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.

The use of rapamycin to treat vascular tumours and malformations: A single-centre experience.

OBJECTIVES: To assess the safety and efficacy of rapamycin in treating children with vascular tumours and malformations. STUDY DESIGN: We performed a retrospective review at a large tertiary care paediatric centre to assess the efficacy and safety of using rapamycin to treat vascular tumours and malformations. Response to therapy was defined by patient-reported symptom improvement, radiological reduction in size of lesions, and/or improvement of laboratory parameters. RESULTS: Forty-two patients (7 with vascular tumours and 35 with vascular malformations) have been treated with rapamycin. Despite 33 of 42 patients being diagnosed in the first year of life, the median age of initiating rapamycin was 11 years. Of the 38 children treated for a minimum of 4 months, 29 (76%) exhibited a clinical response. Twenty-one patients had follow-up imaging studies and of these, 16 (76%) had radiographic decrease in lesion size. Median time to demonstration of response was 49 days. All five children with vascular tumours and all three children with vascular malformations under the age of 4 years showed a clinical response. Response rate was lower for children ≥ 4 years of age (0/2, 0% for vascular tumours; 21/28, 75% for vascular malformations). No patient experienced an infection directly related to rapamycin or discontinued rapamycin due to toxicity. CONCLUSIONS: Rapamycin is safe and efficacious in most children with select vascular tumours and malformations. Young children appear to respond better, suggesting that early initiation of rapamycin should be considered.

Use of Tissue Plasminogen Activator in Abdominal Abscesses in Children-A Single-Center Randomized Control Trial.

PURPOSE: To establish the efficacy of once-per-day intracavitary tissue plasminogen activator (tPA) in the treatment of pediatric intra-abdominal abscesses. METHODS: A single-center prospective, double-blinded, randomized controlled trial of the use of intracavitary tPA in abdominal abscesses in children. Patients were randomized to either tPA-treatment or saline-treatment groups. Primary outcome was drainage catheter dwell (hours). Secondary outcomes were length of hospital stay, times to discharge, clinical and sonographic resolution, and adverse events (AEs). RESULTS: Twenty-eight children were randomized to either group (n = 14 each). Demographics between groups were not significantly different (age P = .28; weight P = .40; gender P = .44). There were significantly more abscesses in the tPA-treated group (P = .03). Abscesses were secondary to perforated appendicitis (n = 25) or postappendectomy (n = 3). Thirty-four abscesses were drained, 4 aspirated, 3 neither drained/aspirated. There was no significant difference in number of drains (P = .14), drain size (P = .19), primary outcome (P = .077), or secondary outcomes found. No procedural or intervention drug-related AEs occurred. No patient in the saline-treated group required to be switched/treated with tPA. CONCLUSION: No significant difference in the length of catheter dwell time, procedure time to discharge, or time to resolution was found. Intracavitary tPA was not associated with morbidity or mortality. The results neither support nor negate routine use of tPA in the drainage of intra-abdominal abscess in children. It is possible that a multicentre study with a larger number of patients may answer this question more definitively.

Abnormal hemostasis in children with vascular anomalies, part I: Thrombocytopenias among different vascular anomalies.

Long before the classification of vascular anomalies from the International Society for the Study of Vascular Anomalies (ISSVA) provided a framework to differentiate vascular anomalies, otherwise known as vascular birthmarks, it was recognized that patients with such lesions can present with acute life-threatening hemostatic and/or thrombotic complications, as well as chronic long-standing bleeding or thrombotic issues. Scenarios such as a rapidly growing vascular lesion with severe acute thrombocytopenia, a visceral hemorrhagic lesion, a lesion associated with repetitive and painful superficial thrombosis, and cases of unprovoked or post-procedural fatal pulmonary embolism highlight the wide spectrum of manifestations of abnormal coagulation in patients with vascular anomalies. The separation of vascular anomalies into two distinct groups, vascular tumors and vascular malformations, was followed by the characterization that their respective coagulopathies were due to either a derangement of platelets or to a disequilibrium of the patient's coagulation/fibrinolytic process. This configuration of coagulopathies will be the foundation for this two-chapter review series. In the initial review, coagulopathies where thrombocytopenia is the main feature will be characterized, whereas the second review will focus on vascular malformations that have a coagulation disorder secondary to some degree of coagulation consumption and/or fibrinolytic pathway derangement.

Klippel-Trenaunay Syndrome.

Klippel-Trenaunay syndrome or KTS is a complex vascular syndrome associated with overgrowth occurring as a result of somatic mutations in the PIK3CA gene. Patients are diagnosed on the basis of physical findings, sometimes with supportive imaging, of commonly a segmental anomaly with a cutaneous port-wine stain, lymphatic and venous malformations and overgrowth. The severity of the component vascular malformations and the degree of overgrowth varies from patient to patient which demands care given by a multi-professional team with regular follow-up in a specialist clinic. Some patients may present with acute life-threatening problems, often as a result of veno-thromboembolic events (VTEs) especially following surgical and invasive radiological procedures. Awareness of such problems is vital and prophylactic measures to reduce such risks are paramount. The interventional radiologist is vital to the care team as he/she can undertake procedures including endovascular closure of significant venous anomalies which predispose to such VTEs. Although these procedures can be lengthy and complex, they can now provide a minimally invasive means to reduce the risk from life-threatening and sometimes fatal VTEs. The results however from such interventions will require long-term studies which to date are unavailable.

Endovenous laser ablation therapy in children: applications and outcomes.

BACKGROUND: Endovenous laser ablation is well recognized as the first-line treatment for superficial venous reflux with varicose veins in adults. It is not widely reported and is not an established practice in pediatric patients. OBJECTIVE: To illustrate a variety of pediatric venous conditions in which endovenous laser ablation can be utilized and to demonstrate its feasibility and safety in children. MATERIALS AND METHODS: We conducted a retrospective review of endovenous laser ablation procedures performed between January 2007 and July 2014 at two large pediatric institutions. RESULTS: We included 35 patients (17 males) who underwent endovenous laser ablation to 43 veins. Median age at first treatment was 14 years (range: 3-18 years). Median weight was 56 kg (range: 19-97 kg). Underlying diagnoses were common venous malformation (15), Klippel-Trenaunay syndrome (8), superficial venous reflux with varicose veins (5), verrucous hemangioma-related phlebectasia (4), venous varix (2) and arteriovenous fistula (1). The most common aim of treatment was to facilitate sclerotherapy. Thirty-four patients had treatment in the lower limbs and one patient in an upper limb. Ten of the veins treated with endovenous laser ablation had an additional procedure performed to close the vein. Complications attributable to endovenous laser ablation occurred in two patients (6%). One patient experienced post-procedural pain and one patient developed a temporary sensory nerve injury. Median clinical follow-up was 13 months (range: 28 days-5.7 years). The aim of the treatment was achieved in 29 of the 35 (83%) patients. CONCLUSION: Endovenous laser ablation is technically feasible and safe in children. It can be used in the management of a range of pediatric venous diseases with good outcomes.

Peritonitis following percutaneous gastrostomy tube insertions in children.

BACKGROUND: Percutaneous retrograde gastrostomy has a high success rate, low morbidity, and can be performed under different levels of sedation or local anesthesia in children. Despite its favourable safety profile, major complications can occur. Few studies have examined peritonitis following percutaneous retrograde gastrostomy in children. OBJECTIVE: To identify potential risk factors and variables influencing the development and early diagnosis of peritonitis following percutaneous retrograde gastrostomy. MATERIALS AND METHODS: We conducted a retrospective case-control study of children who developed peritonitis within 7 days of percutaneous retrograde gastrostomy between 2003 and 2012. From the 1,504 patients who underwent percutaneous retrograde gastrostomy, patients who developed peritonitis (group 1) were matched by closest date of procedure to those without peritonitis (group 2). Peritonitis was defined according to recognized clinical criteria. Demographic, clinical, procedural, management and outcomes data were collected. RESULTS: Thirty-eight of 1,504 children (2.5%; 95% confidence interval, 1.8-3.5) who underwent percutaneous retrograde gastrostomy developed peritonitis ≤7 days post procedure (group 1). Fever (89%), irritability (63%) and abdominal pain (55%) occurred on presentation of peritonitis. Group 1 patients were all treated with antibiotics; 41% underwent additional interventions: tube readjustments (8%), aspiration of pneumoperitoneum (23%), laparotomy (10%) and intensive care unit admission (10%). In group 1, enteral feeds started on average 3 days later and patients were discharged 5 days later than patients in group 2. There were two deaths not directly related to peritonitis. Neither age, gender, weight, underlying diagnoses nor operator was identified as a risk factor. CONCLUSION: Peritonitis following percutaneous retrograde gastrostomy in children occurs in approximately 2.5% of cases. No risk factors for its development were identified. Medical management is usually sufficient for a good outcome. Patients with peritonitis are delayed starting feeds and have a hospital stay that is an average of 5 days longer than those without.

IR Approaches to Difficult Removals of Totally Implanted Venous Access Port Catheters in Children: A Single-Center Experience.

PURPOSE: To identify factors associated with adherence of implanted venous access port catheters in children and describe technical strategies for removing "stuck" ports. MATERIALS AND METHODS: A retrospective single-center review of port removals was conducted between 2003 and 2012. Cases were identified through radiology reports. Clinical details (eg, demographics, disease, port dwell time, interventional techniques) were obtained through patient charts. Cases were classified as difficult removals if there was documented adherence to soft tissues or vein, or simple removals if no difficulty was recorded. Difficult removals were categorized and graded on increasing invasiveness of techniques required. Successful removal was defined as complete removal of the port catheter. Difficult removals were compared with simple removals for factors associated with difficult removal. Of all removals (N = 1,306), 58 were classified as difficult removals (4%). RESULTS: Using various techniques, 57 of 58 (98%) adherent port catheters were successfully removed. Factors identified with difficult removals included primary diagnosis of acute lymphoblastic leukemia (ALL) (78% vs 37%, P < .0001), age at insertion (3.7 y vs 5.4 y, P = .0019), and port dwell time (median 1,087 d vs 616 d, P < .0001). CONCLUSIONS: Difficulty removing port catheters in children is uncommon. Port catheters can usually be removed successfully using various IR techniques ranging in invasiveness. There is an association of difficult removal with early age at insertion, ALL diagnosis, and long port dwell time. Awareness of these factors may help physicians inform parents of potential difficulties and plan the removal procedure.

Are cuffed peripherally inserted central catheters superior to uncuffed peripherally inserted central catheters? A retrospective review in a tertiary pediatric center.

PURPOSE: To assess the use of cuffed peripherally inserted central catheters (PICCs) compared with uncuffed PICCs in children with respect to their ability to provide access until the end of therapy. MATERIALS AND METHODS: A retrospective review of PICCs inserted between January 2007 and December 2008 was conducted. Data collected from electronic records included patient age, referring service, clinical diagnosis, inserting team (pediatric interventional radiologists or neonatal intensive care unit [NICU] nurse-led PICC team), insertion site, dates of insertion and removal, reasons for removal, and need for a new catheter insertion. A separate subset analysis of the NICU population was performed. Primary outcome measured was the ability of the PICCs to provide access until the end of therapy. RESULTS: Cuffed PICCs (n = 1,201) were significantly more likely to provide access until the end of therapy than uncuffed PICCs (n = 303) (P = .0002). Catheter removal before reaching the end of therapy with requirement of placement of a new PICC occurred in 26% (n = 311) of cuffed PICCs and 38% (n = 114) of uncuffed PICCs. Uncuffed PICCs had a significantly higher incidence of infections per 1,000 catheter days (P = .023), malposition (P = .023), and thrombus formation (P = .022). In the NICU subset analysis, cuffed PICCs had a higher chance of reaching end of therapy, but this was not statistically significant. CONCLUSIONS: In this pediatric population, cuffed PICCs were more likely to provide access until the end of therapy. Cuffed PICCs were associated with lower rates of catheter infection, malposition, and thrombosis than uncuffed PICCs.

Symptomatic liver involvement in neonatal hereditary hemorrhagic telangiectasia.

High-flow hepatic vascular anomalies with arteriovenous shunting commonly manifest during the neonatal period with signs and symptoms of congestive heart failure, but to our knowledge, they have never been described in patients with hereditary hemorrhagic telangiectasia (HHT). We report here our experience with 3 patients with hepatic arteriovenous malformations (AVMs) who presented with symptoms of high-output congestive heart failure during the neonatal period and were subsequently diagnosed with HHT. Imaging showed large hypervascular lesions and multiple hepatic arteriovenous shunts that differentiated these lesions from liver hemangiomas. Transcatheter embolization was performed in all cases. One infant died of sepsis shortly after embolization; follow-up at the age of 2.5 years of the surviving infants revealed involution of the vascular lesions and no evidence of symptom recurrence. We conclude that severe symptoms related to hepatic AVMs in HHT can occur in the neonatal period and that HHT should therefore be included in the differential diagnosis of symptomatic neonatal hepatic vascular malformations. Imaging plays a key role in differentiating hepatic AVMs from hemangiomas, because the latter require additional pharmacologic treatments. Early transcatheter embolization seems to be effective, but long-term outcomes still need to be assessed.

Use and accuracy of US guidance for image-guided injections of the temporomandibular joints in children with arthritis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) has an incidence that ranges from 1 to 22 per 100,000 children worldwide, with involvement of the temporomandibular joint (TMJ) in 17-87% of patients. Intraarticular corticosteroid injections are beneficial in the local treatment of JIA and of other types of arthritis. OBJECTIVE: To describe and assess the accuracy of an US-guided technique for visualization of needle placement within the TMJ in children. MATERIALS AND METHODS: Between January 2000 and November 2007, 180 TMJ injections were performed during 116 encounters in 83 children with arthritis (71 girls, 12 boys; mean age 12.0 years). Access was obtained under sterile conditions using US guidance (linear 15-MHz or curvilinear 8-MHz transducers) in a coronal plane, and confirmed with CT. To minimize radiation, a limited focused CT protocol was developed. RESULTS: A bilateral injection was performed in 65 encounters (57%). Twenty-three children had repeat TMJ injections. All injections were performed using US guidance. CT confirmation was used in 127/180 TMJs (70%). In those confirmed with CT, the needle tip was intra-articular in 91% of cases. Triamcinolone hexacetonide was used in 92% of injections and triamcinolone acetonide in 8%. One major complication was encountered (skin atrophy at the injection site). CONCLUSION: In our experience, TMJ injections using sonographic guidance is a safe, effective and accurate procedure.

Symptomatic caval penetration by a Celect inferior vena cava filter.

We report a case of penetration of the inferior vena cava (IVC) by all four primary struts of a Celect caval filter in a 17-year-old girl with Klippel-Trénaunay syndrome. The girl presented with acute lower abdominal and right leg pain 17 days after filter insertion. An abdominal radiograph demonstrated that the filter had moved caudally and that the primary struts had splayed considerably since insertion. Contrast-enhanced CT confirmed that all four primary struts had penetrated the IVC wall. There was a small amount of retroperitoneal hemorrhage. The surrounding vessels and viscera were intact. The filter was subsequently retrieved without complication.

Delayed, life-threatening lower gastrointestinal hemorrhage in an infant after serial transverse enteroplasty: treatment with transcatheter n-butyl-2-cyanoacrylate embolization.

We report a case of n-butyl-2-cyanoacrylate glue embolization of life-threatening lower gastrointestinal (LGI) hemorrhage in a 10-month-old boy. The child had a history of gastroschisis and short-bowel syndrome. Six months prior to the LGI bleed, he had undergone a serial transverse enteroplasty (STEP) to lengthen his intestine. To the best of our knowledge this is both the first report of successful glue embolization for LGI bleeding in a child and also the first report of severe hemorrhage after the STEP procedure.

Early presentation of an extremity arteriovenous malformation.

We report a very rare case of a high flow arteriovenous malformation (AVM) of the upper limb that caused high output cardiac failure at birth. There was early transfer of the baby to the care of a multidisciplinary team. After radiological intervention, the arm distal to the malformation became ischaemic and an urgent amputation through the upper-humerus followed. Methods of treatment are discussed, together with a review of results in the literature.

Intraosseous haemangioma of the mandible: a case report.

An unusual case of an endangering intraosseous haemangioma affecting the mandible in early childhood is presented. The early diagnosis was unclear until a surgical biopsy was performed. MRI confirmed the diagnosis. Successful emergent arterial embolisation and intralesional steroid injection were undertaken to control troublesome bleeding prior to medical treatment.