Epstein-Barr virus–associated smooth-muscle tumors are rare tumors seen in immunocompromised patients. Most cases occur in the context of AIDS and organ transplantation, and very rarely in the setting of congenital immunodeficiency, with only 5 case reports of the latter published so far in the literature. The authors report the case of a previously healthy 8-year-old girl with headaches and precocious puberty who was found to have a large skull base lesion. There was a synchronous left adrenal lesion. She underwent resection of the skull base lesion and a left adrenalectomy. Thorough evaluation for immunodeficiency was negative for a known congenital immunodeficiency syndrome. She had a short course of intravenous immunoglobulin and has had no recurrence of disease or new lesions in the 17 months since presentation. Continued surveillance for the development of opportunistic infections and new or recurrent lesions is warranted in this case. Repeat surgery for surgically accessible tumors or chemoradiation would be recommended for any additional lesions.
Desmond A. Brown, Nicholas L. Deep, Colin L. Driscoll, Michael J. Link, Mark E. Jentoft and David J. Daniels
James M. Johnston, David D. Limbrick Jr., Wilson Z. Ray, Stephanie Brown, Joshua Shimony and Tae Sung Park
Rosai-Dorfman disease (RDD) is an idiopathic histioproliferative disorder that rarely involves the CNS. Rosai-Dorfman disease is exceedingly rare in the pediatric population and has never been observed in the cerebellum of a child. The authors present the case of a 14-year-old male with a cerebellar lesion having radiographic characteristics of Lhermitte-Duclos disease. After a period of observation with a presumptive diagnosis of Lhermitte-Duclos disease, the child underwent suboccipital craniotomy and resection of the lesion due to continuous suboccipital headaches. Histological examination of the tissue demonstrated RDD. The published literature on RDD is reviewed with an emphasis on differential diagnosis.
Martina Stippler, Veronica Ortiz, P. David Adelson, Yue-Fang Chang, Elizabeth C. Tyler-Kabara, Stephen R. Wisniewski, Ericka L. Fink, Patrick M. Kochanek, S. Danielle Brown and Michael J. Bell
Minimizing secondary brain injuries after traumatic brain injury (TBI) in children is critical to maximizing neurological outcome. Brain tissue oxygenation monitoring (as measured by interstitial partial pressure of O2 [PbO2]) is a new tool that may aid in guiding therapies, yet experience in children is limited. This study aims to describe the authors' experience of PbO2 monitoring after TBI. It was hypothesized that PbO2 thresholds could be established that were associated with favorable neurological outcome, and it was determined whether any relationships between PbO2 and other important clinical variables existed.
Forty-six children with severe TBI (Glasgow Coma Scale score ≤ 8 after resuscitation) who underwent PbO2 and brain temperature monitoring between September 2004 and June 2008 were studied. All patients received standard neurocritical care, and 24 were concurrently enrolled in a trial of therapeutic early hypothermia (n = 12/group). The PbO2 was measured in the uninjured frontal cortex. Hourly recordings and calculated daily means of various variables including PbO2, intracranial pressure (ICP), cerebral perfusion pressure (CPP), mean arterial blood pressure, partial pressure of arterial O2, and fraction of inspired O2 were compared using several statistical approaches. Glasgow Outcome Scale scores were determined at 6 months after injury.
The mean patient age was 9.4 years (range 0.1–16.5 years; 13 girls) and 8554 hours of monitoring were analyzed (PbO2 range 0.0–97.2 mm Hg). A PbO2 of 30 mm Hg was associated with the highest sensitivity/specificity for favorable neurological outcome at 6 months after TBI, yet CPP was the only factor that was independently associated with favorable outcome. Surprisingly, instances of preserved PbO2 with altered ICP and CPP were observed in some children with unfavorable outcomes.
Monitoring of PbO2 demonstrated complex interactions with clinical variables reflecting intracranial dynamics using this protocol. A higher threshold than reported in studies in adults was suggested as a potential therapeutic target, but this threshold was not associated with improved outcomes. Additional studies to assess the utility of PbO2 monitoring after TBI in children are needed.
Jacob K. Greenberg, Donna B. Jeffe, Christopher R. Carpenter, Yan Yan, Jose A. Pineda, Angela Lumba-Brown, Martin S. Keller, Daniel Berger, Robert J. Bollo, Vijay M. Ravindra, Robert P. Naftel, Michael C. Dewan, Manish N. Shah, Erin C. Burns, Brent R. O’Neill, Todd C. Hankinson, William E. Whitehead, P. David Adelson, Mandeep S. Tamber, Patrick J. McDonald, Edward S. Ahn, William Titsworth, Alina N. West, Ross C. Brownson and David D. Limbrick Jr.
There remains uncertainty regarding the appropriate level of care and need for repeating neuroimaging among children with mild traumatic brain injury (mTBI) complicated by intracranial injury (ICI). This study’s objective was to investigate physician practice patterns and decision-making processes for these patients in order to identify knowledge gaps and highlight avenues for future investigation.
The authors surveyed residents, fellows, and attending physicians from the following pediatric specialties: emergency medicine; general surgery; neurosurgery; and critical care. Participants came from 10 institutions in the United States and an email list maintained by the Canadian Neurosurgical Society. The survey asked respondents to indicate management preferences for and experiences with children with mTBI complicated by ICI, focusing on an exemplar clinical vignette of a 7-year-old girl with a Glasgow Coma Scale score of 15 and a 5-mm subdural hematoma without midline shift after a fall down stairs.
The response rate was 52% (n = 536). Overall, 326 (61%) respondents indicated they would recommend ICU admission for the child in the vignette. However, only 62 (12%) agreed/strongly agreed that this child was at high risk of neurological decline. Half of respondents (45%; n = 243) indicated they would order a planned follow-up CT (29%; n = 155) or MRI scan (19%; n = 102), though only 64 (12%) agreed/strongly agreed that repeat neuroimaging would influence their management. Common factors that increased the likelihood of ICU admission included presence of a focal neurological deficit (95%; n = 508 endorsed), midline shift (90%; n = 480) or an epidural hematoma (88%; n = 471). However, 42% (n = 225) indicated they would admit all children with mTBI and ICI to the ICU. Notably, 27% (n = 143) of respondents indicated they had seen one or more children with mTBI and intracranial hemorrhage demonstrate a rapid neurological decline when admitted to a general ward in the last year, and 13% (n = 71) had witnessed this outcome at least twice in the past year.
Many physicians endorse ICU admission and repeat neuroimaging for pediatric mTBI with ICI, despite uncertainty regarding the clinical utility of those decisions. These results, combined with evidence that existing practice may provide insufficient monitoring to some high-risk children, emphasize the need for validated decision tools to aid the management of these patients.