Sheena Pimpalwar

Imaging the Central Conducting Lymphatics: Initial Experience with Dynamic MR Lymphangiography

PURPOSE To describe a dynamic magnetic resonance (MR) lymphangiography technique after intranodal injection of gadolinium-based contrast agent and to assess its feasibility for evaluation of the central conducting lymphatics (CCL) in patients with pathologic disorders that involve the CCL. MATERIALS AND METHODS A retrospective evaluation of experience with the dynamic MR lymphangiographic technique in six consecutive patients was performed after institutional review board approval. Written informed consent for the percutaneous procedure was obtained from the patient, parent, or the legally responsible guardian. The dynamic MR lymphangiographic technique involves ultrasonographically guided intranodal injection of gadolinium-based contrast material into the inguinal lymph nodes, combined with sequential imaging of the chest and abdomen with a three-dimensional sequence optimized for soft tissue with high spatial resolution that provides time-resolved imaging of lymphatic transit through the CCL. Qualitative assessment of the images was performed for reliability of CCL visualization and for associated findings that could explain the clinical symptoms, including lymphangiectasia, chylolymphatic reflux, and chylous leak. RESULTS The procedure was technically successful in all six patients. The dynamic MR lymphangiographic findings confirmed the presence of normal CCL morphologic structure in two patients and provided a possible explanation for clinical manifestations in the remaining four patients. The dynamic MR lymphangiographic procedure led to a change in management in two patients, continuation of conservative treatment in three patients, and confirmation of an alternative nonlymphatic diagnosis in one patient. Image quality for visualization of the CCL was considered good in all cases by the two readers. There were no known adverse effects related to the procedure. CONCLUSION The dynamic MR lymphangiographic technique with intranodal injection of gadolinium-based contrast material is feasible and can provide useful information in a variety of lymphatic flow abnormalities involving the CCL.

Sildenafil for microcystic lymphatic malformations of the head and neck: A prospective study

BACKGROUND Microcystic lymphatic malformations (LM) are congenital birth defects that can cause severe functional or esthetic deformity. At this time, several treatment interventions are possible, but there is no ideal therapy. A recently published article noted a coincidental improvement in microcystic LMs with the use of sildenafil for pulmonary hypertension, but conclusive and reproducible data is lacking regarding its efficacy. METHODS AND RESULTS A prospective study was conducted to examine the subjective and objective results associated with sildenafil use in the treatment of microcystic LMs. Patients under the age of 18 were enrolled, and after evaluation with pre-intervention magnetic resonance imaging (MRI) studies, each was given a 6-week course of sildenafil. Subjective outcomes were obtained, and postoperative MRIs were used to objectively quantify changes. Five patients between the ages of 4 and 11 were enrolled in the study. All patients had microcystic LMs of the head and neck. All patients had undergone previous treatment interventions utilizing various modalities, and each was now seeking treatment for functional and cosmetic purposes. All patients had minimal to no response from a subjective standpoint. Comparison of pre- and post-treatment MRIs also demonstrated minimal to no response. CONCLUSIONS The use of sildenafil for the treatment of microcystic lymphatic malformations did not have any appreciable effect on our selected population of pediatric patients who had been resistant to previous therapies. Further studies would be necessary to determine if other cohorts may benefit.

Traumatic hepatic artery laceration managed by transarterial embolization in a pediatric patient

While blunt abdominal trauma with associated liver injury is a common finding in pediatric trauma patients, hepatic artery transection with subsequent treatment by transarterial embolization has rarely been reported. We present a case of a child who suffered from a hepatic artery injury which was successfully managed by supraselective transarterial microcoil embolization, discuss management strategies in these patients, and provide a review of currently available literature.

Multimodality Image-Guided Sclerotherapy of Low-Flow Orbital Vascular Malformations: Report of Single-Center Experience

PURPOSE To evaluate the role of multimodality imaging tools for intraprocedural guidance and outcome evaluation during sclerotherapy of low-flow orbital vascular malformations. MATERIALS AND METHODS A retrospective review was performed of 17 consecutive patients with low-flow orbital malformations (14 lymphatic, two venous, and one venolymphatic) who underwent multimodality image-guided sclerotherapy between November 2012 and May 2015. Sclerotherapy technique, image guidance tools, and complications were recorded. Sclerotherapy outcome was evaluated using clinical response, magnetic resonance (MR) image-based lesion volumetry, and proptosis quantification. RESULTS There were 22 sclerotherapy sessions performed. Intraprocedural ultrasound (US), fluoroscopy, cone-beam computed tomography (CT) and MR image fusion were used for image guidance with 100% technical success. Resolution of presenting symptoms was observed in all patients at 1-month follow-up. Four major sclerotherapy complications were successfully managed. Statistically significant reduction in lesion volume (P = .001) and proptosis (P = .0117) by MR image analysis was achieved in all patients in whom 3-month follow-up MR imaging was available (n = 13/17). There was no lesion recurrence at a median follow-up of 18 months (range, 8-38 mo). CONCLUSIONS Multimodality imaging tools, including US, fluoroscopy, cone-beam CT, and MR fusion, during sclerotherapy of low-flow orbital malformations provide intraprocedural guidance and quantitative image-based evaluation of treatment outcome.

Low-Flow Vascular Malformations of the Orbit: Evaluation and Management

Low flow vascular malformations of the orbit are uncommon, yet vision threatening lesions, that are difficult to manage surgically by virtue of their infiltrative morphology and tendency to recur. With the recent availability of advanced image guidance tools in the angiography suite, sclerotherapy has emerged as a minimally invasive treatment option for these lesions. A multi-disciplinary approach involving oculoplastic surgery, ophthalmology and interventional radiology is recommended for optimal management.

Oral Cavity and Oropharynx

Three vascular lesions are commonly encountered in the oropharynx. These are hemangioma, venous malformation, and lymphatic malformation. A fourth type of vascular anomaly, arteriovenous malformation, may also occur in the oropharynx but only as an extension of a nearby larger lesion. As such, it is better considered outside of the anatomic subsection of the oropharynx. Each of these three types will be discussed separately with respect to phenotype, etiology, natural history, and treatment.

Temporal Evolution and Management of Fast Flow Vascular Anomalies in PTEN Hamartoma Tumor Syndrome

Abstract Phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome (PHTS) is characterized by formation of recurrent benign tumors described as PTEN hamartoma of soft tissue that may contain fast flow vascular anomalies (FFVA). The purpose of this study is to review the temporal evolution and management of FFVA in PHTS. A retrospective review of 22 patients (9 males), age 1 to 18 (median 9) years diagnosed with PHTS at a tertiary care pediatric hospital between October 2002 and August 2017 revealed 4 patients with FFVA. Imaging, management, and treatment complications were reviewed. During median follow‐up of 8 (range: 4‐13) years, ultrasound and magnetic resonance imaging performed for recurrent pain, showed progressive increase in the size of hamartomas and development of new FFVA in three‐fourth patients. Medical management included pain medications, oral sirolimus, and physical and psychiatric therapy. Surgical excision of hamartoma (n = 1) resulted in recurrence within 3 months. Between 4 and 24 (average 1.5/year) embolizations were performed per patient. Pain related to FFVA responded well to embolization. Pain secondary to PTEN hamartoma responded poorly to percutaneous sclerosant injection, but demonstrated improvement with sirolimus. There was no correlation between serum sirolimus levels and frequency/timing of recurrence of FFVA/hamartoma. Complications included sclerosant migration into digital arteries (n = 1), subclavian vein stenosis due to glue migration (n = 1), oral mucositis (n = 4), and elevated triglycerides (n = 4). Patients with PHTS present with recurrent pain requiring life‐long management with a multi‐disciplinary team. Pain due to FFVA responds to embolization, and pain due to hamartoma responds to sirolimus. This improves quality of life, but does not prevent disease progression.

Dynamic contrast enhanced magnetic resonance lymphangiography: Categorization of imaging findings and correlation with patient management

OBJECTIVE To review the technical aspects and categorize the imaging findings of dynamic contrast enhanced magnetic resonance lymphangiography (DCMRL) and correlate the findings with patient management options. MATERIALS AND METHODS A retrospective review of patients who underwent DCMRL between June 2012 and August 2017 at a tertiary care paediatric hospital was performed. Twenty-five DCMRL studies were performed in 23 patients (9 males, 13 females, 1 ambiguous gender) with a median age of 4 years (range: 1 month-29 years). DCMRL imaging findings were reviewed, categorized and the impact on patient management was studied. RESULTS DCMRL was technically successful in 23/25 (92%) studies. DCMRL findings were categorized based on the status of central conducting lymphatics (CCL) and alternate lymphatic pathways as follows: Type 1 - normal CCL with no alternate lymphatic pathways, Type 2 - partial (2a) or complete (2b) non-visualization of CCL with reflux of contrast into alternate pathways and Type 3 - normal CCL with additional filling of alternate pathways. Type 1 DCMRL patients (n = 5) were reassured and conservative management was continued, Type 2 patients (n = 10) had evidence of CCL obstruction hence thoracic duct ligation or embolization was avoided and other options such as lymphatic fluid diversion using Denver® shunt or lympho-venous anastomosis were used, and Type 3 patients (n = 8) were evaluated for elevated central venous pressure as a cause of lymphatic backflow in addition to Denver® shunt, lympho-venous anastomosis, thoracic duct ligation or embolization. CONCLUSION DCMRL is an evolving imaging technique for understanding abnormalities of the central conducting lymphatics. Categorization of imaging findings may be helpful in guiding selection of management options.