1/18. Secondary myelodysplasia with monosomy 7 arising after treatment for acute lymphoblastic leukemia in childhood.monosomy 7 is recognized as a characteristic, clonal abnormality associated with acquired myelodysplasia (MDS) or acute myeloid leukemia (AML). It can occur as a late complication of cytotoxic therapy and is usually associated with exposure to alkylating agents or radiation therapy. We report two patients with therapy-related myelodysplasia (t-MDS) associated with monosomy 7 occurring in children after completion of therapy for acute lymphoblastic leukemia (ALL). Both children were noted to have t-MDS with monosomy 7 at the time of cessation of chemotherapy. Neither child had received an alkylating agent or radiation therapy during treatment. One child had a unique dicentric marker chromosome that was shown by fluorescent in situ hybridization to be derived from chromosome 7. This report emphasizes the need to identify and minimize therapy-related side effects without compromising cure rates.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
2/18. Neurenteric cyst with alteration of signal intensity on follow-up MR images.SUMMARY: Intracranial neurenteric cysts are uncommon and usually have low intensity on T1-weighted MR images and high intensity on T2-weighted MR images. We report a case of a neurenteric cyst that was situated in front of the medulla oblongata and the size of which increased with alteration of MR signal from high to isointense compared with that of brain on T1-weighted images obtained 33 months after the initial MR images. We think that the signal change of the cyst was probably caused by a change of protein concentration.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
3/18. Thoracic intradural extramedullary lipomas. Report of three cases and review of the literature.BACKGROUND: Indication for surgery and the surgical strategy for treatment of spinal intradural lipomas are still discussed controversially. Among spinal lipomas the cervicothoracic region is rarely affected. We report on our experience with spinal decompression for the treatment of three intradural thoracic lipomas. FINDINGS: Three adult patients with extramedullary intradural lipomas of the thoracic spinal cord demonstrated progressive neurological symptoms and signs without evidence of growth of the lipoma. All patients underwent surgery with decompression of the affected spinal levels. No attempt was made to reduce the size of the lipomas. Postoperatively, each patient demonstrated significant clinical improvement. Interpretation. Attempts to remove lipomas are associated with significant risks of surgical morbidity. Lipomas are hamartomas which change their size according to alterations of body fat. Therefore, decompression of the affected spinal levels is sufficient to achieve significant neurological improvement.- - - - - - - - - - ranking = 2keywords = size (Clic here for more details about this article) |
4/18. A case of diastematomyelia associated with myeloschisis in a hemicord.We report a case of myeloschisis on the right hemicord in a patient with diastematomyelia. The patient was a female neonate with myeloschisis visible at birth in the upper lumbar region. Radiological examination, including three-dimensional CT and MRI, clearly revealed a bony septum as well as the myeloschisis on the right hemicord, which enabled us to make a precise preoperative diagnosis of this complex anomaly. Closure of the myeloschisis and removal of the septum were successfully accomplished in one stage to prevent subsequent infection and neurological deterioration. The presence of combined diastematomyelia and myeloschisis is consistent with the hypothesis of an ontogenic basis of development and emphasizes the importance of early imaging for diagnosis in this complex anomaly.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
5/18. Neurenteric cyst in the cerebellopontine angle with xanthogranulomatous changes: serial MR findings with pathologic correlation.We report serial MR findings in a middle-aged woman with a neurenteric cyst involving the cerebellopontine angle cistern with xanthogranulomatous changes. On the initial gadolinium-enhanced T1-weighted MR images, the solid portion of the lesion had homogeneously strong enhancement. Follow-up MR images obtained 6 months later showed that the mass had increased in size; however, the solid portion decreased in size. The enhancing solid portion corresponded to the xanthogranulomatous changes on pathologic correlation.- - - - - - - - - - ranking = 2keywords = size (Clic here for more details about this article) |
6/18. Presacral neurenteric cyst in an infant.Intraspinal neurenteric cysts are rare congenital cystic lesions, formed due to displacement of elements forming the alimentary canal. We report an 11-month-old infant presenting with a soft, fluctuant and transilluminant swelling posterior to the anus since birth; the size of the swelling varied with alterations in intraspinal pressure subsequent to crying, coughing, or sneezing. MRI showed a multiloculated lesion anterior to the sacrum and coccyx. Near total excision was carried out without any worsening in the neurological status.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
7/18. Giant supratentorial enterogenous cyst: report of a case, literature review, and discussion of pathogenesis.OBJECTIVE AND IMPORTANCE: To describe a histologically well-documented adult case of a giant supratentorial enterogenous cyst (EC). Fewer than 15 cases of supratentorial ECs are on record: 8 associated with the brain hemispheres or the overlying meninges, 4 with the sellar region, and 2 with the optic nerve. CLINICAL PRESENTATION: A 31-year-old woman complained of long-standing mild left brachial and crural motor deficit precipitated by headache and signs of intracranial hypertension. magnetic resonance imaging revealed a huge cyst overlying the frontoparietal brain. INTERVENTION: Symptoms were relieved by evacuation of the cyst content by means of a Rickam's reservoir, and the lesion was subsequently removed in toto. Histological and immunohistochemical examination of the cyst wall clearly established the enterogenous nature of its epithelium. Follow-up for up to 2 years after intervention showed no sign of recurrence, and symptoms, including treatment-resistant seizures in the postoperative period, have entirely subsided. CONCLUSION: Supratentorial ECs, distinctly rare in adult patients, may in some cases present as giant lesions. Total removal seems to be curative once careful examination has eliminated the possibility of a metastasis from an unknown primary. A correct histological diagnosis is important because, in contrast to other benign cysts of similar location and size, ECs may be prone to intraoperative dissemination.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
8/18. Transient ventriculoperitoneal shunt dysfunction in children with myelodysplasia and urinary bladder infection. Report of three cases.The authors present three children born with myelomeningocele and hydrocephalus. Each presented with symptoms/signs of ventriculoperitoneal shunt malfunction. All patients at the time of presentation exhibited significant urinary bladder infections and were appropriately treated for their infection. No patient was found to have an underlying shunt infection. All patients without medically threatening symptoms were carefully observed and noted to have resolution of their shunt dysfunction symptoms/signs following treatment of their urinary bladder infections and thus did not undergo a shunt operation. Based on the courses of these patients, we believe that significant urinary bladder infection in patients with myelodysplasia in whom a shunt has been placed may often be enough to bring a subclinical shunt malfunction to clinical attention or even to be the cause of temporary distal peritoneal shunt malabsorption. Although the exact mechanism for this dysfunction is unclear, treatment of the bladder infection may address the symptomatic shunt dysfunction in some patients so as to avoid operative intervention. We emphasize, however, that careful observation of these patients should be performed during hospitalization because they often rely on adequate cerebrospinal fluid diversion. Only patients with mild symptomatology should be observed first as the initial line of treatment.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
9/18. association of neural tube defects with omphalocele in chromosomally normal fetuses.Omphalocele is frequently associated with other congenital malformations or a chromosome abnormality. Previously published series of omphalocele have emphasized the association of congenital heart defects or chromosomal abnormalities. We present five cases of omphalocele with concurrent neural tube defect from among the 15 cases of omphalocele evaluated between 1981 and 1985. Fourteen of 15 were detected prenatally. A sixth case is presented in which both a neural tube defect and an omphalocele were suspected on a prenatal ultrasound, but only the latter was found on autopsy. We recommend that a systematic evaluation be performed on every fetus with an omphalocele to include amniotic fluid alpha fetoprotein, acetylcholinesterase levels, chromosome study, and careful ultrasonography looking for evidence of other abnormalities, especially neural tube defects, before counseling the parents.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
10/18. Diastematomyelia of the cervical spinal cord with tethering in an adult.A case of cervical diastematomyelia associated with spinal cord tethering in an adult is presented. The differences between cervicodorsal and lumbar region spinal dysraphic states are emphasized with a review of the embryological factors involved. Possible causes and subsequent treatment are outlined.- - - - - - - - - - ranking = 1keywords = size (Clic here for more details about this article) |
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