Image-Guided Transrectal Drainage of Pelvic Abscesses in Children.

OBJECTIVE: To describe our technique and share our experience with image-guided transrectal drainage (TRD) of pelvic abscesses in children. MATERIALS AND METHODS: Retrospective review and analysis of indications for image-guided TRD and examination of procedural outcomes in pediatric patients with pelvic abscesses over 8 years. RESULTS: A total of 69 patients (33 males and 36 females) with symptomatic pelvic abscesses underwent image-guided TRD. The median age and weight of the patients were 11.5 years (range, 3-18) and 46.8 kg (range, 15.1-118.0), respectively. The etiologies of the pelvic abscesses were perforated appendicitis (72.5%) and post-operative collections (27.5%). All patients presented with abdominal pain. Fever, emesis, and diarrhea were also common symptoms. The size of the pelvic abscesses ranged from 24.0 to 937.1 mL (median, 132.7). Technical success was achieved in 68 of 69 TRD procedures (98.6%). Clinical improvement was observed in all patients with technically successful TRD. The TRD catheter dwell time ranged from 0 to 10 days (median, 4.0). Most patients who underwent TRD for perforated appendicitis subsequently underwent elective appendectomy after the resolution of the pelvic abscess (84.0%). The median time from TRD to elective appendectomy was 2.8 months (range, 0.3-6.1). There were no procedure-related complications. CONCLUSION: Image-guided TRD is a safe and effective procedure with high technical and clinical success rates for the treatment of pelvic abscesses in children.

Age-specific Dose Catalog for Diagnostic Fluoroscopy and Fluoroscopically Guided Interventional Procedures from a Pediatric Hospital.

Background Fluoroscopy is an imaging modality associated with a wide range of dose levels, characterized using a variety of dose metrics, including effective dose. However, for clinical procedures, effective dose is a seldom-used and unregulated metric in the United States, and thus, it is not extensively studied in radiology despite potentially large clinical implications for patients, especially children and infants. Purpose To formulate and report a dose catalog across all diagnostic and interventional radiology (IR) fluoroscopy examination or procedure types at a specialized tertiary care pediatric hospital. Materials and Methods In this retrospective study, dose metrics taken from radiation dose structured reports of fluoroscopy between October 2014 and March 2023 were analyzed. The reports included fluoroscopy across 18 diagnostic examination types and 24 IR procedure types. The National Cancer Institute dosimetry system for Radiography and Fluoroscopy Monte Carlo software was used to estimate age-specific effective dose from dose-area product (DAP). The DAP-to-effective dose conversion factors were estimated per IR procedure type and diagnostic fluoroscopy examination type based on age. Results A total of 11 536 individual diagnostic fluoroscopy examinations (18 types) and 8017 individual IR procedures (24 types) were analyzed. Median effective dose values per diagnostic fluoroscopy examination type ranged from 0.0010 to 0.44 mSv (mean, 0.0808 mSv ± 0.0998 [SD]). Calculated DAP-to-effective dose conversion factors ranged from 0.04 to 2.48 mSv/Gy · cm2 (mean, 0.758 mSv/Gy · cm2 ± 0.614) across all diagnostic fluoroscopy examination types. Median effective dose values per IR procedure type ranged from 0.0007 to 3.90 mSv (mean, 0.6757 mSv ± 0.8989). Calculated DAP-to-effective dose conversion factors ranged from 0.001 to 0.87 mSv/Gy · cm2 (mean, 0.210 mSv/Gy · cm2 ± 0.235) across all IR procedure types. Conclusion A pediatric fluoroscopy dose catalog was created, including age-specific effective dose, using a repeatable robust method based on accurate clinical data. © RSNA, 2024 Supplemental material is available for this article. See also the editorial by Borrego and Balter in this issue.

Intrathecal catheter and port placement for nusinersen infusion in children with spinal muscular atrophy and spinal fusion.

BACKGROUND: Spinal administration of medications is challenging in patients with complete posterior spinal fusion. We describe percutaneous image-guided intrathecal port placement for administration of the antisense oligonucleotide nusinersen for children and young adults with spinal muscular atrophy. OBJECTIVE: To describe and present our initial experience with a new technique for administering nusinersen in patients with spinal muscular atrophy and posterior spinal fusion. MATERIALS AND METHODS: We reviewed medical records of 13 patients who received intrathecal ports using DynaCT, biplane fluoroscopy and iGuide from April 2018 through June 2019, and we describe the clinical course over 1 year. RESULTS: Image-guided catheter and port implantation was successful in all cases. Two ports were subsequently removed, one for persistent cerebrospinal fluid leak and one for superficial infection. The other 11 have functioned successfully for a minimum of 23 months. CONCLUSION: We report our experience with image-guided intrathecal port placement in children with complete posterior spine fusion. The implanted port permits dosing in an outpatient setting and avoids the need for multiple future radiologic procedures, and it reduces discomfort, procedural costs and potential risks and sequelae of multiple anesthetics and radiation exposures. Further studies are needed to define the relative risks and benefits of intrathecal ports compared to other approaches such as repeated transforaminal lumbar punctures.

Contrast enhanced colostography: New applications in preoperative evaluation of anorectal malformations.

INTRODUCTION: Understanding details of anatomic relationships between the colon and surrounding structures is a critical piece of preoperative planning prior to surgical repair of anorectal malformations (ARMs). Traditional imaging techniques involve ionizing radiation, distention of the rectum with supraphysiologic intraluminal pressures, and sometimes require sedation. Recent developments in the field of contrast agents have allowed the emergence of an ultrasound-based technique that can avoid these requirements while continuing to provide high resolution structural information in three dimensions. METHODS: Fourteen children (13 male, 1 female, age 1-11 months) with ARMs underwent contrast enhanced colostography (ceCS) in addition to traditional preoperative imaging techniques to delineate anatomic relationships of pelvic structures. RESULTS: ceCS and traditional imaging yielded concordant anatomic information, including structural relationships and fistulous connections, in 10/14 patients (71%). ceCS detected fistulous connection in 2/13 patients (15%) that were not seen by traditional imaging. Ultrasonography failed to detect the fistulous connection in one patient. CONCLUSIONS: ceCS is a safe, effective and flexible method for defining important structural information in ARM patients. When compared with traditional methods, it provided equivalent or superior results 93% of the time and bears consideration as a standard tool in preoperative planning for this population. TYPE OF STUDY: Retrospective Comparative Study. LEVEL OF EVIDENCE: Level III.

Case series: Comparison of contrast-enhanced genitosonography (ceGS) to fluoroscopy and cone-beam computed tomography in patients with urogenital sinus and the cloacal malformation.

In this case series, contrast enhanced genitosonography is compared to genitography performed using fluoroscopy and cone-beam computed tomography in patients with urogenital sinus and the cloacal malformation. The method of contrast enhanced genitosonography is described, including contrast preparation, contrast administration, ultrasound imaging approaches, as well as the benefits and potential pitfalls of this technique compared to fluoroscopy and computed tomography.

Contrast-Enhanced Colosonography for the Evaluation of Children With an Imperforate Anus.

This case series describes a novel method for showing the preoperative anatomy of children with anorectal malformations using ultrasound contrast, which we have termed "contrast-enhanced colosonography (ceCS)." Six patients with anorectal malformations without a perineal fistula were studied both by fluoroscopic distal colostography and ceCS, and their results were confirmed surgically. Contrast-enhanced CS precisely showed the complex anatomic relationships in all cases. Compared to traditional fluoroscopic studies, ceCS has the benefit of no associated ionizing radiation and thus is safer for children.

Endovascular Treatment of Congenital Arterioportal Fistulas.

PURPOSE: To characterize anatomy of congenital arterioportal fistulas (CAPF) and correlate this with technique and outcomes of transcatheter embolization (TCE). MATERIALS AND METHODS: Retrospective review was conducted of children with CAPF that underwent TCE in a 10-year period. Medical records, imaging and procedure details were reviewed. TCE was performed via transarterial (n = 5), portal (n = 5) or patent ductus venosus (n = 1) approach. Embolic agents used were coils (n = 10), Onyx (n = 1) and Amplatzer septal occluder (n = 1). RESULTS: A total of 7 patients were included (4 female). Median age at treatment was 4 months (2 month-3 year). Most common symptoms were GI bleeding (n = 4), anemia (n = 4) and ascites (n = 3). Hepatopetal flow was seen in all on pre-procedure sonography. On angiography, a simple lesion, with direct fistulous connections, from hepatic arterial (HA) branches to portal venous (PV) system was seen in 3. A complex lesion with multiple connections was identified in 4. In simple lesions, cessation of shunting was achieved in 1 procedure, with embolization of afferent vessels. In complex lesions, multiple procedures were required in 3 of 4 patients. All 4 with complex connections required embolization of the aneurysmal PV segment. There were no major complications. Two minor complications were noted: localized biliary leak in 1 and femoral pseudoaneurysm in 1. There was resolution of symptoms in 6. CONCLUSIONS: TCE is effective as primary treatment of CAPF. Patients with simple arteriovenous connections can be treated with embolization of afferent vessels, but children with multiple complex connections usually require embolization of the dilated PV segment.

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.

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.

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.

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.

Metastatic neuroblastoma mimicking infantile hemangioma.

Many lesions can masquerade as deep infantile hemangioma, the most common tumor of infancy. We describe an infant with infantile hemangioma and concomitant metastatic subcutaneous neuroblastoma mimicking deep hemangioma. The patient presented at 8 months of age with 3 superficial infantile hemangiomas as well as 3 subcutaneous masses. History, physical examination, and ultrasonography of the deep lesions were consistent with subcutaneous hemangioma. New masses appeared at 10 months of age that prompted biopsy; histopathology demonstrated metastatic neuroblastoma. Deviation from the predictable clinical features of a deep infantile hemangioma should prompt consideration for other causes of a subcutaneous mass in infancy, including metastatic neuroblastoma.

Infantile myofibroma or lymphatic malformation: differential diagnosis of neonatal cystic cervicofacial lesions.

A 5-day-old male neonate was referred to our vascular anomalies center with a large cystic submandibular mass. History and physical examination and ultrasonographic results indicated the diagnosis to be macrocystic lymphatic malformation. Consequently, the child was treated with sclerotherapy and subtotal excision. The histopathological examination result showed that the lesion was infantile myofibroma. This diagnostic error was attributed to atypical features of infantile myofibroma in this child: unusually large cysts, rapid enlargement, and coagulopathy. This report expands the clinical spectrum of infantile myofibromatosis and suggests its consideration in the differential diagnosis of neonatal cystic cervicofacial lesions.

Renal pseudoaneurysm following laser lithotripsy: endovascular treatment of a rare complication.

Hemorrhagic complications of renal transplantation can be life threatening and require prompt and timely intervention. This brief report describes the exceedingly rare formation of an extrarenal pseudoaneurysm of a transplant renal artery following laser lithotripsy for nephrolithiasis in a teenage male. The pseudoaneurysm ruptured into the renal collecting system. Transarterial coil embolization of the wide-neck saccular pseudoaneurysm of the extraparenchymal renal artery with the assistance of a 'safety microguidewire' was successfully performed and the allograft was preserved.

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.

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 Children's 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.

The use of inferior vena cava filters in young children.

The present report describes the authors' experience with the placement and retrieval of inferior vena cava (IVC) filters in children with a maximum IVC diameter of 1 cm. Over a 14-month period, three filters were placed in three children. Two filters were placed via the right internal jugular vein, with one placed via the right femoral vein. The filters were deployed successfully in all three children and retrieved in two. In one case, the child was receiving palliative care and removal was not attempted. No complications were encountered during placement, the period of implantation, or retrieval.

Percutaneous sclerotherapy for lymphatic malformations: a retrospective analysis of patient-evaluated improvement.

PURPOSE: To evaluate the midterm outcomes of percutaneous sclerotherapy of lymphatic malformations (LMs) as judged by patients. MATERIALS AND METHODS: A 13-item survey questionnaire was sent to 74 patients who had undergone at least one sclerotherapy procedure in our hospital from January 1997 through January 2003. Information regarding the anatomic location, specific symptoms reported, history, treatment satisfaction, postprocedural complications, and number of treatment sessions was elicited. Four sclerosing agents (as single agents or in combination with other agents) were used: ethanol, sodium tetradecyl sulfate 3% (STS), OK-432, and doxycycline. RESULTS: Fifty-five patients or their caregivers completed the survey. The patients' ages ranged from 6 months to 48 years at the time of the first procedure (mean, 12 y; median, 4 y). A majority of LMs were located in the cervicofacial region. The size and location of the lesion, recurrent infection, and pain were the most frequent indications for treatment. Fifty-one percent of these patients received sclerotherapy alone or in conjunction with surgery as primary treatment. Ethanol was the most common sclerosing agent used, followed by doxycycline, STS, and OK-432. Response varied with the type of LM, with 100%, 86%, and 43% of the patients reporting good to complete response for macrocystic, microcystic, and combined-type LMs, respectively. Skin blistering and ulcers were the most common complications. Permanent complications were uncommon and were largely related to ethanol use. CONCLUSIONS: Percutaneous sclerotherapy provides effective midterm primary treatment for LMs. Treatment outcomes appear to vary according to the morphology of the malformation.