Hand ischemia associated with elbow trauma in children

ObjectivesPrevious observational studies suggest that children with hand ischemia following elbow trauma can be safely observed if Doppler signals are present in the wrist arteries (pink pulseless hand, PPH). Nonoperative management of PPH is predicated on the assumption that PPH results from local arterial spasm, but the mechanism of arterial compromise has not been investigated. We hypothesized that PPH signifies a brachial artery injury that requires surgical repair.MethodsRetrospective review of operations performed on children with hand ischemia following elbow trauma at a level I trauma center pediatric hospital.ResultsBetween 2003 and 2010, 12 children (seven males, mean age 7.4 years) underwent brachial artery exploration for hand ischemia following elbow trauma (11 supracondylar fractures, one elbow dislocation) due to falls (n = 10) or motor vehicle crashes (n = 2). At presentation, three subjects had normal radial pulses, eight subjects had Doppler signals but no palpable pulses, and one had weak Doppler flow with advanced hand ischemia. Six of the nine subjects without palpable pulses also had neurosensory changes. All 12 subjects underwent brachial artery exploration either initially (n = 2) or following orthopedic fixation (n = 10) due to persistent pulselessness. At operation, eight of 12 patients (67%) had focal brachial artery thrombosis due to intimal flaps, and four had brachial artery and median nerve entrapment within the pinned fracture site. At discharge, all 12 subjects had palpable radial pulses, but three with entrapment had dense median nerve deficits. One of the three subjects with dense neurologic deficit had complete recovery of neurologic function at ten months. The other two subjects had residual median nerve deficits with partial recovery at 5 and 6 months follow-up, respectively. No patient developed Volkman's contracture.ConclusionsBrachial artery injuries should be anticipated in children with hand ischemia associated with elbow trauma. Neurovascular entrapment at the fracture site is a possible complication of orthopedic fixation. Absence of palpable wrist pulses after orthopedic fixation should prompt immediate brachial artery exploration. PPH should not be considered a consequence of arterial spasm in these patients

FAK Inhibition Decreases Hepatoblastoma Survival Both In Vitro and In Vivo

AbstractHepatoblastoma is the most frequently diagnosed liver tumor of childhood, and children with advanced, metastatic or relapsed disease have a disease-free survival rate under 50%. Focal adhesion kinase (FAK) is a nonreceptor tyrosine kinase that is important in many facets of tumor development and progression. FAK has been found in other pediatric solid tumors and in adult hepatocellular carcinoma, leading us to hypothesize that FAK would be present in hepatoblastoma and would impact its cellular survival. In the current study, we showed that FAK was present and phosphorylated in human hepatoblastoma tumor specimens. We also examined the effects of FAK inhibition upon hepatoblastoma cells using a number of parallel approaches to block FAK including RNAi and small molecule FAK inhibitors. FAK inhibition resulted in decreased cellular survival, invasion, and migration and increased apoptosis. Further, small molecule inhibition of FAK led to decreased tumor growth in a nude mouse xenograft model of hepatoblastoma. The findings from this study will help to further our understanding of the regulation of hepatoblastoma tumorigenesis and may provide desperately needed novel therapeutic strategies and targets for aggressive, recurrent, or metastatic hepatoblastomas

Juvenile xanthogranuloma: diverse presentations of noncutaneous disease

Juvenile xanthogranulomas (JXGs) are benign cutaneous lesions of childhood that often spontaneously involute. They rarely present as a noncutaneous tumors. However, JXG tumors have been described in numerous noncutaneous anatomic sites, presenting with a variety of symptoms. The severity of symptoms and accurate preoperative diagnosis of JXG should determine operative and nonoperative treatment options of these uncommon, benign, and self-limiting tumors. We report 3 cases of symptomatic, noncutaneous JXG from disparate anatomic sites all treated with aggressive surgical resection