Survival following decompressive hemicraniectomy for hemiconvulsion-hemiplegia-epilepsy syndrome: case report.

Hemiconvulsion-hemiplegia-epilepsy (HHE) is an uncommon epileptic syndrome that affects young children. Typical management includes early initiation of benzodiazepines to abate the initial seizure activity quickly. Patients in whom epilepsy develops require prolonged use of antiepileptic agents. Herniation due to diffuse cerebral edema from HHE is rare; however, decompressive craniectomy has been described as a lifesaving measure. The authors present the case of a patient in whom a decompressive craniectomy was performed. They advocate a proactive approach in the detection and management of cerebral edema in HHE causing intracranial hypertension. In HHE cases that exhibit radiographic evidence of malignant cerebral edema (although not previously described in this disease, but similar to the setting of stroke and trauma), the authors advocate early neurosurgical consultation and evaluation for insertion of an intracranial pressure monitor for those patients who do not have a reliable neurological examination (i.e., Glasgow Coma Scale score ≤ 8).

Acute Precursor B-Cell Lymphoblastic Leukemia in a 1-Year-Old White Male: Diagnostic Evaluation and Flow Cytometric Analysis.

CLINICAL HISTORY PATIENT: 1-year-old white male. CHIEF COMPLAINT: Bruising, pallor, and decreased activity. HISTORY OF PRESENT ILLNESS: Bruising had first been noticed on both lower extremities 3 weeks before the day he arrived at the hospital seeking treatment. The patient experienced a fall at that time that resulted in a lip laceration, which resolved spontaneously without increased bleeding time. His activity level was normal until the day of hospital admission, when he was noticed to be somewhat lethargic and pale. He was brought to the Emergency Department after 2 episodes of epistaxis, which resolved spontaneously. MEDICAL HISTORY: Treated with amoxicillin the previous week for tonsillitis. FAMILY HISTORY: Mother has history of anemia. PHYSICAL EXAMINATION RESULTS: The patient appeared pale and tired, with dry and pale oral mucosa. Dried blood was noted on the nares bilaterally. The boy was noted to have extensive ecchymosis to the upper and lower extremities, chest, and back; he was also noted to have moderate petechiae with a similar distribution. No organomegaly or lymphadenopathy was present, but mild abdominal distension was noted.

Solid noninfectious growing masses over cerebrospinal fluid shunts: report of 3 cases.

The authors describe 3 children who presented with progressively enlarging skin-covered solid masses over the shunt catheter in the neck/clavicular region. The authors reviewed the clinical, laboratory, pathological, radiographic, and follow-up data for all 3 patients and reviewed the literature on the subject. The patients had no clinical evidence of an infectious process. Surgical exploration revealed that masses were surrounding and encasing the shunt tubing to which they were strongly attached. Pathological studies of the tissues demonstrated varying degrees of exuberant chronically inflamed granulation tissues, interstitial fibrosis, and dystrophic calcification. One patient had associated thinning of the skin overlying the mass and subsequently developed ulceration. No infectious organisms were observed. The cerebrospinal fluid aspirates from the shunts did not yield any organisms. There has been no recurrence of the masses. The presence of a growing mass over the shunt tube in the neck or the chest region without clinical evidence of infection does not indicate that the mass should be treated with antibiotics and complete shunt removal. Rather, the mass can be cured by extirpation and with "bypass" new shunt tubing locally.