Meningeal signs as the presenting feature of medulloblastoma.
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Biomedical subjects
Publications and source records attributed to S Tomarchio.
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Lipoblastomas are most commonly superficially-located tumors. Our experience with three examples of deep-seated lipoblastomas is reported. The CT scans of all cases showed low-attenuation densities (less than 0 Hansfield units), strongly suggesting fatty composition. Fine-needle aspiration biopsy (FNAB) smears contained uni- and multivacuolated lipoblasts, myxoid areas, and a plexiform capillary network. The combination of CT scan and FNAB findings seems to be enough for definitive preoperative diagnosis of lipoblastoma, allowing differential diagnosis with other deep-seated tumors.
BACKGROUND: The very low frequency of rhabdomyosarcoma (RMS) of the extrahepatic biliary tree has impeded the development of a standardized form of treatment. PROCEDURE: Based on the good response of embryonal RMS to chemotherapy, we used a multi-drug protocol as the initial treatment of a 3-year-old girl after obtaining adequate transparietohepatic biliary drainage. The treatment achieved complete remission. Later conservative laparoscopic surgery revealed only residual scar tissue. The patient is alive and well 1 year after remission. DISCUSSION AND CONCLUSIONS: Management of this difficult tumor using modern less invasive techniques for diagnosis and treatment, with the help of preoperative chemotherapy, prevented extensive damage to the biliary tree and allowed complete recovery.
Sixty consecutive cases of tumor of the right colon operated upon between 1981 and 1991 are reviewed. Age and sex distribution, clinical findings, preoperative diagnostics and system used staging, TNM classification, surgical management, operative mortality, follow up and survival were analyzed. Results are discussed and compared with published data. In the second of the two 5-year period considered an increased incidence of right-sided carcinoma compared with tumors of the left colon and rectum was observed. According to the TNM classification, more than 50% of patients were in stage III. Operative mortality was 5%. The overall 5-year survival rate was 58%, while the survival rate of patients treated with radical intent was 70%.
117 cases of spontaneous pneumothorax are reviewed. The authors report their experience and consider surgical drain to be the best method in most cases to achieve a rapid recovery. Surgical management or conservative approach can also to be used in selected patients.
Personal experience in an enterostomy centre over a 5-year period is report. The problems deriving from the presence of a stoma are examined with particular emphasis on surgical complications which are grouped into early and late forms.
The Authors report a case of coeliac axis atheromatous obstruction, with superior mesenteric artery steal. The coeliac axis, stopped up the origin was indirectly revascularized by hepatic artery. The early diagnosis of chronic intestinal ischaemia should avoid the intestinal infarction. The development of intravenous digital angiography will certainly help an early diagnosis.
34 patients admitted for suspected acute cholecystitis were evaluated using 99mTc IDA cholescintigraphy. The results of these studies are reviewed and compared with other diagnostic tests and the subsequent clinical diagnosis. Cholescintigraphy proved to be a safe, simple, highly accurate and sensitive technique. Therefore, 99mTc-IDA cholescintigraphy is proposed as the initial procedure of choice in the evaluation of patients with suspected acute cholecystitis.
Three Sicilian siblings had a typical cone-rod dystrophy. The same ocular anomaly was probably present in their mother and in the maternal grandmother. The three probands were also carriers of Hb S, but this last anomaly was also present in an unaffected brother. It is concluded that the cone-rod dystrophy of this family is inherited as an autosomal dominant character and that its association with sickle-cell trait is coincidental.
Visual-evoked potentials (VEPs) were studied in 55 asymptomatic children with leukemia or solid tumors in remission in order to detect subclinical demyelination of the optic pathway after CNS prophylaxis. In group I (11 patients with ALL studied prospectively), VEP latency was increased in ten after cranial radiation (CR) as compared with previous values. Group II (18 patients with ALL in maintenance) and group III (16 patients with ALL off therapy) were studied retrospectively and VEP latency was found above normal limits in 33 and 31%, respectively. In group IV (four patients with solid tumors and six with leukemia, all of whom received no CR), VEP latency was normal despite periodical intrathecal methotrexate administrations to five of them. We conclude that CR determines a slowing of conduction on VEP test, probably due to demyelination of the optic pathway, in a high proportion of patients. The future clinical significance of these findings must be established throughout a prolonged follow-up period.
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A Sicilian family is reported in which 36 individuals, in 5 successive generations, were affected with congenital cataracts, microcornea and myopia with an autosomal pattern of inheritance.
The authors examine a clinical case of the Marcus Gunn phenomenon, characterized by the lid retraction when the patient closes her mouth. The peculiarity of this clinical case would consist in a partial synkinesis involving the masseter and temporal muscles.
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Serial blood T4 and TSH determinations were performed on a total number of 352 low birth weight (LBW) infants: 210 healthy newborns and 142 infants affected by respiratory distress syndrome (RDS), sepsis, hyperbilirubinaemia, hypocalcemia, or hypoglycemia. The healthy infants have been divided into three groups in relation to gestational age (less than or equal to 33 wk, 34-36 wk, greater than or equal to 37 wk). Statistical evaluation among groups shows that reduced T4 concentration in LBW infants is strictly related to gestational age, while TSH failed to demonstrate any significant correlation. No significant influence of birth weight on T4 and TSH has been observed. The preterm newborns affected by RDS or sepsis showed a significant reduction of mean T4 concentrations up to 20th day of life when compared with healthy controls similar for gestational age and birth weight. TSH levels are not significantly different. No significant modifications of T4 and TSH have been found related to the other pathological conditions. The significant reduction of T4 and the high incidence of subjects with low T4 values observed in healthy and sick preterm population lend further support to the use of TSH determination for optimum screening of congenital hypothyroidism.
The authors report on the surgical procedure for retroequatorial myopexis, with and without rearward relocation. Analysis of the surgical procedure for retroequatorial myopexis of the rectus lateralis and medialis with and without rearward relocation: 1) simple myopexis - anchoring the muscle on the sclera 14 mm from the insertion of the rectus medialis (19 mm in the case of the rectus lateralis); 2) combined procedure (myopexis and rearward relocation). Forty patients with esotropia and unstable angle, as well as clear signs of a blockage, underwent surgery. In 47% of the patients sensoric orthophoria was achieved, in 43% a significant reduction of the angle; in 5% treatment resulted in overcorrection and in 5% the angle was unchanged.
Congenital epiphora due to absence or imperfection of lacrimal puncta and/or canaliculi was present in 21 members from five consecutive generations of a Sicilian family. The anomaly was transmitted as an autosomal-dominant characteristic with apparently complete penetrance and highly variable expressivity.