Thrombotic Features as the Primary Cause of SARS-CoV-2 Related Acute Abdomen in Children.

OBJECTIVES: We performed a retrospective case control study to evaluate the histological characteristics of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) positive pediatric patients undergoing laparoscopic exploration for acute abdomen symptoms. To our knowledge this is the first study that analyzes histopathological characteristics of abdominal tissues in SARS-CoV-2 children. STUDY DESIGN: We enrolled 8 multisystem inflammatory syndrome in children (MIS-C) patients and 4 SARS-CoV-2 positive patients who underwent intestinal resection versus 36 control appendectomies from 2 pediatric tertiary referral centers between March 2020 and July 2021. Surgical resection samples were evaluated on several histological sections focusing on general inflammatory pattern and degree of inflammation. Peculiar histological features (endotheliitis and vascular thrombosis) were semi-quantitatively scored respectively in capillary, veins, and arteries. RESULTS: All SARS-CoV-2 related surgical samples showed thrombotic patterns. Those patterns were significantly less frequent in SARS-CoV-2 negative appendectomies ( P = 0.004). The semi-quantitative score of thrombosis was significantly higher ( P = 0.002) in patients with SARS-CoV-2 related procedures. CONCLUSIONS: Our results showed that SARS-CoV-2 can cause thrombotic damage in abdominal tissues both in the acute phase of the infection (SARS-CoV-2 related appendectomies) and secondary to cytokine storm (MIS-C).

Testicular torsion during the COVID-19 pandemic: Results of a multicenter study in northern Italy.

INTRODUCTION: The literature reported an increased avoidance of the Emergency Department (ED) during COrona VIrus Disease 19 (COVID-19) pandemic, causing a subsequent increase of morbidity and mortality for acute conditions. Testicular torsion is a surgical emergency, which can lead to the loss of the affected testicle if a delayed treatment occurs. As testicular loss is time-related, outcome was hypothesized to be negatively affected by the pandemic. OBJECTIVE: The aim is to investigate whether presentation, treatment and outcomes of children with testicular torsion were delayed during COVID-19. STUDY DESIGN: Medical records of pediatric patients operated for testicular torsion of six Paediatric Surgical Units in Northern Italy between January 2019 and December 2020 were retrospectively reviewed. Patients were divided as for ones treated during (dC) or before the pandemic (pC). To reflect possible seasonality, related to lockdown restrictions, winter and summer calendar blocks were also analysed. For all cohorts, demographic data, pre-operative evaluation, operative notes and post-operative outcomes were reviewed. Primary outcomes were referral time, time from diagnosis to surgery and ischemic time, while secondary outcomes were orchiectomy and atrophy rates. Statistic was conducted as appropriate. RESULTS: A total of 188 patients with acute testicular torsion were included in the study period, 89 in the pre-COVID-19 (pC) period and 99 during COVID-19 (dC). Time from symptom onset to the access to the Emergency Department (T1) was not different among the two populations (pC: 5,5 h, dC: 6 h, p 0.374), and similarly time from diagnosis to surgery (pC: 2,5 h, dC: 2,5 h, p 0.970) and ischemic time (pC: 8,2 h, dC: 10 h, p 0.655). T1 was <6 h in 46/99 patients (46%) pC and 45/89 patients (51%) dC (p = 0.88, Fisher's exact test). Subgroup analysis accounting for different lockdown measures, confirm the absence of any difference. Orchiectomies rate was 23% (23/99) dC and 21% (19/89) pC (p = 0.861, Fisher's exact test) and rate of post-operative atrophy was 9% dC (7/76) and 14% pC (10/70), p = 0,44, Fisher's exact test. DISCUSSION: Despite worldwide pediatric ED accesses reduction, we reported that neither ischemic time nor the long-term outcomes in children with testicular torsion increased during the COVID-19 pandemic. In the available literature, few studies investigated the topic and are controversial on the results. Similarly to our findings, some studies found that timing and orchiectomy rates were not significantly different during the pandemic, while others reported a correlation to pandemic seasonality. Furthermore, in the recent pediatric literature it has been reported a delayed testicular torsion diagnosis due to shame in informing parents. Strengths of this study are the large numerosity, its multicentric design and a long study period. Its main limitation is being retrospective. CONCLUSIONS: We reported our large cohort from one of the most heavily COVID-19-affected regions, finding that referral, intra-hospital protocols and ischemic time in testicular torsion were not increased during to the pandemic, as well as orchiectomy rate and atrophy.

Currarino syndrome: does the presence of a genetic anomaly correlate with a more severe phenotype? A multicentre study.

BACKGROUND/PURPOSE: Currarino syndrome (CS) phenotype, initially described as the triad of hemisacrum, anorectal malformation (ARM) and presacral mass, can be extremely variable. The triad is often incomplete and 3 main CS phenotypical subtypes have been described: Complete, Mild and Minimal. Various associated malformations are often present. Mutations in the MNX1 gene are the main genetic background of CS, although they are not present in almost half of the cases. Aim of our study is to analyze the distribution of the 3 CS subtypes and the incidence of associated malformations in a large sample of patients and to add information about the role of the genetic testing in guiding the diagnostic and prognostic evaluation of CS patients. METHODS: A multicentre retrospective data collection was performed. CS patients' phenotype was accurately analyzed according to a diagnostic-therapeutic standardized data collection sheet. The distribution of the three CS types and the frequency of each associated malformation were calculated. The phenotype of the patients with a known genetic anomaly was compared to the phenotype of the population with no genetic diagnosis, in order to determine whether the presence of a known genetic defect could correlate with a more severe CS phenotype. RESULTS: Data from 45 patients were analyzed. Twenty patients (44.5%) presented a Complete CS type, 19 (42.2%) a Mild CS and 6 (13.3%) a Minimal CS. In addition to the classical triad elements, 38 (84.5%) patients showed associated anomalies. The group of patients who resulted positive for a MNX1 mutation comprised a higher number (56.5%) of Complete CS cases than the group of patients that did not carry any MNX1 mutation (13%) (p = 0.0085). We could not find any relationship between CS subtype and the number of associated anomalies (p = 0.5102). CONCLUSIONS: The presence of a MNX1 mutation seems to correlate with a more severe CS phenotype. MNX1 seems the main responsible for the expression and the severity of the CS triad, while the associated anomalies appear to be prevalently determined by genes sited on different loci. A thorough multidisciplinary diagnostic overview of CS patients should always include genetic counseling and analysis, both in postnatal and prenatal settings. TYPE OF STUDY: Retrospective Study. LEVEL OF EVIDENCE: II.

"Spiral intestinal lenghtening and tailoring (SILT)" for a child with severely short bowel.

We report a child with post-surgical short bowel state who underwent bowel expansion followed by spiral intestinal lengthening and tailoring (SILT) at 10 months of age. Growth at 1-year follow-up is along the 15-25th centile on 82 % oral calories as normal diet and 18 % as parenteral nutrition, and he is passing 2-3 semisolid motions daily. SILT is a versatile technique for reconstructing dilated bowel towards improved propulsion and absorption, and has a role in the management of the short bowel state.

Gastric Burkitt's lymphoma in a child: A rare case.

Burkitt's lymphoma (BL) is a high-grade lymphoma which represents 8-10% of all tumors in individuals younger than 15 years old. It may occur as an abdominal tumor, which in rare cases may include gastric mucosa, although in most cases, the tumor is located in the ileum or cecum. Primary gastric lymphoma constitutes as low as 1.48% of all gastric cancers in children. In this study, we described a case of gastric BL in a 4-year-old child, presenting discontinuous abdominal pain, weight loss, constipation and irritability. Despite its rarity in children, this tumor should be treated aggressively and long-term survival has been reported.

Sibling vesicoureteral reflux in twins.

AIM OF STUDY: Few studies have evaluated the incidence of familial vesicoureteral reflux (VUR) among multiple gestation births. The purpose of this study was to determine the incidence and characteristics of VUR in twins in a large cohort of families with VUR. METHODS: Between 1998 and 2010, data were collected on 251 families (538 children) with at least 2 siblings who had VUR. The incidence of affected twins among the families was analysed. Data were assessed for age at presentation, gender, grading of VUR, laterality of affectation and renal scarring. VUR was diagnosed by voiding cystourethrography (VCUG) and dimercaptosuccinic acid (DMSA) scans were used to assess renal scarring. RESULTS: There were 12 families (4.8%) in which twins were affected with VUR. There were 12 index cases (7 males/5 females), and 12 siblings (1 male/11 females). All index cases presented with urinary tract infection (UTIs), whereas 2 siblings had UTIs and 10 were screened. All cases presented between 3 months and 2 years. Among the index cases, three had unilateral and nine had bilateral VUR. Among the siblings, four had unilateral and eight had bilateral VUR. Thirty-seven (90%) of the 41 affected renal refluxing units had high-grade VUR. Three index cases had renal scarring. No scarring was seen in the siblings. CONCLUSIONS: The vast majority of twins with VUR are females who have high grade VUR and a low incidence of renal scarring. Twins with VUR may represent a genetically different subgroup with female preponderance and severe reflux.

A thoracic wall lipoblastoma in a 3-month-old infant: A case report and review of the literature.

Lipoblastoma is a rare benign tumor of adipose tissue seen almost always in infancy and early childhood. Lipoblastoma is present in 2 forms which are pathologically identical: circumscribed and diffuse. It is typically located in the extremities, and less frequently in head-neck region, trunk, and various organs. Lipoblastoma is a tumor with good prognosis with no reported metastases, despite its potential for local invasion and rapid growth. Our patient was a 3-month-old boy who was brought to our clinic for rapidly growing mass in the right hemithorax. With the aim of both diagnosis and treatment, the lipomatous mass was removed by local resection. In addition to the patient's age, histologic and cytogenetic analyses assisted the diagnosis of diffuse lipoblastoma. In the postoperative period, the thorax wall was unaffected, and after 6 years of follow-up no recurrence was observed. In the English literature, 8 cases of thoracic wall lipoblastoma have been previously reported, and only 3 of diffuse form. Here, we report, at our knowledge, the fourth case of thoracic diffuse lipoblastoma, in which cytogenetic analysis showed a previously undescribed karyotype aberration involving chromosomes 8, 13, and 16.