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Biomedical subjects

C Di Rocco

Publications and source records attributed to C Di Rocco.

At least 91 records · Page 5Linked to original sources

Metopic synostosis: in favour of a "simplified" surgical treatment.

Metopic synostosis is a relatively simple form of craniosynostosis, resulting from premature fusion of the metopic suture. In this pathology different degrees of dysmorphia of the anterior cranial fossa and the presence of associated anomalies of the skull might enable specific subgroups to be identified. Since most functional and cosmetic anomalies benefit from early surgical treatment, over the last few years neurosurgeons have been forced to elaborate less drastic, but nonetheless effective, surgical techniques. In the present report we analyze the surgical results obtained in a series of 62 infants with trigonocephaly operated on within their 1st year of life. Patients were subdivided into two groups (group I: 8 patients; group II: 54 patients) according to the specific dysmorphic characteristics of the frontal bone and anterior cranial fossa, and the presence of compensatory deformities affecting the anterior cranial base and temporo-parietal region. All the patients were treated using one of two relatively simple surgical techniques (procedure A: inversion of two hemifrontal bone flaps--48 cases; procedure B: the "shell" operation--14 cases). Both surgical procedures appeared to be effective, allowing adequate functional and cosmetic correction of the cranial deformity. In patients operated on following procedure B surgical time and blood loss were dramatically reduced. Long-term outcomes were satisfactory in all cases, irrespective of the surgical technique used. In the group II patients, however, progressive normalization of the interorbital distance was constantly observed, suggesting a different degree of stenotic involvement at the level of the anterior cranial base in these patients.

Adolescent↗

Hemimegalencephaly associated with somatic hemihypertrophy and a malformation of the feet: case report.

Hemimegalencephaly (HME) is a rare neuronal migration anomaly, rarely associated with hemihypertrophy or some other somatic malformation; it can coexist with intracranial arteriovenous shunts which cause cardiac failure. We report a rare case associated with hemihypertrophy of the face, trunk and limbs and malformation of the feet. The neuroradiological findings are underlined and the differential diagnoses discussed.

Abnormalities, Multiple↗

Neonatal diagnosis of tuberous sclerosis.

Intracranial tumors are rare in the neonatal period, and generally the most common histological types are astrocytoma, medulloblastoma, choroid plexus papilloma and neuroectodermal tumors. The early diagnosis of these tumors is often very difficult. The authors report a case of a full-term newborn who presented with opisthotonus. A subependymal mass was detected by cerebral ultrasonography, and when the child was 1 month of age depigmentations appeared on the trunk and on the right leg, confirming the suspicion of tuberous sclerosis. At 3 months of age the child suffered infantile spasm with hypsarrhythmia. The developmental delay, the marked progressive neurological deterioration and the daily seizures suggested surgical resection. Histologic studies showed a subependymal giant cell astrocytoma such as typically occurs in tuberous sclerosis.

Brain↗

Cavernomas of the central nervous system in children. A report of 22 cases.

A series of 22 patients under the age of 15 years with cavernomas were treated at the Section of Paediatric Neurosurgery of the Catholic University of Rome between 1981 and 1995. The most common symptoms at presentation were epilepsy, in 12 children, and intracranial hypertension, in 9 children; seizures were present on admission in 14 patients; no patient had a family history of cerebro-vascular malformations, two had multiple lesions. Radiological signs of significant acute and subacute haemorrhage were found in 17 cases. The supratentorial compartment was the most frequent location of the lesions, with only three subtentorial cavernomas. The post-surgical results were good: only four children had a persistence of pre-surgical neurological signs, even though they were ameliorated by the treatment. No patient showed a progression of pre-operative neurological signs, nor the onset of new deficits. Only one child died, but his clinical condition was critical before surgery, because of deep coma, with bilaterally dilated and fixed pupils, and no response to any stimulation. Our results suggest that the younger children present significant haemorrhage more frequently than the older ones; this is demonstrated by the analysis of the mean age of the patients with macro-haemorrhage (= 7 years) and the average age of those without macro-haemorrhage (= 11 years). The removal of cavernomas was always able to control the seizure disorders of our patients, proving that in these cases lesionectomy alone may be sufficient to resolve epilepsy.

Adolescent↗

Surgical options in the treatment of interhemispheric arachnoid cysts.

BACKGROUND: Arachnoid cysts located within the interhemispheric fissure are a matter of discussion as to the surgical indication and the choice of the most appropriate surgical procedure. In fact, in spite of the large dimensions that they can reach, the clinical manifestations may remain subtle and apparently stable over the years. On the other hand, the larger utilization of prenatal ultrasound investigations has increased the number of lesions detected before they can provoke clinical manifestations. As far as surgery is concerned, two main options are available-an extrathecal shunting procedure or a direct approach to the lesion, with excision of its wall. METHODS: In this report, we analyze the results obtained in 25 children harboring an interhemispheric arachnoid cyst, surgically treated in the period 1978 to 1994. Mean age at diagnosis was 18.8 months (10 days to 15 years). RESULTS The main clinical manifestations included macrocrania, mild to moderate signs of increased intracranial pressure, cranial bulging, developmental delay, and neurologic signs. In 14 cases, the diagnosis had been obtained prenatally. The cyst was located in one hemicranium in 16 cases, whereas it was on the midline, with bilateral extension, in the remaining 9 cases; in the latter cases, variable degrees of callosal agenesis were also observed. All 25 children were operated on. In 16 of them, the treatment consisted of a craniotomy with wide excision of the cyst lining and marsupialization into the subarachnoid spaces of the midline (and/or into the ventricular system). In 6 children, the surgical procedure was a cystoperitoneal (CP) shunt. The last 3 children of our series underwent craniotomy and cyst excision after having been treated by means of a ventriculoperitoneal shunt. At follow-up examination, 17 children are normal, and the remaining 8 disclose mild to moderate psychomotor retardation. CONCLUSIONS: Although both craniotomy and CP shunt are associated with good results, the first surgical procedure offers the advantage of avoiding the insertion of a permanent cerebrospinal fluid shunt device and the known complications of this type of procedure.

Adolescent↗

[Optic gliomas in the first year of life].

BACKGROUND: Optic gliomas are mainly observed in pediatric patients. Twenty-five percent of cases are diagnosed in the first year of life. Infants with optic gliomas must be considered distinctly from the rest of pediatric patients with this type of lesion, because of the more invasive nature of the tumour and a worse prognosis. Most authors think that surgical treatment of optic gliomas has to be limited to a diagnostic biopsy of the lesion and, in selected cases, to partial decompressive excision. Chemotherapy is useful in the control of the disease, particularly when combined with surgery. Radiotherapy has important secondary effects on the immature brain, and its use is restricted to patients who have reached the 2nd year of life. METHODS: We report our observations on 11 patients with optic gliomas diagnosed in the first year of life, and treated at the Section of Pediatric Neurosurgery of the Catholic University of Rome between 1980 and 1994. RESULTS: In 45.4% of cases the tumour involved the intracranial optic nerve, the optic chiasm, the optic tract and the thalamus. Nystagmus was the most frequent clinical sign, and it was observed in all cases. Signs of intracranial hypertension were observed in 72.6% of cases. We performed a partial decompressive excision of the tumour in 82% of patients. In 2 of these cases we have observed a spontaneous disappearance of the tumour residue and in 5 cases a prolonged time of survival. Mortality has been lower in our group of patients, compared with other authors' experience (36.4% vs 50-80%). Also morbidity has been lower. A severe neurological worsening was observed in two patients and a complete blindness in other two cases (18.1% vs 66.6-100%). CONCLUSIONS: We think that the lower morbidity and mortality observed in our patients can, at least in part, be ascribed to a more aggressive surgical treatment of the lesions.

Child↗

Carboplatin in childhood medulloblastoma/PNET: feasibility of an in vivo sensitivity test in an "up-front" study.

Sixteen patients with high risk MB/PNET at diagnosis were included in a pilot study employing carboplatin (CBDCA) as a single drug prior to conventional therapy. The main goal of the study was to identify in a short-term trial a significant response that would predict further response to CBDCA in the single patient. Exploration of CBDCA activity was focused on response after the first course as compared to the response following the second course. A course consisted of CBDCA 600 mg/m2 on days 1 and 2 administered in a 1 h infusion to be repeated 3-4 weeks later. After two cycles we observed 1 CR and 9 PR, that is a 62% response rate. The first course resulted in 5 PR, 5 MR, 5 SD, and 1 PD; after the subsequent course in all responding patients, response persisted or improved whereas in no patient with SD any improvement was observed. The correlation of response to the first course with response to the second course was statistically significant (P = 0.0009). The main toxicity of the single course was hematologic and consisted of rapidly reversible grade 3-4 neutropenia and thrombocytopenia in 94% of patients. Pharmacokinetic studies showed a very limited interpatient variability of both Cmax 57.6 +/- 9.9 micrograms/ml) and AUC (15.3 +/- 1.5 mg/ml.min) of free CBDCA, which eliminates an important variable in the evaluation of response. In conclusion, this "in vivo test" appears effective, reasonably safe, and reproducible in identifying patients likely to benefit from CBCDA: after a period of time as short as 3-4 weeks following the first course, multidrug chemotherapy including CBDCA may be employed in the responding patients, whereas an alternative regimen would be indicated in the non-responding patients.

Adolescent↗

Preoperative chemotherapy with carboplatin alone in high risk medulloblastoma.

Few studies have been carried out regarding preoperative chemotherapy utilized in the treatment of primitive neuroectodermal tumors/medulloblastomas (PNET/MB). In this paper, the authors report 3 cases of children under three years of age, with a presumed diagnosis of PNET/MB, who were preoperatively treated with chemotherapy consisting of high doses of carboplatin alone. The treatment improved the childrens' clinical condition prior to surgery and facilitated tumor removal, resulting in partial regression of the tumor. The preoperative single-drug therapy did not affect the histological diagnosis or prevent the presence of an effective degree of tumor sensitivity to the drug. All three children are still disease-free, after a mean follow-up period of 42 months.

Antineoplastic Agents↗

Surgical treatment of late neurological deterioration in children with myelodysplasia.

Late deterioration of the neurological condition in patients operated on for myelomeningocele repair has been repeatedly reported in the literature. At the present time magnetic resonance imaging (MRI) allows one to recognize various pathological conditions which can be amenable to surgical correction in these patients, such as Chiari type II malformation, hydro/syringomyelia, decompensated hydrocephalus, and tethered spinal cord. The authors report their experience with 26 myelodysplastic children operated on for myelomeningocele repair in the early neonatal period, who exhibited late deterioration at variable time intervals from the first operation. The children were examined pre-operatively by means of MRI; the results were compared with those provided by MRI in 46 myelodysplastic children who underwent the investigation as a routine follow-up control. The MRI findings were subdivided into 4 main groups of increasing severity from 1 to 4; in some subjects, associated pathological conditions (Chiari II malformation, hydromyelia, etc.) were detected as well. Twenty-two out of the 26 patients with late neurological deterioration were operated on. Eleven of them (grades 2 to 4) underwent detethering of the fixed conus, with an improvement of their clinical picture from mild to good. On the other hand the remaining 11 subjects (grades 1 to 2) improved their condition following the correction of the associated abnormalities (malfunctioning CSF shunt: 5 cases; hydromyelia: 4 cases; symptomatic Chiari II malformation: 2 cases).

Child↗

On the treatment of subependymal giant cell astrocytomas and associated hydrocephalus in tuberous sclerosis.

Between 1980 and 1992, 10 children affected by tuberous sclerosis and intraventricular subependymal giant cell astrocytomas were surgically treated at the Institute of Neurosurgery, Section of Pediatric Neurosurgery, Catholic University of Rome. Nine patients presented with signs and/or symptoms of intracranial hypertension; in all of them the neuroradiological investigations demonstrated the presence of a space-occupying lesion in the region of the foramen of Monro with secondary ventricular dilation. In the remaining patient, a 5-month-old male infant, an intraventricular mass was discovered by means of an ultrasound examination performed after the first epileptic fit. Three patients underwent a ventriculoperitoneal CSF shunt as first surgical procedure; in 2 of them it was subsequently necessary to remove the intraventricular tumor due to the frequent occlusion of the CSF shunt device. Seven subjects underwent the direct surgical excision of the lesion. In all of them the procedure resulted in the control of the associated hydrocephalus. On the basis of such an experience, the authors conclude that the surgical removal of the intraventricular tumors in patients with tuberous sclerosis and hydrocephalus is the most appropriate treatment. In fact, in the series considered here, the removal of the tumor was not accompanied by significant morbidity, and was followed by improvement in clinical conditions. In particular, in cases in whom the occurrence of hydrocephalus was associated with a worsening in the seizure disorder, the tumor removal and the correction of intracranial hypertension was followed by a significant reduction in frequency or even by the disappearance of the seizures. However, in no case presenting with mental impairment was a significant improvement observed in mental performances as a consequence of the surgical treatment.

Adolescent↗

[Tuberous sclerosis: neuropsychological implications].

The role of neurosurgical therapy in tuberous sclerosis is still controversial; generally, a surgical procedure is performed only to treat an increase in intracranial pressure secondary to an obstructive hydrocephalus caused by a neoplasm in the region of the foramen of Monro. The role of surgical treatment in the control of epileptic fits is much more limited because of multifocal EEG epileptogenic foci due to multiple cortical lesions. Recently some authors, however, have suggested that the surgical treatment may be useful in selected patients. In the present report 9 children affected by tuberous sclerosis who were operated on in the period 1982-1992, because of increased intracranial pressure are described. The neuroradiological examination showed tumors in the region of the foramen of Monro in all the patients, with secondary obstruction in the CSF shunt circulation. Operative procedures included CSF shunts and/or giant cell astrocytoma removal. The follow-up period ranged from 1 to 14 years (mean 6 years). According to our experience, tumor excision is the most satisfying therapy, allowing the removal of the cause of the hydrocephalus as well as the possibility of histological diagnosis. A CSF shunt procedure may resolve the symptomatology, but it is often weighted by frequent malfunctioning of the CSF shunt system due to catheter obstruction secondary to abnormally high CSF protein concentration; furthermore, in some patients the insertion of the ventricular catheter may induce intraventricular bleeding when the highly vascularized tumor is accidentally punctured during the procedure of shunt insertion or in cases in which the presence of the ventricular catheter causes chronic trauma on the tumoral surface.

Adolescent↗

[Magnetic resonance and computerized tomography of posterior cranial fossa tumors in childhood. Differential diagnosis and assessment of lesion extent].

In the last thirty years much progress has been made in the treatment of brain tumors in children, thanks to modern diagnostic imaging techniques, together with neurosurgery and radiotherapy, for the diagnosis and follow-up of these lesions. MRI and paramagnetic contrast agents have revolutionized the imaging of intracranial conditions, especially in the posterior cranial fossa. The superiority of MRI over CT is well known and reported in the literature, but the adequacy of the two methods has rarely been compared. Therefore, this study was aimed at assessing and quantitating the accuracy of MRI and CT relative to two essential parameters: preoperative "histologic" diagnosis and local and distant intracranial tumor extent. In 52 selected patients the final histologic diagnosis was cerebellar medulloblastoma in 20 cases, cerebellar astrocytoma in 12 cases, brain stem glioma in 12 cases and ependymoma in 8 cases. CT allowed the correct diagnosis to be made in 25 patients (48%), with questionable findings in 21 patients (40%) and 6 misdiagnoses (12%). MRI allowed the correct diagnosis to be made in 43 patients (83%), with questionable findings in 9 patients (17%) and no misdiagnoses. MRI depicted local and distant tumor spread in 34 patients while CT showed it in 22 patients only. The analysis of the results confirmed the superiority of MRI over CT, relative to the parameters considered, i.e. histology and the assessment of tumor spread. In the clinical suspicion of brain tumors of the posterior cranial fossa in children, MRI--if available--should be the examination of choice if the patient's clinical status does not prevent its use. CT should be used only in emergency cases (acute intracranial hypertension) to identify hydrocephalus and intratumoral hemorrhages. Unenhanced CT also yields useful pieces of information for lesion "histology" and may be used to integrate MR findings in selected cases.

Adolescent↗

Patterns of failure in surgical therapy of medulloblastomas.

To better understand the relationship between the modalities of the surgical ablation of medulloblastomas--partial versus total tumor exeresis--and the pattern of tumoral relapse (local regrowth or metastases at distance), we have analyzed forty-five children affected by cerebellar medulloblastomas, observed at the Policlinico Gemelli, Catholic University Medical School, Section of Pediatric Neurosurgery, between 1981 and 1992. According to the entity of the surgical exeresis, the patients have been divided in three groups: group I, 25 children with complete neoplastic excision confirmed by post-surgical Computed Tomographic (CT) scans (total exeresis); group II, 11 patients with minimal tumor residual or infiltration in vital areas at the operation, known to the surgeon, but not visualized on post-operative CT examination (subtotal exeresis); and group III, 9 children with a residual tumoral mass, known to surgeon and visualized in the immediate postoperative CT scan and further confirmed in late CT controls (partial exeresis). Our results confirm that a significant difference in survival is observed among patients with total removal, compared to those with subtotal and partial tumor exeresis, so stressing the importance of even minimal (not detectable at the postoperative CT scan) tumoral brain stem infiltration. The analysis of our series shows a different local tumor recurrence rate in the three groups of patients, that appear to indicate an inverse relationship between degree of tumoral surgical resection and possibility of its local recurrence. However, even though a complete removal of the tumor appears associated with a minor figure of local recurrence, it does not appear to influence significantly the ability of the tumor to metastatize in the long run.

Adolescent↗

[Late clinical manifestations of hydrocephalus associated with aqueductal stenosis].

Hydrocephalus associated with aqueductal stenosis is not uncommon after the 2nd year of life. In some patients, stenosis is due to slow-growing periaqueductal tumors that can only be revealed by magnetic resonance examination. We reviewed 31 cases of children with aqueductal stenosis and hydrocephalus diagnosed after the second year of life, and operated on in the Section of Paediatric Neurosurgery of UCSC between 1982 and 1993. Eighteen cases (Group I) had nonneoplastic aqueductal stenosis, while in 13 cases (Group II) it was demonstrated a periaqueductal tumor by NMR. Intracranial hypertension was the most frequent symptom at diagnosis in both groups. In Group I mental and growth retardation were frequent, while cerebellar signs were quite common in Group II. The treatment of choice for hydrocephalus was a V-P shunt. Tumors in Group II were not directly treated, because of their benign behaviour. Subdural hematoma was a relatively common shunt complication in these patients. In 2 cases we observed an atypic complication: patients developed an altered level of consciousness, upsight paresis and distony, without any sign of increased intracranial pressure, or cerebro-spinal fluid infection. One of these patients died; we treated the second patient with L-Dopa (Sinemet 150 mg/die); he progressively improved and had a complete remission of symptoms. Surgical mortality was nil; long-term mortality was 12.5%. In 76.1% of Group II patients we have not observed any tumor growth; 50% of Group I patients has still a complete remission of preoperative symptoms (follow-up 2-12 years).

Adolescent↗

Is the slit ventricle syndrome always a slit ventricle syndrome?

The term 'slit ventricle syndrome' (SVS) refers to the occurrence of headache, vomiting, and possibly some degree of consciousness impairment in shunted hydrocephalic children in whom slit-like ventricles are seen on computerized tomography (CT) scan examination. The syndrome has been typically observed in older hydrocephalic children operated on in early infancy, even though it has also been observed occasionally in young children and adults. The clinical characteristics of the SVS, the results of the laboratory investigations and the therapeutic measures utilized in the various cases described in the literature differ so widely as to suggest that different clinical conditions are actually being described. In some cases, the diagnosis of SVS should be avoided and substituted by a correct diagnosis of CSF shunt malfunction or of disturbances unrelated to the CSF shunting therapy, e.g., childhood migraine.

Cerebral Ventricles↗

A survey of the first complication of newly implanted CSF shunt devices for the treatment of nontumoral hydrocephalus. Cooperative survey of the 1991-1992 Education Committee of the ISPN.

The results of an international multicenter study concerning the first complication of newly implanted cerebrospinal fluid shunts in nontumoral hydrocephalus are the subject of the present report. The authors have collected information on 773 cases from four continents. In particular, the following data were evaluated in relation to the general incidence of complications recorded in the first follow-up year: the patient's age at the operation, the etiology of hydrocephalus, the type of CSF shunt device used, and the modality of the surgical procedures. The overall complication rate in the series was 29%. Age and etiology of hydrocephalus appear to play a major role in influencing the complication rate; on the other hand, the choice of a specific CSF shunt device seems to be less important in this respect.

Adolescent↗

Clinical pharmacokinetics of carboplatin in children.

The present study was undertaken to evaluate in children the plasma pharmacokinetics of free carboplatin given at different doses and schedules and to evaluate the inter- and intrapatient variability and the possible influence of schedule on drug exposure. A total of 35 children (age range, 1-17 years) with malignant tumors were studied. All patients had normal renal function (creatinine clearance corrected for surface body area, above 70 ml min-1 m-2; range, 71-151 ml min-1 m-2) and none had renal involvement by malignancy. Carboplatin was given at the following doses and schedules: 175, 400, 500, and 600 mg/m2 given as as a 1-h infusion; 1,200 mg/m2 divided into equal doses and infused over 1 h on 2 consecutive days; and 875 and 1,200 mg/m2 given as a 5-day continuous infusion. A total of 57 courses were studied. Carboplatin levels in plasma ultrafiltrate (UF) samples were measured both by high-performance liquid chromatography and by atomic absorption spectrophotometry. Following a 1-h infusion, carboplatin free plasma levels decayed biphasically; the disappearance half-lives, total body clearance, and apparent volume of distribution were similar for different doses. In children with normal renal function as defined by creatinemia and blood urea nitrogen (BUN) and creatinine clearance, we found at each dose studied a limited interpatient variability of the peak plasma concentration (Cmax) and the area under the concentration-time curve (AUC) and a linear correlation between the dose and both Cmax (r = 0.95) and AUC (r = 0.97). The mean value +/- SD for the dose-normalized AUC was 13 +/- 2 min m2 l-1 (n = 57).2+ The administration schedule does not seem to influence drug exposure, since prolonged i.v. infusion or bolus administration of 1,200 mg/m2 achieved a similar AUC (13.78 +/- 2.90 and 15.05 +/- 1.44 mg ml-1 min, respectively). In the nine children studied during subsequent courses a limited interpatient variability was observed and no correlation (r = 0.035) was found between AUC and subsequent courses by a multivariate analysis of dose, AUC, and course number. The pharmacokinetic parameters were similar to those previously reported in adults; however, a weak correlation (r = 0.52, P = 0.03) between carboplatin total body clearance and creatinine clearance varying within the normal range was observed.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗