Horacio M. Padua

Percutaneous Sclerotherapy of Lymphatic Malformations with Doxycycline

BACKGROUND Lymphatic malformations (LMs) can be effectively treated by percutaneous intralesional injection of a variety of sclerosant drugs. This study aims to evaluate the efficacy of doxycycline in the treatment of LMs. METHODS AND RESULTS We reviewed the medical records of all patients with LMs who underwent sclerotherapy with doxycycline between January 1, 2003 and September 1, 2004 at Childrens Hospital Boston. Follow-up imaging was performed to assess for change in lesion size. Surveys were sent to all study patients, to assess perceived improvements in symptoms and satisfaction with the results. Sixty sclerotherapy procedures were performed on 41 patients in the 20-month study period. The median age was 6.9 years (3 mo-31 y). The most common location was cervicofacial (66%), followed by truncal (19%) and extremity (15%). The most common lesion type was macrocystic (49%), followed by combined (44%) and microcystic (7%). The major and minor complication rates were 2% and 10%, respectively. The mean outcome score by imaging was 4.41/5 with a 95% CI of [4.13-4.68] corresponding to about an 83% reduction in lesion size, and by patient survey was 4.47/5 with a 95% CI of [4.15-4.79] corresponding to between a good to complete response. Higher complication rates were associated with microcystic and combined lesions (p = 0.03), and greater doxycycline dose (p = 0.05). CONCLUSIONS Doxycycline is a safe and effective sclerosant for LMs. Greater follow-up is necessary to quantify long-term outcomes and assess the risks of lesion recurrence.

Intranodal Lymphangiography: Feasibility and Preliminary Experience in Children

PURPOSE To review an initial experience studying the lymphatic system using direct injection of ethiodized oil contrast medium into lymph nodes (ie, intranodal lymphangiography) in children with chylous disorders. MATERIALS AND METHODS Children with chylous disorders who underwent intranodal lymphangiography were included in this retrospective study. Under general anesthesia, ultrasonography was used to guide the placement of a small-bore (22-25-gauge) needle into an inguinal lymph node. Ethiodized oil contrast medium was very slowly injected into the node. Opacification of the lymphatic system was documented with fluoroscopic and digital substraction imaging and videofluoroscopic clips. RESULTS Five children (age range, 6 wk to 17 y) with chylous vaginorrhea (n = 1), postoperative chylothorax (n = 2), or spontaneous chylothorax (n = 2) underwent intranodal lymphangiography. The amount of ethiodized oil injected was 0.5-4.5 mL. Intranodal lymphangiography was successfully completed in four patients. One procedure was terminated because of patient motion and extravasation of contrast medium. Lymphangiographic findings included a spectrum of lymphatic channel disorders including incompetence, obstruction, collateralization, chylous reflux, and chylous leak. There were no complications. CONCLUSIONS The simplified technique of injecting contrast medium into a lymph node to opacify the lymphatic system in children can be an alternative to the more elaborate conventional lymphangiography.

Lymphatic malformations: Diagnosis and management

Lymphatic malformations are benign vascular lesions that arise from embryological disturbances in the development of the lymphatic system. They encompass a wide spectrum of related abnormalities, including cystic lymphatic lesions, angiokeratoma, lymphatic malformations that occur in bones (Gorham-Stout Syndrome), lymphatic and chylous leak conditions, and lymphedema. This article will focus only on lymphatic malformation mass lesions, whereas other related disease entities will be covered elsewhere in this journal issue. Lymphatic malformations occur frequently in lymphatic-rich areas such as the head and neck region, but they can also be found on any anatomical site in the body. In general, lymphatic malformations are categorized into macrocystic, microcystic, or combined depending on the size of the cysts contained within the lesion. Lymphatic malformations can cause both deformation of the anatomical site involved and functional deficits. The goal of this article is to discuss the etiology, epidemiology, treatment modalities, and comorbidities associated with lymphatic malformations.

Fibro-adipose vascular anomaly: clinical-radiologic-pathologic features of a newly delineated disorder of the extremity.

Background: The diagnosis and management of vascular anomalies of the extremities can be challenging as these disorders are uncommon and may clinically overlap. The aim of this paper is to describe the clinical, radiologic, and histopathologic features of fibro-adipose vascular anomaly (FAVA), a previously unrecognized disorder of the limb. Methods: The clinical, imaging, operative, and histopathologic data from patients with a unique intramuscular lesion of the extremities comprising dense fibrofatty tissue and slow-flow vascular malformations were retrospectively reviewed. Results: Sixteen patients diagnosed with FAVA of the extremity (3 male and 13 female individuals) met the clinical, radiologic, and histopathologic inclusion criteria. The age at presentation ranged from the time of birth to 28 years. The locations of the lesions were: calf (n=10), forearm/wrist (n=3), and thigh (n=3). Fourteen patients presented with severe pain. Seven of the patients with calf lesions had limited ankle dorsiflexion. On imaging, the complex intramuscular lesions replaced muscle fibers with fibrofatty overgrowth and phlebectasia (dilation of the veins). The extrafascial component comprised fatty overgrowth, phlebectasia, and an occasional lymphatic malformation. The histopathologic features comprised dense fibrous tissue, fat, and lymphoplasmacytic aggregates within atrophied skeletal muscle. Adipose tissue also infiltrated skeletal muscle at the periphery of the lesion. There were large, irregular, and sometimes excessively muscularized venous channels and smaller, clustered channels. Other findings include organizing thrombi, a lymphatic component, and dense fibrous tissue–encircled nerves. Conclusions: The constellation of clinical, radiologic, and histopathologic features constitutes a distinct entity comprising fibrofatty infiltration of muscle, unusual phlebectasia with pain, and contracture of the affected extremity. The clinical and radiologic findings permit the diagnosis of FAVA with major therapeutic implications. Level of Evidence: Level III.

Sclerotherapy of Abdominal Lymphatic Malformations with Doxycycline

PURPOSE To assess the safety and efficacy of percutaneous image-guided sclerotherapy with doxycycline as primary treatment of intraabdominal lymphatic malformations (LMs). MATERIALS AND METHODS Retrospective review was performed of all cases of abdominal, mesenteric, or retroperitoneal LMs referred to a single center that were subsequently treated with image-guided percutaneous sclerotherapy. RESULTS Ten patients were included, of whom six were male. The mean age was 13 years (range, 2-28 y). Preprocedural cross-sectional imaging demonstrated a macrocystic malformation in nine patients and a mixed macrocystic/microcystic malformation in one. The malformation was accessed under sonographic guidance, followed by injection of opacified sclerosant agent under fluoroscopic guidance. A drainage catheter was placed in eight cases, in which sclerotherapy was repeated through the catheter for another 1 day (n = 2) or 2 days (n = 6). Doxycycline was reconstituted at 10 mg/mL, with a mean per-session dose of 608 mg (range, 80-1,000 mg) and a mean total dose of 1,230 mg (range, 80-3,000 mg). Peritoneal spill was identified in one case, but the patient remained asymptomatic. No other complications were encountered. Follow-up imaging was available in eight patients: complete resolution was seen in seven, with partial resolution in one. There was no recurrence of clinical symptoms in the follow-up period. CONCLUSIONS Initial results indicate that percutaneous image-guided sclerotherapy of macrocystic intraabdominal LMs with doxycycline is a safe and effective procedure.

Sonography-guided percutaneous liver biopsies in children.

OBJECTIVE The purpose of this study was to evaluate the safety and efficacy of sonography-guided percutaneous core needle liver biopsy in infants and children. MATERIALS AND METHODS We conducted a retrospective analysis of all patients who underwent sonography-guided percutaneous core needle liver biopsies over a 7.5-year period by pediatric interventionalists at a single tertiary center. RESULTS A total of 597 procedures were performed in 470 patients (270 male and 200 female), with a mean age of 10.5 years (age range, 1 month-21 years). The main indications for biopsies were abnormal liver enzymes (n=129, 21.6%), grading and staging of chronic hepatitis B or C (n=105, 17.6%), evaluation of transplanted liver (n=111, 18.6%), iron overload (n=73, 12.2%), miscellaneous other diffuse parenchymal abnormalities (n=124, 20.7%), and focal hepatic lesions (n=55, 9.2%). The procedures were performed either under sedation (n=311, 52.1%) or general anesthesia (n=286, 47.9%). Diagnostic yield was obtained in 596 biopsies (99.8%) from an average of 2.4 cores in patients with diffuse disease (n=541, 90.6%) and 6.5 cores in patients with focal disease (n=55, 9.2%). Ten patients (1.7%) experienced a major complication, including pneumothorax (n=1, 0.2%), abdominal wall pseudoaneurysm (n=1, 0.2%), and symptomatic bleeding (n=8, 1.3%). Five of these children required transfusion, two were only admitted for observation, and one required surgical evacuation. There were no procedure-related deaths. Minor complications (n=49, 8.2%) included a symptomatic subcapsular hematoma (n=35) and stable small hemoperitoneum (n=9). CONCLUSION Sonography-guided percutaneous core liver biopsy is a safe and effective procedure in children that has a high diagnostic yield and very low complication rate.

Diffuse lipofibromatosis of the lower extremity masquerading as a vascular anomaly.

Lipofibromatosis is a slow-growing, childhood soft-tissue neoplasm that is often confused with other conditions. We report a patient with lipofibromatosis causing extremity enlargement at birth. The lesion initially was thought to be a vascular anomaly or lipedema on clinical and MRI examination. When involving the lower extremity, diffuse lipofibromatosis must be differentiated from more common causes of lower limb enlargement in children: lymphatic malformation, lymphedema, or lipedema. Compared with these more frequent conditions, lipofibromatosis usually causes less morbidity. Management of the tumor includes observation or excision. Because complete extirpation of the lesion is difficult, the recurrence rate is high.

Radiologist-Supervised Ketamine Sedation for Solid Organ Biopsies in Children and Adolescents

OBJECTIVE Pediatric interventional radiologists are frequently challenged when faced with organ biopsies. Because of the need for patient immobility and the potential risk of morbidity with patient movement during biopsies, many radiologists prefer general anesthesia to sedation. We present our experience with radiologist-supervised ketamine sedation in pediatric patients undergoing renal and hepatic biopsies. MATERIALS AND METHODS Quality assurance data were accessed from a computerized database that prospectively collects demographics, outcome parameters, and adverse events on all patients who receive ketamine sedation. Patients received an IV ketamine bolus of 2 mg/kg followed by a continuous infusion of ketamine of up to 150 mcg/kg/min titrated to the responsiveness of the patient. RESULTS Sixty-five children received ketamine for liver (n = 35) and renal (n = 30) biopsies. The mean age of the study group was 7.0 +/- 2.7 (SD) years. The cohort included patients with an American Society of Anesthesiologists (ASA) physical status classification of ASA 1 (3%), ASA 2 (78%), and ASA 3 (19%). The duration of ketamine sedation averaged 39 +/- 20 (SD) minutes, with an average procedure time of 32 +/- 19 (SD) minutes. All procedures were successfully completed, and there were no major adverse events. CONCLUSION Interventional radiologists performing solid organ biopsies in the pediatric population often use general anesthesia to ensure immobility, adequate analgesia, and safe conditions. Our experience suggests that interventional radiologists may supervise a nurse-administered ketamine protocol to provide safe, effective analgesia and sedation for liver and renal biopsies.

Transcranial Doppler analysis of cerebral hemodynamics in primary craniosynostosis: Study in progress

BACKGROUND Regional elevations in intracranial pressure (ICP) have been monitored adjacent to areas of cranial stenosis by some authors [9,19,20]; however, noninvasive techniques such as transcranial Doppler (TCD) would be preferable if increased ICP could be detected just as reliably. METHODS TCD examinations of basal cerebral arteries were performed in 31 children with skull deformities. Skull X rays and computed tomography (CT) scans or magnetic resonance imaging (MRI) were analyzed for degree of cranial vault abnormality, as well as for presence or absence of intracranial pathology. Neurodevelopmental examination and clinical signs of increased ICP, if any, were recorded. RESULTS The overall incidence of increased resistivity indices (RI) was 70%. Patients with asymmetric skull deformities (plagiocephaly) or sagittal synostosis (scaphocephaly) were statistically more prone to exhibit increased RI compared to previously reported prevalences. CONCLUSIONS Because TCD is a convenient, rapid, accurate, and noninvasive method by which to gauge ICP, it should be considered in the preliminary work-up of a patient with craniosynostosis.

Noninvasive assessment of testicular torsion in rabbits using frequency-domain near-infrared spectroscopy: prospects for pediatric urology

We present a quantitative near-IR spectroscopy study of the absolute values of oxygen saturation of hemoglobin before and after surgically induced testicular torsion in adult rabbits. Unilateral testicular torsions (0, 540, or 720 deg) on experimental testes and contralateral sham surgery on control testes are performed in four adult rabbits. A specially designed optical probe for measurements at multiple source-detector distances and a commercial frequency-domain tissue spectrometer are used to measure absolute values of testicular hemoglobin saturation. Our results show: (1) a consistent baseline absolute tissue hemoglobin saturation value of 78+/-5%, (2) a comparable tissue hemoglobin saturation of 77+/-6% after sham surgery, and (3) a significantly lower tissue hemoglobin saturation of 36+/-2% after 540- and 720-deg testicular torsion surgery. Our findings demonstrate the feasibility of performing frequency-domain, multidistance near-IR spectroscopy for absolute testicular oximetry in the assessment of testicular torsion. We conclude that near-IR spectroscopy has potential to serve as a clinical diagnostic and monitoring tool for the assessment of absolute testicular hemoglobin desaturation caused by torsion, with the possibility of serving as a complement to conventional color and spectral Doppler ultrasonography.