Biomedical subjects
Publications and source records attributed to S Constantini.
A newborn with Dandy-Walker syndrome was found to have a trisomy 21 karyotype. This is the first reported case of coexisting Dandy-Walker and Down's syndromes. The pathogenetic alternatives for the development of Dandy-Walker syndrome and the implications of the case are discussed.
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Aluminium introxication in uraemic patients has been reported both in epidemic and sporadic forms. Desferrioxamine (DFO) results in strong Al mobilisation, but definitive treatment schedules, times of stop, and actual long-term Al removal her not been established. This study was carried out on three sporadic cases of Al intoxication treated with DFO for 3 1/2, 4 1/2 and 1 years. Clinical and laboratory parameters were employed to check the brain, bone and red cell status. Direct evaluation of Al kinetics showed that actual Al removal may be overestimated. In our experience it did not exceed 100 mg/year, and the ratio (DFO used/Al removed) decreased to 150 g/25 mg after two years of DFO, though considerable tissue deposits persisted in the patient who died after 3 1/2 years of treatment. Despite unexplained EEG worsening, neurological symptoms improved. Relapses, however, occurred after many years of DFO. Bone status improvement, at least in parathyroidectomised patients, proved to be only partial and time-related. The therapeutic effectiveness of DFO in these patients seemed to be exhausted after two years even if Al deposits had not vanished.
The authors evaluate the efficacy of a protocol of prevention and treatment of aluminum (Al) overload in RDT patients during a 7-year period (from 1981, 164 patients, to 1987, 161 patients). Al in dialysate solutions was always less than 25 micrograms/l. Baseline Al levels greater than 100 micrograms/l were found in 22% of patients in 1981 but in none in 1987, while the percentage of values less than 60 micrograms/l increased from 55 to 91%. DFO tests were positive in 54% and 7% of cases in 1981 and 1987, respectively. A clinical diagnosis of Al intoxication was performed in 6 patients in 1981, and no further cases were diagnosed later. DFO treatment (50 mg/kg once a week) was employed preventively in 31 patients owing to positive DFO-tests, and in the 6 Al-intoxicated patients therapeutically. In the former patients none developed clinical intoxication. In the latter group clinical improvement was only temporary in the three parathyroidectomized patients. Al hydroxide [Al(OH)3] as a phosphate binder was tapered off in 1981 and substituted by Al-free chelants. In 1987, 66% of patients were given CaCO3 or Mg (OH)2 alone or in association, while 34% still needed Al(OH)3, although at low dosages (less than 2 g/day). The conclusion is that such a protocol is able to prevent and to treat cases of Al intoxication, albeit only partially.
We report a case of a 14-month-old boy with a primary intraventricular oligodendroglioma and obstructive hydrocephalus. The child underwent a bilateral ventriculoperitoneal shunt and developed a massive and fatal intratumoral hemorrhage initiated by a mild trauma when introducing the left ventricular catheter. To our knowledge, this is the first report in the literature of such a complication during a shunting procedure for obstructive hydrocephalus of tumoral origin.
Torsade de pointes (atypical ventricular tachycardia) occurred in a 75-year-old man 1 day after blunt head injury, in association with a focal intracerebral hematoma in the left thalamic area. The causal relationship between the traumatic stimulation of the left sympathetic pathway and the development of this life-threatening arrhythmia is discussed.
A retrospective study of 514 consecutive patients whose intracranial pressure (ICP) was monitored after elective supratentorial or infratentorial surgery is reported. Of the 412 patients operated on in the supratentorial region, 76 (18.4%) had a postoperative sustained ICP elevation exceeding 20 torr. Abnormally high ICP occurred after 13 (12.7%) of the 102 infratentorial operations. Risk factors for postoperative ICP elevation were: resection of glioblastoma in 27.2% of cases, repeat surgery in 42.9% of cases, and protracted surgery (greater than 6 hours) in 41.7% of cases. Of the 89 patients with elevated ICP, 47 (52.8%) had an associated clinical deterioration. In 19 of these, the rise in ICP occurred before this deterioration was noticed, leading as a rule to quick diagnostic and management response. In eight patients clinical deterioration was noticed before the rise in ICP, and in 20 it happened simultaneously. The higher the level of ICP elevation, the greater were the chances of associated deterioration. The most common findings on computerized tomography scanning in 35 of 89 patients with elevated ICP were brain edema (19 cases) and bleeding in the tumor bed (15 cases). Mannitol, thiopental, additional hyperventilation, and reintubation (in patients who were previously extubated) were used to reduce ICP, in addition to surgical decompression whenever indicated. Thirteen patients with raised ICP and clinical deterioration underwent reoperation. The postoperative infection rate was 1.2% (six cases). In only one patient could infection be attributed to ICP monitoring. It was concluded that ICP monitoring is advantageous in the immediate postoperative management after elective intracranial surgery and is almost risk-free. It should therefore be used liberally, especially when risk factors for ICP elevation can be identified prior to the end of surgery.
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A hydrocephalic child with a V-P shunt developed transient blindness following shunt revision. A year later, visual function deteriorated when shunt malfunction occurred. Following shunt revision, the child regained sight. The effects of intracranial hypertension and hypotension on the visual pathways are discussed.
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A 17-year-old girl presented with signs of increased intracranial pressure. On computed tomography, an enhancing intraaxial lesion in the region of the foramen magnum was demonstrated. Surgical excision was performed. The pathological diagnosis was low grade fibrosarcoma. Her subsequent course was complicated by cerebrospinal fluid seeding, a posterior fossa recurrence, and repeated subarachnoid hemorrhage with cerebral vasospasm. A combination of radiotherapy and intraventricular chemotherapy has left the patient symptom-free 2 years after operation. The intramedullary appearance of fibrosarcoma and the unusual subsequent clinical course are discussed.
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A traditional philosophy statement can be transformed into a positioning statement that articulates a "vision" for a department of nursing and provides a framework when planning to thrive rather than merely survive in today's changing and competitive health care environment. Internal and external forces appropriately assessed enable nurse executives to empower staff to respond proactively to change and prepare for the future.
A 4-year-old girl with a right ventriculoperitoneal shunt presented with a huge subcutaneous fluid collection in the scalp on the right side. There were no neurological symptoms or signs. On CT, a normal-sized ventricular system was seen. The shunt was disconnected distal to the antisiphon device and transmitted fluid into a subgaleal collection, which functioned as a reservoir for excess fluid. The ability of the subgaleal space to serve as a reservoir for cerebrospinal fluid is discussed.
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The treatment of brain stem gliomas has evolved over the last few decades, reflecting advances in imaging (MR), microsurgical techniques and biological understanding. The aim of this chapter is to provide a preoperative classification for intrinsic brain stem lesions that will predict histopathology and biological behavior from the clinical syndrome and the MR appearance. Such a classification system may help selecting children with brain stem tumors that can benefit from surgery. Technical considerations, potential surgical complications, and the ways to avoid them are discussed.
The purpose of this study was to deliver tamoxifen as antiangiogenic therapy to children with recurrent progressive malignant brain tumors. Tamoxifen was administered orally in very high dosage to one child as monotherapy and to two children in combination with oral etoposide and dexamethasone. One boy was diagnosed with high-grade astrocytoma in the brain stem, one girl with anaplastic ependymoma of the fourth ventricule, and one girl with high-grade astrocytoma in the midbrain. Conventional treatment with multiple surgeries, first- and second-line chemotherapy, and external beam therapy had failed. Tumor reduction was seen in radiographic images together with clinical improvement in 2 children, and clinical and radiographic halting of tumor progression was demonstrated in the patient with anaplastic ependymoma. None of the patients developed complications from the treatment. Follow up of the patients ranged from 15 to 30 months with a mean of 17 months. These encouraging preliminary results suggest a potential for this type of therapy. More studies are needed to start clinical trials and prove that angiostatic activity may contribute to the therapeutic effect of antiestrogens in estrogen receptor-negative tumors.
Fine needle aspiration biopsy was performed in two case of infiltrating skull lesions. The role played by this relatively simple procedure in avoiding a major operation is discussed.