Pediatric nutritional surgery and its implications: results from a unicentric retrospective analysis.

PURPOSE: Existing guidelines provide weak recommendations on the surgical management of nutritional problems in children. The objective was to design a management pathway to address the best nutritional surgery (NS) procedure in a given patient. METHODS: Retrospective analysis of children treated at our department from January 2015 to December 2019. The sample was divided into two groups according to presence or absence of neurological impairment (NI). Patients with NI (Group 1) were classified in three subgroups based on presenting symptoms: A-Dysphagia without gastroesophageal reflux (GER); B-GER with or without dysphagia; C-Symptoms associated with a delayed gastric emptying. RESULTS: A total of 154 patients were included, 111 with NI. One-hundred-twenty-eight patients underwent only one procedure. Complications and mortality were superior in Group 1. In subgroup A, isolated gastrostomy was the first NS in all patients. In subgroup B most of patients were subjected to a Nissen fundoplication, while in 5 cases total esophagogastric dissociation (TEGD) was the first intervention. Considering the entire sample, 92.3% patients who underwent a TEGD did not require further procedures. CONCLUSION: NS encompasses various procedures depending on presenting symptoms and neurological status. A management flowchart for these patients is proposed.

Magnetic resonance urography of congenital abnormalities - what the radiologist needs to know.

Congenital abnormalities of the kidney and urinary tract include a wide range of malformations ranging from asymptomatic to life-threatening conditions. Although pediatric urogenital system imaging is based on the use of US (pre- and postnatal), voiding cystourethrography and scintigraphic study, magnetic resonance (MR) urography plays a fundamental role in the classification and management of congenital abnormalities of the kidney and urinary tract, giving an overview of the different clinical pictures, thanks to its panoramicity and high anatomical detail. In fact the anomalies of the urinary tract are phenotypically variable because they can affect simultaneously several segments of different embryonic derivation, with complex clinical pictures; they can appear both as isolated phenotypes or as complex malformative conditions, involving renal parenchyma, collecting system and bladder. A deep knowledge of this complex embryogenesis and its possible phenotypic patterns allows a correct interpretation of MR urography images. We describe the embryology and pathophysiology of congenital abnormalities of the kidney and urinary tract as well as MR urography technique and findings. Congenital abnormalities of the kidney and urinary tract are classified into four groups: (1) obstruction (proximal, middle and distal), (2) budding with respect to the Wolffian duct (site and number of ureter), (3) ascent and rotation (ectopia, malrotation and fusion of kidney) and (4) anomaly of metanephric differentiation (dysplasia, megapolicalycosis).

Ultrasound detected prenatal hyperechoic lung lesions and concordance with postnatal findings: A common aspect for multiple diagnoses.

OBJECTIVE: Hyperechoic lung lesions are largely detected prenatally but their underlying etiology is still poorly defined. The aim of the study was to determine the concordance between pre and postnatal diagnosis of hyperechoic lung lesions. METHODS: Prenatal ultrasound (US) evaluation was performed by a fetal medicine specialist. Postnatal diagnosis was based on CT-scan. Pre- and postnatal features were retrieved from medical charts. RESULTS: Seventy five patients were included from January 2009 to December 2018. Main prenatal diagnoses were bronchopulmonary sequestrations (BPS) (n = 24%-32%), pulmonary cystic malformations (PCM) (n = 19%-25%), congenital lobar emphysemas (CLE) (n = 15%-20%). Mediastinal shift was observed in 18 cases (24%). The prenatal detection of a systemic arterial supply had a diagnostic accuracy of 90%, while the prenatal detection of a cystic component had a diagnostic accuracy of 76.5%. All 16 neonates with prenatal isolated mediastinal shift were asymptomatic at birth. Seven neonates showed respiratory distress that was not predicted prenatally. CONCLUSIONS: Hyperechoic lung malformations reflect a heterogeneous group of lesions with a good concordance for bronchopulmonary sequestration, but not a satisfying prediction for cystic lesions.

Non-contrast-enhanced magnetic resonance angiography for detecting crossing renal vessels in infants and young children: comparison with contrast-enhanced angiography and surgical findings.

BACKGROUND: Knowing that ureteropelvic junction obstruction is due to a crossing renal vessel is essential in choosing the appropriate surgical treatment. OBJECTIVE: To evaluate the diagnostic accuracy of non-contrast magnetic resonance (MR) angiography in identifying crossing renal vessels in children younger than 4 years old with unilateral hydronephrosis. MATERIALS AND METHODS: A retrospective review of preoperative MR urography of children with unilateral hydronephrosis was conducted by two independent readers. The presence or absence of crossing renal vessels was identified and compared with surgical findings. RESULTS: Twenty-nine patients were included. The disagreement between MR angiography with and without contrast enhancement in detecting a crossing renal vessel was 8%. The disagreement between non-contrast-enhanced MR and surgical findings was 17%. The disagreement between contrast-enhanced MR angiography and surgical findings was 25%. The balanced triggered angiography without contrast enhancement had a sensitivity of 70% (95% confidence interval [CI]: 35-93%) and a specificity of 93% (95% CI: 66-100%). Contrast-enhanced MR angiography had a sensitivity of 56% (95% CI: 21-86%) and a specificity of 91%. (95% CI: 59-100%). CONCLUSION: MR without contrast enhancement may be a reliable, valid and safe alternative to contrast-enhanced MR angiography for identifying crossing renal vessels.

Diagnostic accuracy of upper gastrointestinal series in children with suspected intestinal malrotation.

Intestinal malrotation (IM) results from an altered or incomplete rotation of the fetal midgut around the superior mesenteric artery axis. The abnormal anatomy of IM is associated with risk of acute midgut volvulus which can lead to catastrophic clinical consequences. The upper gastro-intestinal series (UGI) is addressed as the gold standard diagnosis procedure, but a variable failure degree has been described in literature. The aim of the study was to analyze the UGI exam and describe which features are the most reproducible and reliable in diagnosing IM. Medical records of patients surgically treated for suspected IM between 2007 and 2020 at a single pediatric tertiary care center were retrospectively reviewed. UGI inter-observer agreement and diagnostic accuracy were statistically calculated. Images obtained with antero-posterior (AP) projections were the most significant in terms of IM diagnosis. Duodenal-Jejunal Junction (DJJ) abnormal position resulted to be the most reliable parameter (Se = 0.88; Sp = 0.54) as well as the most readable, with an inter-reader agreement of 83% (k = 0.70, CI 0.49-0.90). The First Jejunal Loops (FJL), caecum altered position and duodenal dilatation could be considered additional data. Lateral projections demonstrated an overall low sensitivity (Se = 0.80) and specificity (Sp = 0.33) with a PPV of 0.85 and a NPV of 0.25. UGI on the sole AP projections ensures a good diagnostic accuracy. The position of the third portion of the duodenum on lateral views showed an overall low reliability, therefore it was not helpful but rather deceiving in diagnosing IM.

Non-standard approach to infants and children with megacolon: laparotomy and endorectal pull-through for diagnosis and treatment in difficult countries with low resources in a non-profit setting: return to the past Soave's ERPT.

BACKGROUND: Hirschsprung's disease is an important cause of pediatric constipation with high risk of bacterial enterocolitis. Its diagnosis is histological and the suction biopsy is the gold standard. In resource-limited countries, the main diagnostic exam is the contrast enema and mini-invasive surgery lacks. We present the management of a cohort of patients with megacolon in Haiti, a low-resource country. METHODS: Children with megacolon and fecal impaction admitted at St Damien Children Hospital in Port-Au-Prince in June, August and December 2017 were included. We considered only patients with an evident transition zone on contrast enema who underwent endorectal pull-through (ERPT). Short term complications were recorded. RESULTS: Twenty children with clinical megacolon were admitted, eleven were included in the study. No suction rectal biopsy and intraoperative histological evaluation were performed. In ten children a Soave ERPT with anastomosis at 5POD was performed, in the other case a Boley primary anastomosis was preferred. One patient complicated with a peritonitis. No major complications were recorded. Colostomy was not considered a good option. CONCLUSIONS: In developing countries, Soave ERPT with definitive anastomosis after few days could be considered a valid option. Colostomy is suggested only in case of scant general conditions or bad colon appearance.

Enterostomy-related complications in Hirschsprung's disease in a single cohort.

BACKGROUND: Hirschsprung's disease (HSCR) is a frequent cause of intestinal obstruction in children and may require an enterostomy. The study aimed to describe the most common enterostomy-related complications in a series of patients treated in a single center. METHODS: A series of consecutive HSCR patients treated or followed-up at our institution between January 1993 and December 2016 were included. Data about HSCR type, enterostomy site, duration and complications of the stoma were recorded. RESULTS: Three hundred one patients with HSCR were followed-up. Sixty-one had ultralong forms (TCSA/TIA), 21 had long forms (L-HSCR) and 219 had classic short forms (S-HSCR). One hundred thirty-seven patients required a stoma (100% of patients with TCSA/TIA, 66.7% with L-HSCR and 28.3% with S-HSCR). We observed 64 stoma-related complications: 36 major complications and 28 minor complications. Major complications occurred more often in long forms (P=0.037). The presence of an ileostomy was statistically associated with an increased rate of complications compared to colostomy. The longer the stoma was in site, the higher the complication rate was. CONCLUSIONS: Long and ultra-long forms are associated with a longer duration of the stoma and to a major risk of stoma-related complications.

Case report: Late-onset hypertrophic pyloric stenosis in a 3-year-old boy: It is never too late.

Hypertrophic Pyloric Stenosis (HPS) represents a relatively rare occurrence beyond infancy. Here, we present the case of a barely 3-year-old boy diagnosed with late-onset HPS and successfully treated with extra-mucosal pyloromyotomy. We review the literature, challenging the principle that more aggressive surgical approaches should be preferred over less invasive ones.

Functional Magnetic Resonance Urography in Ureteropelvic Junction Obstruction: Proposal for a Pediatric Quantitative Score.

Background: Ureteropelvic junction obstruction (UPJO) is one of the most frequent causes of congenital hydronephrosis. It is essential to distinguish UPJO which needs surgical treatment. fMRU combines high quality morphological details of the kidney and excretory pathways with functional data. Objective: This study aims to introduce a new radiological score based on fMRU findings to be able to differentiate surgical from non-surgical kidneys. Materials and Methods: We retrospectively selected patients with hydronephrosis due to UPJO who underwent fMRU (January 2009-June 2018). A multidisciplinary team identified a list of fMRU morpho-functional predictive variables to be included in the analysis. To evaluate the role of different independent variables in predicting the outcome, a multivariable logistic regression model has been performed; the outcome variable was the surgical intervention. For each predictive variable, Odds Ratio and 95% Confidence Intervals were calculated. The likelihood ratio test was used to assess the significance of the variables. Using the regression model, we assigned a numerical value to each predictive variable, rounding up the beta-coefficients. The cut-off value of the total score was obtained from the ROC curve analysis. Results: A total of 192 patients were enrolled, corresponding to 200 pathological kidneys. All of them underwent fMRU; 135 were surgically treated, while 65 underwent ultrasound or MRU follow-up. Predictive variables significantly associated with surgery resulted to be the urographic phase, the presence of abnormal vessels, and a baseline anterior-posterior pelvic diameter >23 mm. Beta coefficients of the logistic regression model were then converted in scores. The ROC curve of the score showed high sensitivity (84.3%) and specificity (81.3%) with a cut-off > 2.5. Conclusions: We propose a new fMRU score able to identify surgical vs. non-surgical kidneys with UPJO.

Surgical validation of functional magnetic resonance urography in the study of ureteral anomalies distal to the uretero-pelvic junction in a pediatric cohort.

BACKGROUND: Ureteral anomalies distal to the Uretero-Pelvic Junction (UPJ) belong to the wide spectrum of congenital anomalies of the kidney and urinary tract (CAKUT). They can cause severe obstruction requiring a detailed anatomical depiction to define the surgical approach. Up to date, ultrasonography, voiding cystourethrography and scintigraphy are considered the gold-standard diagnostic tools to study obstructive anomalies of the urinary tract, however, they do not provide accurate ureteral anatomical details. The aim of our study was to evaluate the concordance between functional magnetic resonance urography (fMRU) and intraoperative findings to define ureteral anomalies distal to UPJ. METHODS: Pediatric patients with ureteral anomalies distal to the UPJ who underwent surgery after performing fMRU were retrospectively collected. Surgical data were compared with radiological results. The concordance was assessed considering both pathological and non-pathological urinary tracts and was calculated by means of the Cohen's kappa coefficient. fMRU diagnostic accuracy was defined by sensitivity, specificity, and binomial exact confidence intervals. RESULTS: We included 46 patients. The sensitivity and specificity of fMRU were 98.0% and 83,3%; positive predictive value 90,4%, negative predictive value 96.2%. The concordance between surgical findings and fMRU was 92,3%, with a k Cohen's coefficient of 0.83 (excellent). CONCLUSIONS: Our study demonstrates the excellent agreement between fMRU and surgical findings in the definition of ureteral anomalies distal to the UPJ in children. Thus, it could be considered a valid imaging technique in the preoperative planning as it provides the surgeon with important information regarding the etiology and site of the obstruction.

Antithrombin supplementation for prevention of vascular thrombosis after pediatric liver transplantation.

AIMS: After liver transplantation (LT), synthesis of coagulation factors by the graft recovers faster for pro thrombotic than anti thrombotic factors, resulting in a potential pro thrombotic imbalance. We studied the thrombotic and hemorrhagic complications in our pediatric LT series, providing supplementation of fresh frozen plasma (FFP) and/or antithrombin (AT) in the prophylactic antithrombotic regimen. METHODS: This was a retrospective observational single center study. All isolated pediatric LTs performed between 1/11/2009 and 31/12/2019 (n = 181) were included. Postoperatively, in addition to low molecular weight heparin, 22 patients (12%) received FFP (10 ml/kg twice daily for 10 days), 27 patients (15%) were given FFP (reduced duration) and AT (50-100 IU/kg/day if AT activity remained <70%), and 132 (73%) received AT only. Complications, outcome, and coagulation profiles in postoperative days 0-10 were analyzed. RESULTS: In all three treatment groups, AT activity normalized by day 4 while prothrombin remained <70% of normal until day 9. Hepatic artery thrombosis (HAT), portal vein thrombosis (PVT), and hemorrhagic complications occurred in 2.8%, 3.3%, and 3.9% of LTs. One- and 5-year patient and graft survival were 88% (±2.4% Standard Error) and 84% (±2.5%), and 86% (±2.6%) and 84% (±2.7%), respectively, without difference between groups. HAT were associated with low AT on days 0 and 1, and PVT with low AT on day 0. CONCLUSIONS: Low antithrombin activity after LT was associated with postoperative thromboses. FFP and/or AT supplementation allowed early normalization of AT activity, while thrombotic or hemorrhagic complications were rare, suggesting efficient and safe management of post-LT coagulopathy.

Complications and trends in minimally invasive repair of pectus excavatum: A large volume, single institution experience.

BACKGROUND/PURPOSE: Complication risk in minimally invasive repair of pectus excavatum (MIRPE) is not negligible, particularly during learning curve. We reviewed the complications of a large series, evaluated the correlation with technical details and learning curve, and presented the strategies implemented to reduce them. METHODS: Data on MIRPE patients from 2005 to 2020 (divided in two groups: before and after 2013) were collected prospectively and reviewed. Complications were correlated to the number and type of bar/stabilizers, and to the surgeon learning curve. RESULTS: We placed 783 bars (484 Biomet, 273 Intrauma and 26 others) in 600 patients and removed 524 bars in 436 patients. Complications occurred in 108 MIRPE (18%): 3.7% intraoperative, 14.3% postoperative. No cardiac perforations were reported; in the second period we had fewer complications (15.3% versus 28.2%) particularly in intraoperative ones (0.3% versus 9%), the intervention was faster (64 versus 83 min and 92 versus 127 for 1 and 2 bars) and hospitalization shorter (5 versus 7.5 days). Complication rate of bar removal was 2.7% (all Biomet bars): one intra-thoracic bleeding and 2 lung injuries. CONCLUSIONS: MIRPE has a significant rate of complication, especially during learning curve. Postoperative complications are more frequent and less dependent on learning curve. Severe complications can be observed during bar removal. We have adopted new bars to reduce complications, but dislocation remains an unresolved problem.

Blood metal levels after minimally invasive repair of pectus excavatum.

OBJECTIVES: Minimally invasive repair of pectus excavatum (MIRPE) is the most popular surgical approach for paediatric patients with pectus excavatum. A substernal stainless still bar is inserted and left in place for 3 years and then removed. Our goal was to investigate blood metal levels after MIRPE and to correlate them with surgical details, such as the numbers of bars and stabilizers and the length of time the bar was in place. METHODS: Blood levels of iron, chromium, manganese, molybdenum and nickel were analysed in 130 teenagers (108 boys and 22 girls) who had MIRPE using inductively coupled plasma mass spectrometry. A total of 62 patients were operated on using MIRPE (study group) and 68 patients were evaluated at implant time (control group). Differences between the numbers of bars implanted and the presence or absence of stabilizers were also considered. RESULTS: Significant increases in the levels of abnormal chromium were found in patients in the study group compared with the controls (P = 0.02). When we compared the group of patients with 2 or more bars with the group with 1 bar, the percentage of patients with a value above the threshold increased by 29 (P = 0.05). A significant increase in chromium levels was observed in patients with stabilizers (P = 0.03). Above-threshold levels of molybdenum were found in 5.1% of patients in the control group, but the number was not statistically significant (P = 0.09). CONCLUSIONS: We demonstrated that stainless steel devices used in MIRPE can elevate blood metal levels in paediatric patients. Moreover, we demonstrated that the use of metal stabilizers is associated with higher metal levels, probably due to increased dispersion.

Total oesophago-gastric dissociation in neurologically impaired children: Laparoscopic vs robotic approach.

PURPOSE: To evaluate and compare the feasibility and short-term results of laparoscopic and robotic total oesophago-gastric dissociation (TOGD) with a Roux-en-Y oesophago-jejunostomy. Minimal invasive surgery has multiple advantages in neurologically impaired patients. Robotic approach has overcome disadvantages linked to laparoscopy, in particular, referring to the surgeon fatigue. METHODS: A retrospective study comparing five laparoscopic and five robotic TOGD was conducted between February and October 2016 in Giannina Gaslini Children's Hospital and Section of Pediatric Surgery of Siena. Neurologically impaired children scheduled for TOGD were included. Age, sex, weight, symptomatology, presence of epilepsy, and preoperative X-ray contrast were considered. Operative time, hospital stay, postoperative complications, redo surgery, nutrition rehabilitation, and X-ray contrast study after 5 days and after 1 month from the intervention were recorded. RESULTS: In our series, there were no intraoperative complications, no conversions to open surgery, and no vagal lesions. In two of five robotic cases, a pyloroplasty was necessary. The median operative time was statistically longer in the robotic group. One dehiscence in the robotic group was recorded, and no dumping episodes occurred. No statistical differences in terms of complications were detected. CONCLUSION: TOGD is feasible both with laparoscopic and robotic-assisted surgery with similar results. Robotic approach is considered feasible. At the same time, high laparoscopic skills allow to reach the same results as robotic approach with shorter operative time.

Comparative Study Between Functional MR Urography and Renal Scintigraphy to Evaluate Drainage Curves and Split Renal Function in Children With Congenital Anomalies of Kidney and Urinary Tract (CAKUT).

Background: Obstructive congenital anomalies of the kidney and urinary tract have a high risk of kidney failure if not surgically corrected. Dynamic renal scintigraphy is the gold standard technique to evaluate drainage curves and split renal function (SRF). Objectives: To compare functional magnetic resonance (MR) urography with dynamic renal scintigraphy in measuring volumetric SRF and in the classification of drainage curves in patients with congenital anomalies of the kidney and urinary tract. Materials and Methods: We retrospectively collected patients with hydroureteronephrosis or pelvicalyceal dilatation at renal ultrasound, who underwent both functional MR urography and dynamic renal scintigraphy (DRS) within 6 months. DRS studies were evaluated by a single nuclear medicine physician with a double reading. Functional MR urography renograms were blind evaluated twice by two radiologists. The functional MR urographyintra- and inter-reading agreements as well as the agreement between the two imaging techniques were calculated. SRF was evaluated by Area Under the Curve and Rutland-Patlak methods. Drainage curves were classified as normal, borderline or accumulation patterns by both the techniques. Results: Fifty-two children were studied, 14 with bilateral involvement. A total of 104 kidney-urinary tracts were considered: 38 normal and 66 dilated. Considering Area Under the Curve and Rutland-Patlak for SRF, the intra- and inter-reader agreements of functional MR urography had excellent and good results, respectively, and the two techniques demonstrated a good concordance (r2: 67% for Area Under the Curve and 72% for Rutland-Patlak). Considering drainage curves, the inter-readers agreement for functional MR urography and the concordance between the two techniques were moderate (Cohen's k, respectively, 55.7 and 56.3%). Conclusions: According to our results, there are no significant differences between functional MR urography and DRS in measuring volumetric SRF and in the classification of drainage curves in patients with congenital anomalies of the kidney and urinary tract.

Antibiotic prophylaxis in children undergoing abdominal surgery for neoplastic diseases.

Little is known about the effectiveness of antibiotic prophylaxis for prevention of surgical site infections (SSIs) in paediatric abdominopelvic surgical oncology. A retrospective analysis was performed upon the incidence of SSIs in children receiving a 24-hour antibiotic prophylaxis with cefazolin for abdominopelvic oncological surgery. In all, 145 patients (57% females) with a median age of 4 years underwent surgical procedures for abdominopelvic tumours. No SSIs were detected, despite the various risk factors known to be associated with their occurrence (such as pre- and post- surgical chemotherapy, long hospitalization, intensive care unit admission and drain placement). Cefazolin prophylaxis seems to be safe and effective in preventing SSIs in children undergoing abdominopelvic surgery for oncological diseases.