Differences between the pediatric and adult presentation of fibromuscular dysplasia: results from the US Registry.

BACKGROUND: Fibromuscular dysplasia (FMD) is a non-inflammatory arteriopathy that causes significant morbidity in children. METHODS: The clinical features, presenting symptoms, and vascular beds involved are reviewed in the first 33 patients aged <18 years who are enrolled in the United States Registry for FMD from five registry sites and compared with 999 adult patients from 12 registry sites. RESULTS: Mean age at diagnosis was 8.4 ± 4.8 years (16 days to 17 years). Compared with adults, pediatric FMD occurs in more males (42.4 vs 6 %, p < 0.001). Children with FMD have a stronger previous history of hypertension (93.9 vs 69.9 %, p = 0.002). Hypertension (100 %), headache (55 %), and abdominal bruits (10.7 %) were the most common presenting signs and symptoms. FMD affects renal vasculature in almost all children (97 vs 69.7 %, p = 0.003). The extra-cranial carotid vessels are less commonly involved in children (23.1 vs 73.3 %, p < 0.001). The mesenteric arteries (38.9 vs 16.2 %, p = 0.02) and aorta (26.3 vs 2.4 %, p < 0.001) are more commonly involved in children. CONCLUSIONS: In the United States Registry for FMD, pediatric FMD affects children from infancy throughout childhood. All children presented with hypertension and many presented with headache and abdominal bruits. In children, FMD most commonly affects the renal vasculature, but also frequently involves the mesenteric arteries and abdominal aorta; the carotid vessels are less frequently involved.

Quality improvement guidelines for pediatric abscess and fluid drainage.

Image-guided drainage of abscesses and fluid collections is a valuable tool in the treatment of pediatric patients. It may obviate surgery or optimize the child's clinical condition for subsequent surgery. Compared with adults, several differences exist in terms of etiology, risks (especially radiation exposure), preprocedural imaging and planning, technical considerations, support issues such as sedation, and complications. Knowledge of these differences is important in the planning and treatment of these patients. In addition, a quality improvement plan can be used to assess practice performance.

Multiple ruptured cerebral aneurysms in a child with Takayasu arteritis.

The authors report the case of an 18-month-old girl who presented with a ruptured anterior communicating artery aneurysm, and who was later diagnosed with Takayasu arteritis. Her initial aneurysm was successfully treated with clip application. However, over a 6-month period she had multiple ruptures from new and rapidly recurring aneurysms adjacent to the clips. These aneurysms were treated with repeated craniotomy and clip application and then with endovascular coil placement. Aneurysmal subarachnoid hemorrhage is a rare presentation of Takayasu arteritis. To the authors' knowledge, this is the youngest reported patient with Takayasu arteritis to present with a ruptured cerebral aneurysm.

Interventional radiology: a modular approach.

The authors offer a modular approach to the development of new procedures in the field of pediatric interventional radiology as a conceptual model and a springboard for further discussion.

Pseudoaneurysm in children: diagnosis and interventional management.

Pseudoaneurysms (PAn) are uncommon in adults and even less common in children. They are most often encountered after iatrogenic arterial injury. Presentation may be substantially delayed after the iatrogenic event, and diagnosis can be difficult, especially when the PAn occurs in an unexpected location. Treatment of PAn has evolved during the last two decades from a reliance on surgical resection to US-guided compression, coil embolization, covered stents, and stent-graft exclusion. More recently, direct percutaneous US-guided thrombin injection has been used in the treatment of PAn. We present three cases of successful PAn thrombosis by US-guided percutaneous thrombin injection in children, one of the epigastric artery and two of the femoral artery.

Utility of corticosteroid injection for temporomandibular arthritis in children with juvenile idiopathic arthritis.

OBJECTIVE: To assess the effects of computed tomography (CT)-guided injection of corticosteroid into the temporomandibular joint (TMJ) in children with juvenile idiopathic arthritis (JIA) and clinical and magnetic resonance imaging (MRI) evidence of TMJ inflammation. METHODS: Twenty-three children ages 4-16 years with JIA and MRI evidence of TMJ inflammation received CT-guided TMJ injections of corticosteroid (triamcinolone acetonide [n = 16] or triamcinolone hexacetonide [n = 7]). Jaw pain or dysfunction and maximal incisal opening (MIO) distance were assessed before and after injection. Fourteen patients had followup MRI studies of the TMJ 6-12 months after injection. RESULTS: Of the 13 patients with symptoms of jaw pain prior to corticosteroid treatment, 10 (77%) had complete resolution of pain (P < 0.05). Prior to corticosteroid injection, MIO in all 23 patients was below age-matched normal values. After injection, the MIO was improved by at least 0.5 cm in 10 patients (43%) (P = 0.0017). Patients under 6 years of age at the time of injection showed the best response, with a postinjection MIO similar to that in age-matched controls (P = 0.2267). There was involvement of 23 TMJs in the 14 patients who had followup MRI studies; resolution of effusions was observed in 11 (48%) of the TMJs. Other than short-term facial swelling in 2 patients, there were no side effects. CONCLUSION: The majority of children with symptomatic TMJ arthritis improved after intraarticular corticosteroid injection. Approximately half the patients experienced significant improvement in MIO and TMJ effusion. These data suggest that corticosteroid injection may be a useful procedure for the prevention and treatment of morbidities associated with TMJ arthritis in JIA.

Closed reduction with CT-guided screw fixation for unstable sacroiliac joint fracture-dislocation.

BACKGROUND: Unstable posterior pelvic ring fractures and dislocations are uncommon but potentially life-threatening injuries in children. Early definitive management reduces risk of immediate complications as well as chronic pain and gait dysfunction. Conventional operative therapy carries substantial risk of extensive blood loss and iatrogenic neurological and vascular injury. Minimally invasive image-guided intervention may further reduce immediate risk and improve long-term outcome. OBJECTIVE: To describe CT-guided closed reduction and internal fixation (CRIF) and review outcomes of unstable fracture-dislocation of the sacroiliac (SI) joint in children. MATERIALS AND METHODS: Between 2000 and 2003, three children (two girls, one boy) age 8-14 years were referred to interventional radiology for treatment of unstable SI joint fracture-dislocation not adequately treated with anterior external fixation alone. RESULTS: The three affected SI joints (two left, one right) were treated in a combined approach by pediatric interventional radiologists and orthopedic surgeons, using a percutaneous approach under CT guidance. Over a threaded guiding pin, 7.3 mm cannulated screws were used to achieve stable reduction of the affected SI joints. One screw was removed after slight (2 mm) migration. No neurovascular or other complications occurred. All patients had satisfactory healing with near-anatomic reduction, although recovery of the youngest was delayed by associated spinal injury. CONCLUSIONS: Compared to open surgical alternatives, CRIF under CT guidance reduces operating time, decreases blood loss, and allows early definitive fixation and immediate non-weight-bearing mobilization with a low rate of complication for unstable posterior pelvic ring fractures. In addition, CT-guided placement of the guide pin may allow safer screw positioning and may minimize the total number of screws needed to achieve pelvic stability.