CT angiographic imaging characteristics of thoracic idiopathic aortitis

BackgroundIdiopathic aortitis (IA) is characterized by giant cell or lymphoplasmacytic inflammation of aorta without a secondary cause.ObjectiveWe undertook a retrospective case–control study to identify characteristic CT angiographic findings in these patients and to correlate them with known atherosclerotic risk factors.MethodsIA cases and controls with noninflammatory aneurysm (control group I) and patients with secondary aortitis (control group II) were identified with a pathology database. Preoperative CT angiographic images of thoracic aorta were reviewed. Diameter of thoracic aorta, wall thickness, and calcification were measured at various sites. Traditional atherosclerotic risk factors were identified from case records and included hypertension, hyperlipidemia, diabetes mellitus, and smoking.ResultsTwenty-two idiopathic aortitis cases were compared with 18 patients in control group I and 16 patients in control group II. No differences were found in prevalence of hypertension and diabetes, but hyperlipidemia was more prevalent in the control group I than in cases (72% vs 36%; P = .03). Current smoking was more prevalent in cases (24%) than for patients in control group I (6%) and group II (19%) but not statistically significant (P = .18 and .69, respectively). Thoracic aortic diameters at various points were significantly larger in cases than for patients in control group I. Calcification was more frequent in cases than for patients in control group II. No differences in wall thickness were found. No meaningful correlation was observed between atherosclerotic risk factors and aortic diameter and calcification scores.ConclusionsPatients with IA have significantly larger and more diffuse dilatation of the thoracic aorta than patients with noninflammatory aneurysms.     

Syndrome of cerebrospinal fluid hypovolemia following lumbar puncture cerebrospinal fluid leak in a patient with idiopathic intracranial hypertension

An 11-year-old girl presented with headache of 3 months' duration. There was bilateral disc edema. The cerebrospinal fluid pressure was 50 cm of water with normal cerebrospinal fluid cytology and biochemistry. She developed severe headache (different and disabling), dizziness, vomiting, and backache on sitting up 6 hours after lumbar puncture, and lying supine relieved all of her symptoms. Intravenous fluids, analgesics, and complete bed rest did not relieve her symptoms over the next 72 hours. She was completely relieved of her symptoms on receiving two tablets of Caffergot containing 200 mg of caffeine and 2 mg of ergotamine 72 hours after lumbar puncture. The symptoms recurred 48 hours later, and a repeat dose of Caffergot was required. Magnetic resonance imaging (MRI) done 96 hours after lumbar puncture revealed the entire dura overlying the brain, including the posterior fossa, showing intense enhancement on contrast injection with leak at the lumbar puncture site. Oral caffeine (coffee, three times a day) was advised over 1 week. The patient remained asymptomatic, and a repeat MRI scan after 10 days showed complete clearing of the cerebrospinal fluid leak with no dural enhancement. The syndrome of cerebrospinal fluid hypovolemia following lumbar puncture is reported in a girl with idiopathic intracranial hypertension.     

Early postoperative feeding and outcome in neonates

OBJECTIVES: The results of neonatal surgery in the Western world have rapidly improved over the past three decades. Early nutrition support is thought to be one of the key factors. We used transgastric, transanastomotic feeding jejunostomy tubes in every infant undergoing upper gastrointestinal surgery when the expected period of fasting has exceeded 7 d. METHODS: Newborns with duodenal atresia, malrotation, and jejunal atresia were treated consecutively between November 1998 and November 1999. We analyzed the outcome of such a practice. There were 17 consecutive babies recruited into the study. Ten babies had duodenal atresia, one associated with esophageal atresia and tracheo-esophageal fistula, six had malrotation, and one had jejunal atresia. The weights of the babies varied between 1.2 and 3.78 kg (mean = 2.1 kg) and they were referred between the ages of 1 and 23 d. Three babies were younger than 32 wk of gestation and weighed less than 1.5 kg; all had multiple bowel atresia, including one with associated pyloric atresia. None of these three survived. Enteral feeding was started by postoperative day 2 in 14 cases. Period of feeding varied between 3 and 20 d, with a mean of 10.4 d in the surviving babies. Three of the tubes had minor mechanical complications. RESULTS: The weight loss or gain during the period of hospitalization was not significant (P = 0.3) Breast milk was the most common nutrient. Thirteen of the 14 babies weighing more than 1.5 kg were discharged and are being followed; the remaining baby died from neonatal septicemia. Excellent results can be obtained with aggressive enteral nutrition support in newborns undergoing upper intestinal surgery. CONCLUSIONS: Transgastric, transanastomotic feeding jejunostomy was well tolerated by the newborns and is preferable to parenteral nutrition.     

Pediatric acute blastic natural killer cell leukemia

The goal of this report is to describe a rare case of pediatric blastic natural killer (NK) cell leukemia and to compare pediatric blastic NK cell leukemia/lymphoma to other reported cases of pediatric NK cell leukemia. The patient, a 9-year-old girl, presented with acute leukemia with a phenotype similar to adult blastic NK cell leukemia/lymphoma. The blasts were agranular and expressed CD7, 45, 56, and HLA-DR, but not CD3, 11c, 13, 33, or TdT. She had a complete response to ALL-directed chemotherapy, but had multiple relapses involving the cerebrospinal fluid, nasal sinus, lymph node and skin. In addition to the reported case, a review of the literature identified 9 previously reported cases of NK cell leukemia in patients 18 years of age or less. Cases were subdivided into blastic, acute/aggressive, and myeloid precursor NK cell leukemia based upon CD13/33 expression and morphologic characteristics. Compared to pediatric acute/aggressive NK cell leukemia, children with blastic NK cell leukemia showed greater variation in age and race. Prognosis was poor for all groups. Pediatric blastic NK cell leukemia is a distinct clinicopathologic entity which differs from other types of pediatric NK cell leukemia.     

Leptomeningeal metastases: current concepts and management guidelines

Neoplastic infiltration of the meninges occurs when malignant cells gain entry into the cerebrospinal fluid (CSF). This is clinically recognized in 4% to 7% of all cancer patients. Leptomeningeal metastases may involve any part of the neural axis via tumor seeding; thus, a multitude of clinical presentations involving one or more domains exist, including the cerebral hemisphere, cranial nerves, and spinal cord and roots. The diagnosis of CSF metastases is often delayed and not appreciated until fixed neurologic deficits become evident. Adequate cytologic analysis of CSF fluid, neuroradiography of brain and spine, and an appropriate clinical context are the key element in diagnosing leptomeningeal metastases. A major challenge of treating neoplastic meningitis is the importance of treating the entire neural axis and stratifying patients in poor risk or good risk categories. Treatment is palliative and involves stabilizing neurologic status and prolonging survival. Median survival for untreated patients is 4 to 6 weeks. Treatment in a broad perspective entails radiotherapy and chemotherapy (systemic and intra-CSF). Commonly used intra-CSF chemotherapy regimens use drugs such as methotrexate, cytarabine, thiotepa, and a sustained-release liposome-encapsulated form of cytarabine (Depocyt, SkyePharma, London, UK). Patients with neoplastic meningitis usually experience a limited survival, even when treated using close adherence to evaluation algorithms and treatment protocols. In randomized controlled clinical trials using currently available intra-CSF chemotherapeutic agents, median survival in carefully selected, study-eligible groups of patients was 2 to 6 months.