Mark D. Krieger
Tord D. Alden and Mark D. Krieger
Mark D. Krieger, Stefan Blüml and J. Gordon McComb
Diffuse pontine gliomas in children carry a dismal prognosis, with a mean survival of less than 1 year despite therapy. The diagnosis is based on the characteristic changes demonstrated on traditional magnetic resonance (MR) imaging. A few typically MR imaging–appearing pontine masses, however, do not behave in the expected fashion, which calls the original diagnosis into question.
The authors conducted a retrospective review of data obtained in 42 children (age 6 months–13 years) in whom diffuse pontine glioma had been diagnosed at their institution. Five of these patients (12%) survived longer than expected (> 8 months). There were no differences in these patients in terms of demographics, presentation, traditional imaging findings, or treatment compared with the group as a whole. Magnetic resonance spectroscopy, however, demonstrated two distinct patterns not seen in typical diffuse pontine gliomas. In two patients elevated lipid and lactate levels were shown, with decreased levels of choline, myoinositol, and n-acetyl-aspartate (NAA). In the other patients strikingly elevated choline/creatinine ratios and myoinositol levels were observed in comparison with typical pontine tumors.
These MR spectroscopy patterns demonstrated in this retrospective study seem to convey prognostic information and may lead to an expansion of this diagnostic tool.
John A. Jane Jr., Mark D. Krieger and Alan R. Cohen
Douglas L. Brockmeyer
Laurence Davidson, Mark D. Krieger and J. Gordon McComb
The purpose of this study was to evaluate the posterior interhemispheric retrocallosal approach (PIRA) for its safety and efficacy in the resection of pineal region and posterior fossa lesions in children.
Twenty-nine PIRAs were performed in 26 children between March 1997 and March 2009, and these cases were retrospectively reviewed. There were 15 girls and 11 boys in the series. The median age at the time of surgery was 7 years (range 7 months–17 years). Twenty-seven cases were treated for tumor, 1 for loculated hydrocephalus, and 1 for an aneurysm.
Of the 27 cases treated for tumor, there were 20 (74%) gross-total resections, 5 (19%) subtotal resections, and 2 (7%) biopsies. One bridging vein was sacrificed in 6 cases and 2 bridging veins were divided in 1 case, whereas in 3 cases there was sacrifice of a single deep cerebral vein. No patient developed radiographic evidence of venous infarction. Approach-related complications were low, and included 2 cases of transient homonymous hemianopia. There were no surgery-related deaths.
This approach allows for ample access to pineal region and posterior fossa lesions, with low postoperative morbidity.
David I. Sandberg, J. Gordon McComb and Mark D. Krieger
The authors analyzed the chemical composition of intracranial arachnoid cyst fluid to gain greater understanding of potential causes of cyst growth.
The authors studied 54 pediatric patients who underwent craniotomies for arachnoid cyst fenestration. Cyst fluid was analyzed and results were compared with expected values (EVs) for cerebrospinal fluid (CSF).
The arachnoid cyst fluid values were as follows: protein (median 37 mg/dl, mean 178.2 mg/dl, and EV 25.6 mg/dl), osmolality (median 284 mOsm/kg, mean 285.5 mOsm/kg, and EV 287.2 mOsm/kg), potassium (median 2.6 mEq/L, mean 2.68 mEq/L, and EV 2.88 mEq/L), sodium (median 140 mEq/L, mean 142.2 mEq/L, and EV 143.3 mEq/L), and chloride (median 122 mEq/L, mean 120.77 mEq/L, and EV 120.3 mEq/L). Median glucose was 51 mg/dl (range 26–98 mg/dl). The median white blood cell count in cyst fluid was 1/mm3, and the median red blood cell (RBC) count was 1.5/mm3. All Gram stains and cultures were negative.
No statistically significant correlations were found between arachnoid cyst fluid protein level and elevated RBC count, patient age, or the subsequent need for a shunt. Elevated RBC count in cyst fluid was also not associated with the subsequent need for a shunt.
Fluid from arachnoid cysts has a chemical composition similar to that of CSF, but some of them have significantly elevated protein levels. The authors hypothesize that elevated protein content may contribute to arachnoid cyst expansion in some patients.
Michael L. Chen, J. Gordon McComb and Mark D. Krieger
Atypical teratoid/rhabdoid tumors (ATRTs) represent a relatively newly categorized neoplastic entity. They commonly present in childhood, and have a rapidly progressive clinical course with a survival time of less than 1 year. Treatment regimens have been nonuniform. In this retrospective review of patients with ATRTs who were treated at the authors' institution according to a uniform protocol, the goal was to assess the efficacy of the treatment and its outcome.
Over a 7-year period, ATRT was diagnosed in 11 patients (six boys and five girls). The median age of the patients was 61 months, and their ages ranged from 3 months to 17 years. Signs and symptoms began, on average, a little more than 1 month before diagnosis and included the following: headache (36%), nausea and vomiting (46%), lethargy (18%), seizures (27%), cranial nerve findings (46%), ataxia (18%), long tract findings (18%), and hydrocephalus (46%). Tumor location was cortical in four patients, in the pineal region in four, in the posterior fossa in two, and spinal in one. In one patient disseminated disease was revealed on the initial imaging study; seven patients had disseminated tumor subsequently. Treatment consisted of chemotherapy in 11 patients, chemotherapy and local radiation in five, and chemotherapy and craniospinal radiation in three. Six patients are alive, three have died, and two were lost to follow-up review. The mean time to death was 24 months, and ranged from 2 to 67 months. Among the surviving patients the mean duration of follow up is 18.5 months and ranges from 2 to 37 months. The median time to progression was 3.5 months.
Atypical teratoid/rhabdoid tumors are malignant lesions with rapid progression. Further study is necessary to determine the efficacy of therapy.