at 32 weeks of gestation via caesarian section following fetal distress. She was grossly oedematous and diagnosed with severe fetalis hydrops secondary to anaemia. She was resuscitated and stabilized. On the sixth day of life, the neonate's abdomen became severely distended with billous vomiting and failure to pass meconium. We suspected intestinal obstruction and performed an omnipaque enema which revealed dilated small bowel loops and a bowel atresia. Exploratory laparotomy confirmed a Type 1 Bland Sutton CA with mucosal web. An end colostomy was successfully performed and uneventful. In our case report, we describe a rare occurrence of postnatally diagnosed CA, complicated by fetalis hydrops and anaemia.

Anterior urethral valves.

We studied the clinical presentation and management of four patients with anterior urethral valves; a rare cause of urethral obstruction in male children. One patient presented antenatally with oligohydramnios, bilateral hydronephrosis and bladder thickening suggestive of an infravesical obstruction. Two other patients presented postnatally at 1 and 2 years of age, respectively, with poor stream of urine since birth. The fourth patient presented at 9 years with frequency and dysuria. Diagnosis was established on either micturating cystourethrogram (MCU) (in 2) or on cystoscopy (in 2). All patients had cystoscopic ablation of the valves. One patient developed a postablation stricture that was resected with an end-to-end urethroplasty. He had an associated bilateral vesicoureteric junction (VUJ) obstruction for which a bilateral ureteric reimplantation was done at the same time. On long-term follow-up, all patients demonstrated a good stream of urine. The renal function is normal. Patients are continent and free of urinary infections. Anterior urethral valves are rare obstructive lesions in male children. The degree of obstruction is variable, and so they may present with mild micturition difficulty or severe obstruction with hydroureteronephrosis and renal impairment. Hence, it is important to evaluate the anterior urethra in any male child with suspected infravesical obstruction. The diagnosis is established by MCU or cystoscopy and the treatment is always surgical, either a transurethral ablation or an open resection. The long-term prognosis is good.

Sclerosing encapsulating peritonitis in a child secondary to peritoneal dialysis.

Sclerosing encapsulating peritonitis, or "abdominal cocoon," is a rare but serious complication of continuous ambulatory peritoneal dialysis. It is characterized by the diffuse appearance of marked sclerotic thickening of the peritoneal membrane resulting in intestinal obstruction. A 14-year-old adolescent boy with a history of end-stage renal failure on continuous ambulatory peritoneal dialysis presented with symptoms of acute intestinal obstruction. A computed tomography scan of the abdomen revealed distended small bowel loops clustered and displaced to the right upper quadrant. The overlying peritoneum was markedly thickened and calcified. Laparotomy confirmed the diagnosis of sclerosing encapsulating peritonitis and the patient was treated with excision of the fibrocollagenous membrane. Postoperatively, he had prolonged ileus requiring parenteral nutritional support and peritoneal dialysis was restarted on postoperative day 10. A high degree of cognizance is needed to facilitate diagnosis and treatment of this uncommon and potentially life-threatening condition.

Urogenital sinus, rectovaginal fistula, and an anterior stenosed anus--another cloacal variant.

The persistent cloaca is one of the most complex and challenging developmental malformations. It is a rare anomaly occurring only in the female newborn and is represented by the association of urogenital sinus with an anorectal malformation (ARM). Each case is probably unique. We report here one such case of cloaca with the VATER association-tracheoesophageal fistula (TOF) with a urogenital sinus, rectovaginal fistula, and an anteposed stenosed anus, along with preaxial syndactyly of the right hand. The spine, renal, and cardiac systems were normal. Interim management was directed towards repair of the TOF and a right transverse defunctioning colostomy. Despite thorough radioendoscopic preoperative investigations, the complexity of the cloacal anomaly was not delineated until surgery. This case is a rather rare combination of an intermediate form of the cloacal-ARM spectrum. Such patients present with many diagnostic and therapeutic problems. Interval surgery should be directed towards decompression of the affected organ systems, and definitive surgery must be carefully planned and, whenever possible, done in a single stage with simultaneous multisystem repair to correct all significant malformations related to the cloacal complex.