Findings in percutaneous trans-hepatic cholecysto-cholangiography in neonates and infants presenting with conjugated hyperbilirubinemia: emphasis on differential diagnosis and cholangiographic patterns.

BACKGROUND: Biliary atresia (BA) is one of the causes of conjugated hyperbilirubinemia in infants which if untreated leads to end-stage liver disease and death. Percutaneous Trans-hepatic Cholecysto-Cholangiography (PTCC) is a minimally invasive study which can be utilized in the diagnostic work-up of these patients. This study's purpose is to describe the experience with PTCC in neonates, the imaging findings encountered, and the abnormal patterns which warrant further investigation. METHODS: A 16-year single-center retrospective study of patients with persistent neonatal cholestasis (suspected BA) undergoing PTCC. Patient demographics, laboratory values, PTCC images, pathology and surgical reports were reviewed. RESULTS: 73 patients underwent PTCC (68% male, mean age 8.7 weeks, mean weight 4.0 Kg). The majority of studies were normal (55%). Abnormal patterns were identified in 33 cases, 79% were diagnosed with BA and 12% with Alagille syndrome. Non-opacification of the common hepatic duct with a narrowed common bile duct (42%) and isolated small gallbladder (38%) were the most common patterns in BA. CONCLUSION: PTCC is a minimally invasive study in the diagnostic work-up of infants presenting with conjugated hyperbilirubinemia (suspected BA). Further invasive investigations or surgery can be avoided when results are normal.

Association between preoperative hemoglobin levels after iron supplementation and perioperative blood transfusion requirements in children undergoing scoliosis surgery.

BACKGROUND AND AIMS: In this study, we assessed the association between preoperative hemoglobin and red blood cell transfusion in children undergoing spine surgery after the implementation of our preoperative iron supplementation protocol. METHOD: We performed a retrospective analysis of patients who underwent posterior spinal fusion surgery between January 2013 and December 2017 and received preoperative iron supplementation. We used uni- and multivariable logistic regression to determine the association between preoperative hemoglobin level and red blood cell transfusion in patients receiving iron supplementation. RESULTS: A total of 382 patients treated with preoperative oral iron were included. Of these, 175 (45.5%) patients were transfused intraoperatively. Multivariable logistic regression analysis revealed nonidiopathic etiology of the scoliosis (OR 4.178 [95% CI: 2.277-7.668], P < .001), the Cobb angle (OR 1.025 [95% CI: 1.010-1.040], P = .001), and number of vertebrae fused (OR 1.169 [95% CI: 1.042-1.312], P = .008) were associated with red blood cell transfusion. In addition, patients with a preoperative hemoglobin ≥ 140 g/L (OR 0.157 [95% CI: 0.046-0.540], P = .003), and hemoglobin between 130 and 140 g/L (OR 0.195 [95% CI: 0.057-0.669], P = .009) were less likely to be transfused compared with patients with preoperative hemoglobin between 120 and 130 g/L (OR 0.294 [95% CI: 0.780-1.082], P = .066) or <120 g/L (reference). CONCLUSION: Our study suggests that higher preoperative hemoglobin levels (>130 g/L) are associated with a reduced need for red blood cell transfusion in pediatric patients who have received iron supplementation before undergoing posterior spinal fusion in our institution. The effect of iron supplementation, the optimal dosing, and duration of supplemental iron therapy remains unclear at this time.

The effect of dexmedetomidine on motor-evoked potentials during pediatric posterior spinal fusion surgery: a retrospective case-control study.

PURPOSE: Motor-evoked potentials (MEPs) are frequently used in pediatric posterior spinal fusion surgery (PSFS) to detect spinal cord ischemia. Dexmedetomidine is increasingly being used as an adjunct to total intravenous anesthesia, but its effect on MEP amplitude has been variably reported. The purpose of this study was to evaluate the effect of an infusion of dexmedetomidine on the amplitude of MEPs. METHODS: We performed a retrospective case-control study of 30 pediatric patients who received a 0.5 µg·kg-1·hr-1 infusion of dexmedetomidine, ten patients who received 0.3 µg·kg-1·hr-1 dexmedetomidine, and 30 control patients who did not receive dexmedetomidine during PSFS. Two neurophysiologists reviewed the MEP amplitudes in six muscle groups at three time points: when the patient was turned prone (baseline; T1), one hour after incision (T2), and after exposure of the spine but before insertion of the first screw (T3). RESULTS: In all muscles tested, the mean MEP amplitude was reduced by T3 when dexmedetomidine was infused at 0.5 µg·kg-1·hr-1. The greatest reduction from baseline MEP amplitude was 829 µV (95% confidence interval, 352 to 1230; P < 0.001) seen in first right dorsus interosseous. When dexmedetomidine was infused at 0.3 µg·kg-1·hr-1, there was a significant reduction in MEP amplitude in four of the six muscles tested at T3 compared with the control group. CONCLUSIONS: Dexmedetomidine at commonly used infusion rates of 0.3 µg·kg-1·hr-1 or 0.5 µg·kg-1·hr-1 causes a significant decrease in MEP amplitude during pediatric PSFS. We suggest that dexmedetomidine should be avoided in children undergoing PSFS so as not to confuse the interpretation of this important neurophysiological monitor.

RéSUMé: OBJECTIF: Les potentiels évoqués moteurs (PEM) sont fréquemment utilisés lors de chirurgies de fusion spinale postérieure chez l'enfant afin de détecter une ischémie de la moelle épinière. La dexmédétomidine est de plus en plus utilisée comme adjuvant à l'anesthésie intraveineuse totale, mais son effet sur l'amplitude des PEM n'a été rapporté que de façon variable. L'objectif de cette étude était d'évaluer l'effet d'une perfusion de dexmédétomidine sur l'amplitude des PEM. MéTHODE: Pendant une chirurgie de fusion spinale postérieure, nous avons réalisé une étude cas témoins rétrospective auprès de 30 patients pédiatriques ayant reçu une perfusion 0,5 µg·kg−1·h−1 de dexmédétomidine, 10 patients ayant reçu 0,3 µg·kg−1·h−1 de dexmédétomidine, et 30 patients témoins n'ayant pas reçu de dexmédétomidine. Deux neurophysiologistes ont passé en revue les amplitudes des PEM dans six groupes musculaires à trois moments de la chirurgie : lorsque le patient a été tourné sur le ventre (valeur de base; T1), une heure après l'incision (T2), et après l'exposition de la colonne mais avant l'insertion de la première vis (T3). RéSULTATS: Dans tous les muscles testés, l'amplitude moyenne des PEM était réduite à T3 lorsque la dexmédétomidine était perfusée à 0,5 µg·kg−1·h−1. La plus grande réduction par rapport à l'amplitude de base des PEM était de 829 µV (intervalle de confiance 95 %, 352 à 1230; P < 0,001) et a été observée au niveau du premier interosseux dorsal. Lorsque la dexmédétomidine était perfusée à 0,3 µg·kg−1·h−1, une réduction significative de l'amplitude des PEM a été observée dans quatre des six muscles testés à T3 par rapport au groupe témoin. CONCLUSION: La dexmédétomidine, administrée à des taux de perfusion fréquemment utilisés de 0,3 µg·kg−1·h−1 ou 0,5 µg·kg−1·h−1, a entraîné une réduction significative de l'amplitude des PEM pendant une chirurgie de fusion spinale postérieure chez l'enfant. Nous proposons d'éviter l'administration de dexmédétomidine chez les enfants devant subir une chirurgie de fusion spinale postérieure afin de ne pas brouiller l'interprétation de ce moniteur neurophysiologique important.

Image guided sacroiliac joint corticosteroid injections in children: an 18-year single-center retrospective study.

BACKGROUND: Sacroiliitis is commonly seen in enthesitis-related arthritis (ERA), a subtype of juvenile idiopathic arthritis (JIA). Sacroiliitis is characterized by the inflammation of the sacroiliac (SI) joints (+/- adjacent tissues). The treatment options include systemic therapy with or without corticosteroid SI joint injections. Image guided SI joint injections are frequently requested in pediatric patients with sacroiliitis. The purpose of this study was to evaluate the feasibility and efficacy of SI joint injections in children with sacroiliitis. METHODS: A retrospective study of patients referred to Interventional Radiology (IR) for SI joint corticosteroid injections (2000-2018). Clinical information was collected from Electronic Patient Charts and procedural details from PACS. Efficacy was determined clinically, by MRI, or both when available. RESULTS: 50 patients (13.8 years; M:F = 35:15) underwent image-guided SI joint corticosteroid injections. Most common indications were JIA (84%) and inflammatory bowel disease (14%). 80% had bilateral injections. 80% were performed under general anesthesia and 20% under sedation. The corticosteroid of choice was triamcinolone hexacetonide in 98% of patients. Needle guidance and confirmation was performed using CT and fluoroscopy (54%), Cone Beam CT (CBCT, 46%), with initial ultrasound assistance in 34%. All procedures were technically successful without any complications. 32/50 patients had long-term follow-up (2 years); 21/32 (66%) had clinical improvement within 3-months. Of 15 patients who had both pre- and post-procedure MRIs, 93% showed short-term improvement. At 2 years, 6% of patients were in remission, 44% continued the same treatment and 47% escalated treatment. CONCLUSION: Image-guided SI joint injections are safe and technically feasible in children. Imaging modalities for guidance have evolved, with CBCT being the current first choice. Most patients showed short-term clinical and imaging improvement, requiring long-term maintenance or escalation of medical treatment.

Hemodialysis Catheters in Infants: A Retrospective Single-Center Cohort Study.

PURPOSE: Evaluate technical aspects and outcomes of insertion/maintenance of hemodialysis (HD) central venous catheter (CVC) during infancy. MATERIALS AND METHODS: Single-center retrospective study of 29 infants who underwent 49 HD-CVC insertions between 2002 and 2016. Demographics, procedural, and post-procedural details, interventional radiology (IR) maintenance procedures, technical modifications, complications, and outcomes were evaluated. Technical adjustments during HD-CVC placement to adapt catheter length to patient size were labeled "modifications." CVCs requiring return visit to IR were called IR-maintenance procedures. Mean age and weight at HD-CVC insertion were 117 days and 4.9 kg. RESULTS: Of the 29 patients, 13 (45%) required renal-replacement-therapy (RRT) as neonates, 10 (34%) commenced RRT with peritoneal dialysis (PD), and 19 (66%) with HD. Fifteen nontunneled and 34 tunneled HD-CVCs were inserted while patients were ≤1 year. Technical modifications were required placing 25/49 (51%) HD-CVCs: 5/15 (33%) nontunneled and 20/34 (59%) tunneled catheters (P = .08). Patients underwent ≤6 dialysis-cycles/patient during infancy (mean 2.3), and a mean of 4.1 and 49 HD-sessions/catheter for nontunneled and tunneled HD-CVCs, respectively. Mean primary and secondary device service, and total access site intervals for tunneled HD-CVCs were 75, 115, and 201 days, respectively. A total of 26 of 49 (53%) patients required IR-maintenance procedures. Nontunneled lines had greater catheter-related bloodstream infections per 1,000 catheter-days than tunneled HD-CVCs (9.25 vs. 0.85/1,000 catheter days; P = .02). Nineteen patients (65%) survived over 1 year. At final evaluation (December 2017): 8/19 survived transplantation, 5/19 remained on RRT, 2/19 completely recovered, 1/19 lost to follow-up, and 3 died at 1.3, 2, and 10 years. CONCLUSIONS: Placement/maintenance of HD-CVCs in infants pose specific challenges, requiring insertion modifications, and IR-maintenance procedures to maintain function.

Clinical Impact of Chronic Venous Changes Induced by Central Lines in Children: A Cohort with Abnormal Venograms.

PURPOSE: To explore the hypothesis that central venous stenosis/obstructions (CVS/O) in children are influenced by prior central venous access devices (CVADs) and are associated with future risk for thromboses. MATERIAL AND METHODS: A convenience sample of 100 patients with abnormal venography (stenosis, collaterals, occlusions) documented during peripherally inserted central catheter (PICC) placements were identified from consecutive PICC placements (January 2008 to November 2012). The patients (41 males, 59 females, median age 2.7 years, median weight 11 kg) were categorized based on venographic presence (Group A, n = 53) or absence (Group B, n = 47) of visible connection to the superior vena cava. Each patient's CVAD history, before and after venography, was analyzed (until October 2016). RESULTS: Before venogram, Group B patients were associated with a higher number of previous CVADs, larger diameter devices, greater incidence of malposition, and more use of polyurethane catheters than Group A patients (P < .001). An ipsilateral PICC was successfully placed in 98% of Group A, compared to 32% of Group B (P < .001). After venogram, significantly more Doppler ultrasounds (DUS) were performed and thromboses diagnosed in Group B (57% and 36%) compared to Group A (21% and 8%) (P < .003; P = .001), respectively. CONCLUSIONS: Previous catheter characteristics influenced the severity of venographic changes of CVS/O (Group B). Group B was associated with more subsequent symptomatic thromboses. This information may assist parents and referring physicians to anticipate potential adverse sequelae from CVS/O on the child's venous health.