Publications by authors named "Fabrizio Gennari"

29 Publications

  • Page 1 of 1

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

J Pediatr Urol 2022 Jun 12. Epub 2022 Jun 12.

Paediatric Urology, Regina Margherita Children's Hospital, Turin, 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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpurol.2022.06.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9188680PMC
June 2022

Ovarian torsion in the pediatric population: predictive factors for ovarian-sparing surgery-an international retrospective multicenter study and a systematic review.

Arch Gynecol Obstet 2022 Jun 25. Epub 2022 Jun 25.

Department of Pediatric Surgery "Spirito Santo", Hospital of Pescara "G.D'annunzio", University Chieti-Pescara, Pescara, Italy.

Study Objective: Ovarian torsion (OT) in pediatric age is a challenging condition to diagnose and treat. To date, there is still no clear consensus about its management. Our aim was to assess some possible associated factors that can help surgeons in decision-making.

Design: We conducted a retrospective multicentric study of pediatric OT surgically treated between 2010 and 2020 in six Italian and German institutions, comparing our findings with a literature review of the last 10 years (2010-2020).

Participants: Patients aged 0-18 years with a diagnosis of OT intraoperatively confirmed and surgically treated at the involved institutions.

Results: Ninety-seven patients with a mean age at diagnosis of 8.37 years were enrolled in the study. Severe abdominal pain was present in 82 patients (84.5%). Eighty children (82.5%) presented an enlarged ovary with an US diameter > 5 cm and only 32 (40%) of them underwent conservative surgery. A laparoscopic approach was performed in 60 cases (61.9%) although in 15 (15.5%) conversion to open surgery was deemed necessary. A functional cyst was present in 49 patients (50.5%) while 11 children (11.3%) suffered from OT on a normal ovary.

Conclusions: Our results showed that a post-menarchal age (p = .001), a pre-operative US ovarian size < 5 cm, (p = .001), the presence of severe abdominal pain (p = .002), a laparoscopic approach (p < .001), and the presence of a functional cyst (p = .002) were significantly associated with conservative surgery.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00404-022-06522-3DOI Listing
June 2022

Pediatric Surgical Care During the COVID-19 Lockdown: What Has Changed and Future Perspectives for Restarting in Italy. The Point of View of the Italian Society of Pediatric Surgery.

Front Pediatr 2022 19;10:871819. Epub 2022 May 19.

Pediatric Surgery Department of Pediatric Surgery, "V. Buzzi" Children's Hospital, Milan, Italy.

Background: The coronavirus disease 2019 (COVID-19) time exacerbated some of the conditions already considered critical in pediatric health assistance before the pandemic. A new form of pediatric social abandonment has arisen leading to diagnostic delays in surgical disorders and a lack of support for the chronic ones. Health services were interrupted and ministerial appointments for pediatric surgical healthcare reprogramming were postponed. As a result, any determination to regulate the term "pediatric" specificity was lost. The aim is, while facing the critical issues exacerbated by the COVID-19 pandemic, to rebuild future perspectives of pediatric surgical care in Italy.

Methods: Each Pediatric Society, including the Italian Society of Pediatric Surgery (SICP), was asked by the Italian Federation of Pediatric Associations and Scientific Societies to fill a questionnaire, including the following the main issues: evaluation of pre-pandemic criticalities, pediatric care during the pandemic and recovery, and current criticalities. The future care model of our specialty was analyzed in the second part of the questionnaire.

Results: Children are seriously penalized both for surgical treatment as well as for the diagnostic component. In most centers, the pediatric surgical teams have been integrated with the adult ones and the specificity of training the pediatric operating nursing is in danger of survival. "Emotional" management of the child is not considered by the general management and the child has become again an adults patient of reduced size.

Conclusion: A new functional pediatric surgical model needs to be established in general hospitals, including activities for day surgery and outpatient surgery. To support the care of the fragile child, a national health plan for the pediatric surgery is required.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fped.2022.871819DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9162442PMC
May 2022

Colistin Therapy, Survival and Renal Replacement Therapy in Burn Patients: A 10-Year Single-Center Cohort Study.

Int J Gen Med 2022 25;15:5211-5221. Epub 2022 May 25.

Burn Center and Plastic Surgery, Department of General and Specialized Surgery, City of Science and Health, CTO Hospital, Torino, Italy.

Purpose: Colistin is still a therapeutic cornerstone against multidrug-resistant gram-negative bacteria (MDRGN), mostly when other antibiotics do not gain adequate activity on these strains. In the present study, we evaluated in a cohort of burn patients the relationship between colistin therapy, survival and requirement of renal replacement therapy (CRRT).

Patients And Methods: Retrospective study of 133 burn patients treated with iv colistimethate sodium (loading dose 9.0 × 10 IU, maintenance dose 4.5 × 10 IU BID) and 35 treated with other antibiotics for MDRGN infection including , , and between January 2008 and December 2017. Multivariate analysis with logistic regression was used to determine the effect of the predictors such as age, total body surface area (TBSA), third-degree burn areas, Revised Baux score, Charlson comorbidity score, length of stay, colistin dose and duration of treatment, mechanical ventilation, and need of CRRT on in-hospital mortality. To investigate the relationship between colistin and renal function, we focused on survivor patients as the completion of the therapeutic course of colistin represented the basic requirement to analyze its impact on the kidney.

Results: Out of 133 colistin- and 35 other antibiotics-treated patients, 83 (62.4%) and 31 (88.6%) survived, and 53 (39.8%) and 3 (9.7%) required CRRT, respectively. The severity of burns, as well as CRRT requirement and mortality, was significantly higher in colistin-treated patients than in other antibiotics-treated patients. Age and TBSA% were the significant predictors of mortality. Out of 83 colistin-treated survivors, 19 (22.9%) required CRRT (9 before and 10 after the start of colistin), and 64 (77.1%) had a normal renal function. No difference about the colistin dose and baseline characteristics, but the revised Baux score was found between the 9 patients requiring CRRT before the colistin course and the 10 patients after. Similarly, among the 64 patients not undergoing CRRT, no difference was found between the patients treated with the cumulative dose of colistin <99.0 × 10 IU (n = 33, median daily dose of 4.0 × 10 IU) and >99.0 × 10 IU (n = 31, median daily dose of 9.0 × 10 IU) about the baseline characteristics and the daily median plasma creatinine over 24 days of therapy.

Conclusion: Colistin therapy was associated with more severe burns, mortality, and CRRT requirement. A short course therapy, at appropriate cumulative dosage, can lead to clinical success without a significant association with severe renal impairment.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.2147/IJGM.S357427DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9150021PMC
May 2022

Intussusception and COVID-19 in Infants: Evidence for an Etiopathologic Correlation.

Pediatrics 2022 06;149(6)

Department of Pediatric General Surgery, Regina Margherita Children's Hospital, Azienda Ospedaliero Universitaria Città della Salute e della Scienza, Turin, Italy.

Nonrespiratory conditions related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections have been largely described. Ileocolic intussusception has been reported in association with SARS-CoV-2 infection in 10 children, raising the possibility of an etiopathologic role for the virus, but none of these cases documented tissue pathology that would have supported SARS-CoV-2 intestinal inflammation. We report 2 cases of intussusception in patients with SARS-CoV-2 infection who were treated at different pediatric tertiary centers in Europe and provide evidence of the presence of the virus in mesenteric and intestinal tissues of the patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1542/peds.2021-054644DOI Listing
June 2022

Laparo-Endoscopic Rendez-Vous in the Treatment of Cholecysto-Choledocolithiasis in the Pediatric Population.

J Pediatr Gastroenterol Nutr 2022 06 7;74(6):819-822. Epub 2022 Mar 7.

Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera Universitaria Città della Salute e della Scienza di Torino.

Objectives: The incidence of choledocolithiasis is reported to be increasing in children. As for the adult population, several different therapeutic strategies have been described, however it is unclear which of those should be considered the gold standard. There is evidence-based literature in adults that supports a combined "rendez-vous" endoscopic retrograde cholangiopancreatography-laparoscopic cholecystectomy technique. This allows management of the choledocholithiasis during the same anesthetic episode as the cholecystectomy. By contrast, there are just two case reports in children reporting this approach. The aim of this study is to report our experience with this technique in a series of children with choledocholithiasis.

Methods: All patients who underwent the "rendez-vous technique" at our institution between 2009 and 2020 were reviewed and evaluated for outcomes and complications.

Results: Eleven children with cholecysto-choledocholithiasis were evaluated: the procedure was successful in 10 whereas in one patient it was aborted due to technical difficulties. All patients resolved their clinical condition without major complications.

Conclusion: To our knowledge, this is the first consistent series of "rendez-vous technique" in the pediatric population, proving its feasibility and safety.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPG.0000000000003444DOI Listing
June 2022

Factors associated with postoperative hypocalcemia following thyroidectomy in childhood.

Pediatr Blood Cancer 2022 08 7;69(8):e29576. Epub 2022 Feb 7.

Department of Pediatrics, National Cancer Institute, Milan, Italy.

Background: Postoperative hypocalcemia is a frequent complication after thyroidectomy. Hypoparathyroidism may develop as transient (TtHP), with normalization within six months from surgery, or permanent (PtHP) if the patient requires replacement therapy. We analyzed factors associated with the development of postoperative hypoparathyroidism and in detail PtHP following thyroid surgery in a pediatric population.

Procedure: A retrospective multicenter study analyzing 326 patients was carried out. We recorded gender, age, tumor size, thyroiditis, extrathyroidal extension, lymph node dissection (central/lateral compartment, unilateral/bilateral), parathyroid autotransplantation, and histology. Additionally, calcium levels were acquired postoperatively.

Results: We analyzed pediatric patients ≤18 years who underwent thyroidectomy clustered into age groups (≤15 or > 15). Patients' mean follow-up was 5.8 years (1-11 years). Postoperative hypoparathyroidism occurred in 36 (11.0%): 20 cases (6.13%) developed PtHP. Postoperative hypoparathyroidism was more frequent in younger patients (P = 0.014), in larger tumors (P < 0.001), in case of extrathyroidal extension (P = 0.037), and in central compartment (P = 0.020) and bilateral lymph node dissection (P = 0.030). PtHP was more frequent in older patients (P = 0.014), in case of thyroiditis (P < 0.001), and extrathyroidal extension (P < 0.001). Concerning the first postoperative calcium level measurement, in the postoperative hypoparathyroidism group, we registered a 8.17 mg/dL value with 14% pre/postoperative decrease (Δ ), whereas in PtHP patient group calcium level was 7.91 mg/dL with 16.7% Δ .

Conclusions: The risk of postoperative hypoparathyroidism is related to younger age, tumor size, central compartment and bilateral lymph node dissection, extrathyroidal extension, and decrease in postoperative calcium levels. The risk of PtHP is related to older age, thyroiditis, extrathyroidal extension, and decrease in postoperative calcium levels.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1002/pbc.29576DOI Listing
August 2022

Quality of life and long-term results in patients operated on for Esophageal Atresia.

Minerva Pediatr (Torino) 2021 Dec 3. Epub 2021 Dec 3.

Pediatric Surgery Unit, University Hospital of Health and Science, Regina Margherita Children's Hospital, Turin, Italy.

Background: Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) represents the most severe entity among the foregut malformations, with patients at risk of chronic morbidity. This study aims to investigate on health-related quality of life (QoL) and long-term results in patients with EA.

Methods: 50 patients (30M, 20F, mean age: 14-year-old), among 82 patients operated from January 1995 to December 2005, answered the questionnaire. Two groups where compared: 8-12 years (A), 12-18 years (B). The survey investigated on 5 itmes: dysphagia; GERD; pulmonary affections (asthma, bronchitis, episodes of chronic cough during a year); growth and nutritional status (BMI); QoL scores. A control group of 50 healthy children was used.

Results: 42 type III AE (3 long-gap and 1 VACTER association). Median BMI was 18,72 (SD 3,38) (range: 30,52-12,80). In 22 pts BMI < 18,5. 19 pts were positive for dysphagia (EAT-10 score > = 3). A correlation was found between dysphagia and low BMI (Pearson 0,37). Dysphagia and low BMI were more diffuse in the younger population, while decreased in group B. 15 pts treated in their life for GERD (8 with medical therapy; 7 fundoplication). GerdQ test data showed only 4 pts with score > 6. GERD and dysphagia: 11 pts with GERD presented EAT-10 test > 3 (Pearson value confirmed this correlation: 0,59). Respiratory pathologies: 26 pts with positive anamnesis for chronic pulmonary affections (recurrent bronchitis in 21 pts; chronic cough in 15; the association of both in 12; 11 pts with asthma). PedsQL 4.0 median value was 15,4 (SD 10,1), control group value was 15,6 (SD 7,6); difference between the 2 groups was not statistically significant (p-value: 0,11). Correlation test for comparing high value of PedsQL and long-term complication in AE: the strongest association was with the dysphagia (Pearson: 0,55).

Conclusions: Dysphagia resulted the most disabling symptom in group A but swallowing function slightly improved with the growth. Generally, AE seems not heavily influence patients' QoL.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.23736/S2724-5276.21.06597-6DOI Listing
December 2021

Incidental Ultrasound Diagnosis of Neonatal Intussusception Secondary to Meckel's Diverticulum in a Neurologically Impaired Child.

Am J Case Rep 2021 Sep 5;22:e932280. Epub 2021 Sep 5.

Department of Pediatric Surgery, Regina Margherita Children's Hospital, Turin, Italy.

BACKGROUND Intussusception is the most common cause of intestinal obstruction in children, with a peak incidence usually before the second year of age, while in neonates it is a rare entity. We describe a delayed and incidental diagnosis of neonatal intussusception secondary to Meckel's diverticulum in a neonate with shaken baby syndrome (SBS). This is, to the best of our knowledge, the first reported case of a neonatal intussusception with a Meckel's diverticulum as a lead point in a neurologically impaired child. CASE REPORT A term baby presented at 22 days of age at our Emergency Department in severe conditions due to a suspected SBS. Eight days following hospitalization in the Intensive Care Unit, an isolated episode of rectal bleeding occurred, without any worsening of general conditions or abdominal distension. The ultrasonography showed a "doughnut sign" with high suspicion of ileocecal intussusception. A rectal barium contrast enema was performed but was not resolutive. At exploratory laparotomy an ileocecal intussusception with Meckel's diverticulum acting as a lead point was identified and an intestinal resection was needed due to the ischemic condition of the ileum. The post-operative course was uneventful and the baby recovered well; the residual neurological impairment needed long-term follow-up. CONCLUSIONS Intussusception is a rare entity in neonates and, when severe neurological impairment is present, the diagnosis can be missed because of the compromised condition of the baby and the paucity of gastrointestinal manifestations. In addition, due to the high incidence of lead point in neonatal cases, we recommend reserving non-operative treatment only for selected cases.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.12659/AJCR.932280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428622PMC
September 2021

A prompt diagnosis of late-onset congenital diaphragmatic hernia with Point of Care Ultrasound (POCUS) in a Pediatric Emergency Department.

Pediatr Neonatol 2021 09 29;62(5):573-574. Epub 2021 May 29.

Pediatric Emergency Department, Regina Margherita Children's Hospital, University Hospital of Health and Science, Turin, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pedneo.2021.05.010DOI Listing
September 2021

Foreign-body Ingestions in Children During COVID-19 Pandemic in a Pediatric Referral Center.

JPGN Rep 2020 Nov 11;1(2):e018. Epub 2020 Nov 11.

Pediatric Gastroenterology Unit, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza di Torino, Turin, Italy.

In February 2020, the COVID-19 pandemic overwhelmed Italy. We retrospectively reviewed all attendances and emergency (A&E) admissions due to foreign-body ingestions (FBIs) to an Italian pediatric referral hospital, from February 24 to April 24, 2020, COVID-19 lockdown and compared them with the same period in the previous 4 years. A total of 101 cases were recorded. Mean age of admission was 4.6 years. Groups did not differ for gender ( = 0.4) or age ( = 0.3). Among FBIs ingestions, 24.0% occurred in children with <2 years of age and 47.5% in children from 2 to 6 years of age. In the 2020 study period, 9 patients were seen for batteries ingestion compared with a median value of one among compared periods. The rates of batteries ingestions increased significantly over the observational period ( < 0.001). We report a dramatic increase in batteries ingestions in children, a potentially fatal event, during the COVID-19 pandemic lockdown.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/PG9.0000000000000018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7673461PMC
November 2020

Newborn With Supraumbilical and Scrotal Bruising.

Ann Emerg Med 2020 12;76(6):e127-e128

Department of Pediatric General Surgery, Regina Margherita Children's Hospital, Azienda Ospedaliero Universitaria Città della Salute e della Scienza, Turin, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.annemergmed.2020.06.021DOI Listing
December 2020

Pediatric Wilkie's syndrome: Report of two monozygotic sisters managed conservatively.

Pediatr Neonatol 2021 03 26;62(2):227-228. Epub 2020 Oct 26.

Department of Pediatric Surgery, Regina Margherita Children's Hospital, University Hospital of health and Science, Turin, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pedneo.2020.10.008DOI Listing
March 2021

Multisystem inflammatory syndrome in SARS-CoV-2 infection mimicking acute appendicitis in children.

Pediatr Neonatol 2021 01 23;62(1):122-124. Epub 2020 Sep 23.

Pediatric General Surgery and Minimally Invasive Surgery Unit, Italy; Regina Margherita Children's Hospital, University Hospital of Health and Science, Turin, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pedneo.2020.09.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7510411PMC
January 2021

Bowel loop sign in a newborn.

Arch Dis Child Fetal Neonatal Ed 2021 May 6;106(3):1-2. Epub 2020 Jul 6.

Pediatric General Surgery, Ospedale Infantile Regina Margherita, Turin, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1136/archdischild-2020-318939DOI Listing
May 2021

An unusual case of acute postpartum bleeding in a mother and her newborn.

Pediatr Neonatol 2020 08 12;61(4):455-456. Epub 2020 Mar 12.

Department of Pediatric General Surgery, Regina Margherita Children's Hospital, University Hospital of Health and Science, Turin, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pedneo.2020.03.005DOI Listing
August 2020

Handheld metal-detector versus conventional chest and abdominal plain radiography in children with suspected metallic foreign body ingestion: can we safely abandon X-rays?

Minerva Pediatr 2020 Apr 2. Epub 2020 Apr 2.

Department of Pediatric General Surgery, Regina Margherita Children's Hospital, University Hospital of Health and Science, Turin, Italy.

Background: Ingestion of metallic foreign bodies (MFBs) is a frequent occurrence in children and is commonly diagnosed via X-rays. In recent years, the handheld metal detector (HMD) has been increasingly adopted by several paediatric hospitals as it is considered an effective and accurate diagnostic tool that avoids exposure to ionizing radiations. Sensitivity of HMD has been reported high (99.4%) in case of coin ingestion, but significantly lower (46%) when considering the ingestion of other types of MFBs.

Methods: We tested the effectiveness of the HMD in diagnosing ingested MFBs in children less than 14 years of age, in our Paediatric Emergency Department (PED). We prospectively evaluated all cases of MFBs ingestion that presented at the PED of our hospital from March 2015 to July 2017.

Results: 98 patients were included. The overall sensitivity was 63.2% (79.5% for coins, 25.5% for batteries and 56% for other objects) while the specificity was 95%. The HMD could have replaced the X-Ray examination only if a MFB was detected below the xyphoid process.

Conclusions: Based on our findings, a negative result of HMD is not sufficient to exclude an ingestion of MFBs. Therefore, in case of an evocative history and depending on type and size of the foreign body, a radiological investigation is still necessary.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.23736/S0026-4946.20.05674-1DOI Listing
April 2020

Idiopathic bilateral jugular phlebectasia in a 9-year-old boy.

Int J Pediatr Otorhinolaryngol 2020 Apr 18;131:109892. Epub 2020 Jan 18.

Department of Pediatric General and Thoracic Surgery, Regina Margherita Children's Hospital, University Hospital of Health and Science, Turin, Italy.

A nine-year-old boy came to our clinic for the appearance of a voluminous swelling at the base of the neck in the jugular area after coughing. He underwent fibroscopy and a contrast-enhanced chest computed tomography (CT) scan, which did not indicate pathological findings even during the Valsalva maneuvre. After a color-Doppler ultrasound of the epiaortic vessels was obtained, a diagnosis of idiopathic phlebectasia of the internal jugular veins was made. The cause of the jugular phlebectasia remains unclear, and no treatment is indicated for this rare, benign, and self-limiting condition.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijporl.2020.109892DOI Listing
April 2020

Infantile Abdominal and Pelvic Lipoblastomas: A Case Series.

European J Pediatr Surg Rep 2019 Jan 28;7(1):e104-e109. Epub 2019 Nov 28.

Division of Pediatric Surgery, Regina Margherita Children's Hospital, Turin, Italy.

Lipoblastomas are rare benign mesenchymal tumors that arise from embryonal fat cells. They are usually discovered in infants and children under 3 years of age, and mostly occur in the trunk (from 10 to 60%, depending on the study) and extremities (from 40 to 45%), while head and neck localizations are rare, with only five cases described to date. We report on three cases of lipoblastomas in infants younger than 4 years, with unusual localizations: one intra-abdominal, discovered during a laparotomy for an intussusception; one pelvic, misdiagnosed as an ovarian mass; and one gluteal with a pelvic extension. All children underwent magnetic resonance imaging as preoperative workup. All tumors were completely resected with free surgical margins and ultrasonographic follow-up was uneventful for all patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1055/s-0039-1694060DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6882683PMC
January 2019

Laparoscopic Repair of a Gastrocolocutaneous Fistula: Percutaneous Endoscopic Gastrostomy Placement Complications in Children.

J Laparoendosc Adv Surg Tech A 2020 Feb 19;30(2):216-220. Epub 2019 Nov 19.

Department of Pediatric General Surgery, Regina Margherita Children's Hospital, University Hospital of Health and Science, Turin, Italy.

Gastrostomy tube placement (G-Tube) is a frequently offered procedure in children with feeding difficulties. Various procedures exist for G-Tube, with the pull technique more commonly used for a percutaneous endoscopic gastrostomy (PEG) in children, considered by many to be the safer approach. Major complications requiring reoperation range from 3% to 5%, depending on the study. In our center, PEG placement is performed by gastrointestinal endoscopists through the pull technique. In the last 5 years, there were 150 procedures, with 15 minor and 3 major complications. We will describe the last 3 cases, plus a fourth PEG placement at another center. Patients ranged from 2 to 10 years (median age: 4.5 years). Median weight was 10.7 kg (range: 7-18 kg). Patients were neurologically impaired children, except one with severe nephropathy. Laparoscopic repair with a 3-trocar technique was effective, when the patient's general condition allowed for it. After fistula repair, a new gastrostomy was placed; this step can be performed endoscopically under laparoscopic control (or can be performed completely laparoscopically). In 4 patients, we faced unusual PEG placement complications, due to colon interposition during blind gastric puncture. In those with anatomical deformities or previous surgery, or dealing with toddlers (under 10 kg), we suggest laparoscopic-assisted PEG, or a full laparoscopic gastrostomy to avoid the risk of a major complication.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1089/lap.2019.0517DOI Listing
February 2020

Esophageal duplication cyst in newborn.

Pediatr Neonatol 2020 02 5;61(1):121-122. Epub 2019 Sep 5.

Department of Pediatric General Surgery, Regina Margherita Children's Hospital, AOU Città della Salute e della Scienza, Turin, Italy.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.pedneo.2019.08.007DOI Listing
February 2020

Intrahepatic Administration of Liposomal Amphotericin B (Ambisome) for the Management of a Liver Abscess from in a Preterm Infant.

Antimicrob Agents Chemother 2018 12 26;62(12). Epub 2018 Nov 26.

Department of Medical and Surgical Neonatology, Bambino Gesù Children's Hospital, Rome, Italy.

Hepatic fungal abscesses are rare in the neonatal period and often constitute a severe complication of the catheterization of the umbilical vessels. Such life-threatening lesions are observed more frequently in preterm than in other newborn infants and the optimal treatment remains uncertain. We present the case of a preterm neonate, who developed an intrahepatic lesion due to parenteral extravasation, successively contaminated by Despite the maximal pharmacological therapies, the treatment that led to the definitive resolution of the abscess was the placement of surgical drainage followed by the direct intralesional administration of liposomal amphotericin B (Ambisome), never described in neonates in the literature, which turned out to be a safe and effective approach.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1128/AAC.01239-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6256806PMC
December 2018

Paternal isodisomy of chromosome 2 in a child with bile salt export pump deficiency.

Hepatol Res 2012 Mar;42(3):327-31

Units of Pathology Molecular Medicine and Neurosciences Hepatic Surgery, Children's Hospital Bambino Gesù, Rome Unit of Pathology, Molinette Hospital, Turin IRCCS Fondazione Stella Maris, Pisa, Italy.

We describe a child with progressive familial intrahepatic cholestasis (PFIC) of type 2 inherited as uniparental isodisomy of chromosome 2. Bile salt export pump (BSEP) deficiency is a severe, genetically determined subtype PFIC caused by mutations in ABCB11, the gene encoding a bile salt transporter protein. Clinical and pathological diagnosis in PFIC2 is corroborated by an ample array of ABCB11 mutations, inherited in an autosomal recessive fashion. We report clinical, pathological, and molecular studies in a child with PFIC2. A 5.5-year-old boy harbored a described pathogenic mutation (p.R832C) in ABCB11. The mutation was found to be homozygous in the patient and heterozygous in DNA from paternal, but not maternal blood. Having ruled out maternal gene deletion and somatic mosaicism, we showed that the child had inherited an isodisomic paternal chromosome 2, including the 2q31.1 region where ABCB11 is located. The present report is the first description of uniparental isodisomy in a hepatic heritable disorder. Recognizing isodisomic transmission may have a significant impact on genetic counseling helping to define the risk of recurrence in subsequent pregnancies.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1872-034X.2011.00925.xDOI Listing
March 2012

Unresectable multifocal omental and peritoneal inflammatory myofibroblastic tumor in a child: revisiting the role of adjuvant therapy.

J Pediatr Surg 2011 Apr;46(4):e17-e21

Liver Surgery Unit, Bambino Gesù Children's Hospital, Rome - 00165, Italy; Paediatric surgery, University of Roma Tor Vergata, Roma - 00133, Italy. Electronic address:

Inflammatory myofibroblastic tumor is an uncommon lesion, also called pseudotumor, with a variable natural course from benign with spontaneous regression to mimicking malignant tumors. We report a case of diffuse peritoneal and omental pseudotumor in a 10-year-old boy characterized by aggressive behavior at the onset followed by stability after subtotal resection and chemotherapy. Total excision was not possible because of the tumor dissemination over the whole peritoneal surface. Adjuvant antiinflammatory drug (ketorolac tromethamine) and chemotherapy (methotrexate-vinblastine followed by ifosfamide-adriamycin and ifosfamide alone) were helpful to obtain rapidly complete resolution of clinical symptoms and anatomic stability of the residual lesions. Long-term evolution, in the absence of continued therapy, has been characterized by progressive involution and reduction of the residual masses.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jpedsurg.2011.01.007DOI Listing
April 2011

Preemptive liver transplantation in a child with familial hypercholesterolemia.

Pediatr Transplant 2011 Mar 15;15(2):E25-9. Epub 2010 Sep 15.

Metabolic Unit, Bambino Gesù Children Hospital, Rome, Italy.

Familial hypercholesterolemia is an autosomal codominant disorder associated with markedly elevated plasma concentration of LDL-cholesterol and increased cardiovascular risk. Homozygous patients have rapid development of atherosclerosis with death from cardiovascular disease even in childhood. Life-long recurrent apheresis to reduce plasma LDL-cholesterol is considered the gold standard for treatment. Liver transplantation can be curative for this condition, but is usually only considered after the development of cardiovascular disease. We report a 5.5-yr-old child initially misdiagnosed with heterozygous familial hypercholesterolemia and treated by low-fat diet only. In view of persistent hypercholesterolemia and development of xanthomatosis, new molecular studies indicated the presence of two different mutations in the LDL receptor gene, with one being a deletion of two exons not identifiable with standard sequencing analysis. Recurrent plasma apheresis in combination with statins lowered, but did not normalize plasma LDL-cholesterol levels. It caused progressive reduction of the size of xanthomas and prevented the development of vascular complications. After two yr, liver transplantation normalized LDL-cholesterol levels and completely resolved the skin lesions. Preemptive liver transplantation is a definitive cure of familial homozygous hypercholesterolemia and might be more effective if performed before development of vascular complications.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1399-3046.2010.01383.xDOI Listing
March 2011

Remittent hyperammonemia in congenital portosystemic shunt.

Eur J Pediatr 2010 Mar 18;169(3):369-72. Epub 2009 Jul 18.

Department of Pediatrics, University of Torino, Piazza Polonia 94, 10126 Torino, Italy.

Congenital portosystemic shunts (PSS) are rare vascular anomalies with different gross anatomy. Persistent patent ductus venosus (PDV) represents an uncommon cause of intrahepatic PSS. The diagnosis of this condition may not be obvious because of its wide spectrum of clinical manifestations, ranging from asymptomatic to life-threatening disease. We report the case of three boys with neuropsychological symptoms associated with mild fasting hyperammonemia. An oral protein load allowed the detection of a detoxication defect due to PSS related to PDV. This simple procedure can be worthwhile of attention in patients with mental retardation, behavior disturbances, and learning difficulties after exclusion of common causes of inherited hyperammonemia, namely, urea cycle disorders, organic acidemias, and fatty acid oxidation defects.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00431-009-1031-zDOI Listing
March 2010

Spontaneous occlusion of brainstem arteriovenous malformation following ligature of a hepatic patent ductus venosus. Case report and review of the literature.

J Neurosurg 2007 Feb;106(2 Suppl):147-52

Department of Pediatric Neurosurgery, "Regina Margherita" Children's Hospital, San Giovanni Battista Hospital, Turin, Italy.

The authors describe the clinical and radiological findings in a case of brainstem arteriovenous malformation (AVM) associated with a hepatic patent ductus venosus (PDV) in a 12-year-old child. The AVM was discovered on magnetic resonance (MR) imaging performed because of slight mental retardation and headache. The malformation was otherwise asymptomatic and no treatment was proposed. An abdominal ultrasonography study performed 1 year later because of hyperammonemia revealed a PDV, which was surgically ligated. One year later, MR images and angiograms showed complete resolution of the brainstem AVM. This report is the first documentation of an association between these two entities in the same patient, and the possible pathophysiological interactions between them are discussed.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3171/ped.2007.106.2.147DOI Listing
February 2007

The first one thousand liver transplants in Turin: a single-center experience in Italy.

Transpl Int 2005 Dec;18(12):1328-35

Liver Transplantation Center, San Giovanni Battista Hospital, Turin, Italy.

The first Italian liver transplant center to reach the goal of 1000 procedures was Turin. The paper reports this single-center experience, highlighting the main changes that have occurred over time. From 1990 to 2002, 1000 consecutive liver transplants were performed in 910 patients, mainly cirrhotics. Surgical technique was based on the preservation of the retrohepatic vena cava of the recipient. The veno-venous bypass was used in 30 cases only and abandoned since 1997. Operating time, warm ischemia time and length of hospital stay significantly decreased over the years, while operating room extubation became routine. Immunosuppression pivoted on cyclosporine A. Management of retransplantations, marginal grafts, and of HCV-positive, HBV-positive and hepatocellular carcinoma recipients were optimized. Median follow-up of the patients was 41 months. Overall survival rates at 1, 5 and 10 years were 87%, 78% and 72% respectively. Survival rates obtained in the second half of the cases (1999-2002 period) were significantly better than those obtained in the first half (1990-1998 period) (90% vs. 83% at 1 year and 81% vs. 76% at 5 years respectively). Increasing experience in liver transplant surgery and postoperative care allowed standardization of the procedure and expansion of the activity, with parallel improvement of the results.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1432-2277.2005.00215.xDOI Listing
December 2005

Impact of surgical and immunological parameters in pediatric liver transplantation: a multivariate analysis in 500 consecutive recipients of primary grafts.

Ann Surg 2004 Feb;239(2):272-80

Pediatric Liver Transplant Program, Saint-Luc University Clinics Université catholique de Louvain, Brussels, Belgium.

Objective: To assess the respective impact of surgical and immunologic factors on patient/graft outcome and rejection after pediatric liver transplantation.

Summary Background Data: Orthotopic liver transplantation (OLT) constitutes a validated therapeutic modality for acute liver failure and end-stage liver disease in children. Only a few large studies of factors influencing outcome of pediatric OLT are available in the literature. Studies considering the impact of rejection on graft outcome are scarce in adult OLT and are not even available for pediatric recipients.

Methods: Five hundred consecutive pediatric recipients (<15 years) of a primary OLT performed between March 1984 and July 2000 were retrospectively reviewed. The main indication was biliary atresia (n = 328). A living related donor graft was used from July 1993 onwards in 82 children (16%). Survival was calculated and multivariate analysis was performed.

Results: Actuarial survival rates at 1, 5, and 10 years were 85%, 81%, and 79% for patients, and 76%, 71%, and 70% for grafts, respectively. At the multivariate analysis, only 3 factors were found to be independently correlated with better patient survival: year of transplantation (P = 0.001), pretransplant diagnosis (P < 0.001, worst results for liver tumors), and ABO matching (P < 0.001, worst results for ABO incompatibility). Similarly, 3 factors were independently correlated with better rejection-free graft survival: tacrolimus as primary immunosuppressant (P < 0.001), a negative T-cell crossmatch (P = 0.016), and younger age of the donor (P < 0.001).

Conclusions: Pediatric OLT constitutes a complex undertaking with multifactorial impact on results: (1). a strong learning curve effect was shown to impact on overall results; (2). pediatric liver tumors still represent a challenging indication for OLT; (3). primary immunoprophylaxis with tacrolimus provided a lower rejection incidence; (4). the younger donor age effect deserves further immunologic investigations.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/01.sla.0000108681.24374.02DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1356222PMC
February 2004
-->