MR elastography-based staging of liver fibrosis in Fontan procedure associated liver disease is confounded by effects of venous congestion.

AIM: To compare targeted and global liver stiffness measured by magnetic resonance elastography (MRE) with liver biopsy in patients who have undergone the Fontan procedure, and to assess the relationship between liver stiffness and fibrosis stage. MATERIALS AND METHODS: Targeted and global liver stiffness was compared with a quantification of liver fibrosis measured by percentage of Sirius Red (%SR) staining of biopsy samples. MRE values were compared with three other biopsy-scoring methods: Ishak, Scheuer/Ludwig-Batts/Metavir, and congestive hepatic fibrosis score (CHFS). Additionally, in patients who had two or more MRE studies, global liver stiffness was compared for longitudinal assessment. RESULTS: Thirty-four patients were included in the study, with a mean age of 16.2 years. There was no statistically significant correlation between MRE-derived liver stiffness and Ishak score, Metavir score, %SR staining, and CHFS score. Twenty patients had multiple MRE studies, with a mean age of 16.5 years, and these showed a statistically significant increase in mean liver stiffness from 3.72 to 4.68 (26% increase) within an average period of 24 months. CONCLUSIONS: The lack of correlation of liver stiffness with fibrosis stage observed in this study indicates that the effects of venous congestion in Fontan patients can confound the use of liver stiffness as a biomarker for fibrosis as assessed by percentage of SR staining, Ishak score, Metavir score, and CHFS score. These results provide motivation for further development of magnetic resonance imaging-based biomarkers to increase the specificity in the assessment of Fontan-associated liver disease. A steady increase in liver stiffness observed in these patients may be useful for longitudinal follow-up of liver health.

New onset dysphagia and pulmonary aspiration following sclerotherapy for a complex cervical venolymphatic malformation in an infant: Case report and review of the literature.

We present the case of a 2-year-old male with a complex left cervical venolymphatic malformation who underwent doxycycline sclerotherapy at 12 months of age complicated by new onset pulmonary aspiration. A review of the literature reveals this to be a rare complication of sclerotherapy and only the second reported case. METHODS: Procedural details with associated imaging including endoscopic airway and swallowing evaluation are included. A literature review of cervical and laryngeal sclerotherapy complications was performed and discussed. RESULTS: A 12-month-old male underwent sclerotherapy with doxycycline for a complex parapharyngeal and paralaryngeal venolymphatic malformation. The postoperative course was complicated by new onset dysphagia, aspiration, and decreased laryngeal sensation. Gastric feeding and swallowing therapy were necessary due to prolonged difficulty. The sclerotherapy treatment resulted in near elimination of the cervical components of the lesion at 12 months follow up. The child progressed to total oral feeding by 17 months post-treatment with no evidence of decreased laryngeal sensation. An extensive literature review identified only one reported case of new onset dysphagia and decreased laryngeal sensation after doxycycline sclerotherapy. CONCLUSIONS: Doxycycline sclerotherapy for cervical venolymphatic malformations rarely can cause adjacent neural injury resulting in laryngeal complications. Our case report and literature review suggest that symptom management and appropriate aspiration precautions are necessary in infants or children with presumed vagus or laryngeal nerve injury, and injury is likely only temporary.

Percutaneous sclerotherapy of foot venous malformations: evaluation of clinical response.

AIM: To evaluate a single institutional experience with percutaneous sclerotherapy of venous malformations (VM) of the foot. MATERIALS AND METHODS: Sixteen patients (mean age 14.6 years; range 6-27.3 years), who underwent 34 sclerotherapy procedures were retrospectively analysed. Technical success, Puig classification, VM size reduction, and the complication rate were evaluated. In procedures in which C-arm computed tomography (CT) was performed, the VM-to-skin surface distance was measured. Additionally, an e-mail-based questionnaire to evaluate the response to sclerotherapy was answered by the patients. RESULTS: Technical success was 97%. The mean number of procedures per patient was 2.1 (range 1-5). In all procedures, sodium tetradecyl sulphate foam was used. Appropriate follow-up was available for 29/33 procedures (88%). Post-procedural complications occurred after 6/29 procedures (21%), all of which were self-limited skin complications. C-arm CT was performed in 19/33 procedures (58%). The lesion-to-skin surface distance was significantly shorter in patients with skin post-procedural complications (p < 0.001). The e-mail-based questionnaire was completed by 13/16 patients (81%). Decrease in swelling, improvement of foot function and a significant decrease in pain (p = 0.003) was reported. No patient reported dis-improvement after sclerotherapy. CONCLUSION: Percutaneous sclerotherapy is an effective option for treating foot VMs. Skin complication rates are higher with shorter VM-to-skin surface distance.

Preoperative angiography and external carotid artery embolization of juvenile nasopharyngeal angiofibromas in a tertiary referral paediatric centre.

AIM: To evaluate the relationship between intraoperative blood loss and juvenile nasopharyngeal angiofibroma (JNA) vascular supply and tumour stage in patients who underwent superselective external carotid artery (ECA) embolization. This series is unique in that all embolizations were performed by dedicated paediatric interventional radiologists at a tertiary referral paediatric centre. MATERIALS AND METHODS: Seventeen male patients treated from January 2002 to August 2009 underwent preoperative angiography and embolization using polyvinyl alcohol (PVA) particles. Tumours were graded using three different staging systems based on preoperative imaging and correlated to surgical blood loss. All patients underwent bilateral internal and external carotid angiography, with embolization of ECA tumour supply via microcatheter delivery of PVA particles. Particle size ranged from 150-500 µm with a mean size of 250-355 µm. Surgical resection was performed with either endoscopic or open techniques within 24 h and intraoperative blood loss was reported. RESULTS: Seven lesions were supplied strictly by the ECA circulation and had mean surgical blood loss of 336 ml. Twelve lesions had both ECA and internal carotid artery (ICA) supply and had mean surgical blood loss of 842 ml. The difference in blood loss in these two groups was statistically significant (p = 0.03). There was no case of inadvertent intracranial or ophthalmic embolization. There were statistically significant correlations between estimated surgical blood loss and the Andrews (p = 0.008), Radkowski (p = 0.015), and University of Pittsburgh Medical Center (UPMC; p = 0.015) preoperative tumour staging systems, respectively. CONCLUSION: Preoperative embolization of JNA tumours can be safely performed without neurological complications. The present study identified a statistically significant difference in intraoperative blood loss between those lesions with a purely ECA vascular supply and a combination of ECA and ICA vascular supply. Angiography is helpful in delineating ICA supply and can help guide surgical planning.

Distal airway foreign bodies: Importance of a stepwise approach, knowledge of equipment and utilization of other services' expertise.

Many distal airway foreign bodies present as obstructive atelectasis and may be removed using instruments passed through rigid bronchoscopes. Deeply impacted distal foreign bodies remain problematic and sometimes require thoracotomy. The purpose of this paper is to discuss alternate approaches to avoid open surgical removal. A clinical algorithm is outlined. A young child presented to the hospital with an episode of coughing and oxygen desaturation. A chest radiograph demonstrated a radiopaque foreign body in the right upper lobe with distal atelectasis. The foreign body could not be found using rigid bronchoscopy so a flexible bronchoscope (3.5mm) was used to identify the distal primary tooth that was lodged in the inflamed tertiary segment of the bronchus. The tooth could not be removed using instruments passed through the sideport of the bronchoscope including balloon catheters, ureteral baskets, and biopsy forceps. The catheters were too compliant to reach the upper lobe and the forceps could not engage the tooth. A decision was made to treat the child with steroids and attempt removal 48 h later. The inflammation had resolved and the tooth had shifted positions to a more accessible left lower lobe tertiary segment. Using fluoroscopy with guide wires through the endotracheal tube, the tooth was removed. Tools used by different services are available as well to retrieve foreign bodies and may obviate the need for thoracotomy. Steroids decreased swelling allowing better access to the foreign body.

Non-endoscopic removal of radiologically placed percutaneous primary gastrostomy tubes: a new technique.

PURPOSE: To present a new technique for non-endoscopic removal of radiologically inserted primary percutaneous gastrostomy tubes (PGT) and to assess the utility and safety of this technique in the pediatric population. MATERIALS AND METHODS: Over a 9-year period 172 children (80 F, 92 M) mean age 10 years (range 0.29-24 years) underwent removal of radiologically placed PGTs in the Radiology Department. All procedures were performed with conscious sedation. The procedure time ranged from 20 to 45 min (mean 30 min). The procedures were performed electively at > or = 3 14 months for either gastrostomy button placement (70), balloon gastrostomy and jejunostomy placement (55), or G tube removal at termination of enteral feeding (15). RESULTS: PGT disc retrievals were successful performed in 169 of 173 (98%). No technical failures occurred, but five procedures failed due to PGT disc lodgement in gastric wall (four), and gastrocolic fistula (one). One child had a repeat procedure due to premature removal of the primary PGT necessitating replacement. No deaths occurred. PGT discs retrieval was performed at a mean time interval of 14 months from initial PGT insertion (range 3-40 months). CONCLUSION: Non-endoscopic GT disc removal is a quick and safe procedure. It facilitates easy conversion to a gastrostomy button in the radiology setting and avoids the risk of a retained percutaneous gastrostomy tube remnant and intra/transmural migration of discs.

"Push-pull" gastrostomy: a new technique for percutaneous gastrostomy tube insertion in the neonate and young infant.

OBJECTIVE: To evaluate a newly developed method combining antegrade and retrograde techniques for percutaneous gastrostomy tube (PGT) insertion in the neonate and young infant. MATERIALS AND METHODS: From January 1994 to December 2000, 85 children (47 male, 38 female), mean age 4.5 months (range 0.44-9.13 months) underwent PGT insertion using the "push-pull" technique. With the addition, 57 children had a jejunostomy tube placed as well at or within 24 h of the PGT procedure. The mean weight was 3.74 kg, range 1.5-7.0 kg. The indications for the procedure included failure to thrive in 40 patients (25%), static encephalopathy in 21 (25%), neurological/congenital abnormalities in 12 (14%), aspiration in 7 (8%), and cardiac problems in 5 (6%). RESULTS: Eighty-five PGTs were successfully inserted in 85 children. One procedure was initially unsuccessful due to failed conscious sedation and was completed under general anesthesia. Four of 85 patients initially had attempted antegrade placement that failed, and the procedure was successfully completed using the "push-pull" method. One major complication occurred: a gastrocolic fistula at day 5 post-procedure, which was surgically repaired without sequelae. Tube-related problems included; tube dislodgement (n = 1) and procedure-related stomal infection (n = 3). CONCLUSION: The "push-pull" gastrostomy technique is a safe, effective method of percutaneous gastrostomy tube placement in neonates. It facilitates successful placement of the PGT in patients in whom the classic antegrade method is not possible. It has become the procedure of choice in this group.

Infectious aneurysm clipping by an MRI/MRA wand-guided protocol. A case report and technical note.

Infectious aneurysms are potentially deadly sequelae of multiple etiologies, typically associated with subacute bacterial endocarditis (SBE). Since these aneurysms tend to be distal, there are no consistent landmarks by which to localize them, in contrast to more typical aneurysms that occur on the circle of Willis or proximal, large cerebral vessel bifurcations. In addition, they tend to be extremely friable and may be obscured by blood if intracranial hemorrhage (ICH) has already occurred. These factors make clipping these aneurysms technically difficult, and searching for easily ruptured aneurysms without standard landmarks adds risk to the procedure. In this report, we describe the case of a 9-year-old boy with SBE and subsequent ICH secondary to a mycotic aneurysm. This aneurysm was localized to within millimeters by the MRI protocol described herein. The aneurysm was excised and the patient recovered without incident. Thus, MRI/MRA-guided frameless stereotaxy may be useful for localizing distal mycotic aneurysms, improving patient outcome by decreasing morbidity and mortality.

Lingual artery embolization for severe and uncontrollable postoperative tonsillar bleeding.

We performed emergent angiography of the external carotid artery to evaluate a patient who experienced uncontrollable oropharyngeal bleeding 3 days following an adenotonsillectomy. Angiography demonstrated a left lingual artery disruption and hemorrhage. We then performed a coil embolization of the left lingual artery, which immediately halted the hemorrhage. We conclude that coil embolization is a rapid and effective strategy for the management of massive, uncontrollable postoperative tonsillar bleeding.