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Biomedical subjects

Massimo Caldarelli

Publications and source records attributed to Massimo Caldarelli.

18 recordsLinked to original sources
PubMed2006

Symptomatic epilepsy in children with poroencephalic cysts secondary to perinatal middle cerebral artery occlusion.

BACKGROUND: Perinatal cerebral artery occlusion is responsible for ischemic cerebral infarction leading to brain cavitation and gliosis; the territory of the middle cerebral artery is most frequently involved. The resulting poroencephalic cysts are frequently associated with hemiplegia and epilepsy; that can be managed medically in most cases, only 6-7% of them being refractory to medical treatment. This particular subset of congenitally hemiplegic children will be possible candidates for electrophysiological investigation and eventually for resective surgery. Whatever the kind of surgical treatment, surgery should be performed as soon as possible to optimize functional brain reorganization. CLINICAL MATERIAL: Twelve children with poroencephalic cysts and refractory epilepsy were studied and operated on at the Divisions of Child Neurology and Pediatric Neurosurgery, the Catholic University Medical School, Rome. The hemiparesis ranged from mild to moderate; the developmental delay was of mild degree in three cases, moderate in four cases and severe in the remaining five. Behavioral disorders were observed in patients with mental retardation; two of them also manifested autistic features. All the children presented with a severe epileptic syndrome (starting almost invariably during the first year of life); six patients presented with a West syndrome followed by symptomatic partial epilepsy; the other six presented with partial epilepsy, followed in two cases by continuous spike-waves during sleep. The electroencephalograph (EEG) recordings disclosed focal unilateral interictal epileptiform abnormalities that usually corresponded to the side of the cystic lesion; however, paroxysmal activity often spread synchronously over the contralateral hemisphere. The selection of candidates for surgical treatment was based on neuroimaging and video-EEG monitoring; in particular, we did not use invasive intraoperative neurophysiologic techniques. The convergence of neuroimaging and neurophysiologic findings guided us in performing a limited cortical excision corresponding to the malacic cortex (cyst "membrane"). RESULTS: All the patients underwent excision of the cyst wall. Careful attention was paid not to enter the body of the lateral ventricle to avoid ventriculo-subarachnoid fistulas, eventually responsible for subdural hygroma or cerebrospinal fluid leak. There was one surgery-related death secondary to disseminated intravascular coagulation, following an otherwise uneventful surgical procedure. An elevated systemic blood pressure, secondary to repeated adrenocorticotropic hormone therapy, can represent a possible concurrent factor for this event. No major complications were recorded among the remaining 11 children. Seizure control was excellent in all the 11 survivors in the early postoperative period. Two children presented a relapse of seizures, after an initial improvement, respectively 3 and 4 years after the operation. These two children underwent subsequently a functional hemispherectomy. Overall, seizure outcome was excellent in all the cases. Seven patients (including the two who underwent functional hemispherectomy) are seizure-free (Engel's class Ia), and in one of them antiepileptic therapy has been weaned. In the remaining five children, seizures are sporadic and definitely improved (Engel's class II). An improvement of developmental delay, in particular of cognitive competence, was registered in 8 out of the 11 patients. Two of the four severely retarded children, who also presented behavioral abnormalities, did not show any cognitive improvement, whereas some mild improvement of their basal abilities was demonstrated in the other two. All the remaining children, even though maintaining a moderate retardation, definitely improved their abilities; in particular, one of them reached an almost borderline level. The three patients with unchanged neurodevelopmental delay presented also persistent seizures. On the other hand, two children with persistent seizures presented neurodevelopmental improvement. CONCLUSIONS: Simple surgical excision of the cyst "membrane" of epileptogenic poroencephalic cysts can represent an excellent means to control epilepsy in affected children. However, postoperative seizure persistence and late recurrences, although rare, do not allow to exclude that hemispherectomy or partial resections (based on electrocorticography findings) might represent the good answer at least in some cases.

Adolescent↗
PubMed2006

Unusual presentation of intradural endodermal cysts in young children under 2 years of age. Report of two cases.

INTRODUCTION: Spinal endodermal cysts are congenital maldevelopmental lesions usually presenting in early adulthood. Whereas the diagnosis is rare in newborns and it is usually led by cardiopulmonary distress or other associated malformations, no characteristic clinical findings associated with these entities have been described in older patients where spinal endodermal cysts usually behave as intraspinal space occupying lesions. DISCUSSION: The authors report on two children under 2 years of age affected by cervical endodermal cysts that presented with a history of acute motor deficit following a laryngitis. The hypothesis about the pathogenesis is that the upper respiratory tract infection might have stimulated the secretion of the cyst lining, inducing growth of the cyst and the sudden appearance of the motor deficit. Because of the apparent lack of relation between the benign laryngitis and the motor deficit, the occurrence of such an association might have been underestimated in some of the previously reported cases.

Ataxia↗
PubMed2006

Homocysteine, folate, lipid profile and MTHFR genotype and disability in children with myelomeningocele.

STUDY DESIGN: We performed a cross-sectional study in myelomeningocele children. OBJECTIVE: To investigate plasma total homocysteine, folate, lipid profile, 5,10- metylenetetrahydrofolate reductase genotype (MTHFR) and disability. MATERIALS AND METHODS: Sixty patients aged between 2 and 14 years with myelomeningocele (18 ambulatory and 42 non-ambulatory) and 150 healthy children of same age, are investigated for lipid profile, homocysteine concentration and for the determination of MTHFR genotype. RESULTS: Plasma homocysteine concentrations were significantly higher in myelomeningocele children than in the control group. In myelomeningocele female group, there were higher levels of total cholesterol and very-low-density lipoprotein cholesterol with respect to the control group. Myelomeningocele children walking with tutorial aid showed triglyceride levels significantly lower than those observed in myelomeningocele non-walking children. CONCLUSION: Disability, insulin uptake, lipid, homocysteine, hormones plasma levels, and genetic factors such as allelic variants of MTHFR are possible for cardiovascular disease in myelomeningocele children. This study highlights the importance of a continuous surveillance of any changes in the lipid profile that should be corrected as soon as possible. Constant physical activity necessary to increase HDL levels should be planned in all susceptible children. Nonetheless, further investigations are necessary to identify new homocysteine susceptible genes for prevention of early atherosclerosis and consequent cardiovascular disease.

Adolescent↗
PubMed2006

The role of endoscopic choroid plexus coagulation in the surgical management of bilateral choroid plexuses hyperplasia.

BACKGROUND: Bilateral choroid plexus hyperplasia is a rare condition often associated with cerebrospinal fluid (CSF) overproduction. CSF overproduction is usually so high that the placement of a CSF ventriculoperitoneal shunt almost always results in progressive ascites leading to the necessity of removing the inserted shunt device. A direct surgical treatment of the hyperplastic choroid plexuses is then mandatory. Endoscopic coagulation of the choroid plexuses has been recently proposed as an alternative to open surgical plexectomy. However, the effectiveness of the procedure in controlling CSF overproduction is still debated. TECHNIQUE: We report a case of bilateral choroid plexus hyperplasia in which an extensive bilateral endoscopic coagulation of the choroid plexuses failed to reduce the CSF formation rate sufficiently. A one-stage bilateral open surgical plexectomy was performed. RESULTS: The procedure succeeded to control CSF overproduction. Intraoperative blood loss during the surgical removal of the choroid plexuses was significantly reduced due to the previous coagulation of their surface. CONCLUSION: On these grounds, we suggest that endoscopic choroid plexuses coagulation, even when failing to normalize CSF production, may still be considered as a valid adjuvant procedure in the management of this condition.

Adult↗
PubMed2006

Late closure of the stoma by spreading of a periaqueductal glioma: an unusual failure of endoscopic third ventriculostomy. Case report.

The late failure of endoscopic third ventriculostomies (ETVs) has been described as a consequence of scarring phenomena involving the third ventricular floor. Although the possibility of closure of the stoma by intraventricular tumor seeding has been hypothesized, no case of ETV failure caused by tumor diffusion has been reported in the literature. The authors report on a 19-year-old patient, affected by obstructive hydrocephalus associated with a periaqueductal pilocytic astrocytoma, who underwent late secondary lesional obstruction of an ETV performed at diagnosis. The authors suggest tumor extension along the diencephalic neural pathways as the possible cause of ETV failure in this patient.

Adult↗
PubMed2006

Surgical management of craniopharyngiomas--experience with a pediatric series.

Because of their peculiar location and the possible related postoperative sequelae, craniopharyngiomas usually present difficult management, especially in children. The authors report on 20 years experience with a pediatric series. Fifty-four consecutive children were operated on. The goal of surgery was total tumor removal. When pre-operative neuroimaging and/ or intra-operative findings suggested more cautious behavior, subtotal/partial excision was performed, followed by radiotherapy only in selected cases. Gross total mass removal was obtained in 78% of patients, subtotal in 17%, and partial in 5%. Recurrence rate was 7% after total removal and 50% after subtotal/ partial excision. Surgical mortality accounted for 3.7% (11% in case of re-operation). After 9 years median follow-up, up to 60% of the surviving patients rely on hormone replacement, 40% present diabetes insipidus, and 18% are obese. All but two patients enjoy a normal social life. Total surgical removal of craniopharyngiomas should be attempted in children whenever possible in order to minimize the risk of recurrence. In case of huge tumors involving the hypothalamus, a more conservative resection should be performed, followed by a 'wait and see' policy or possible radiotherapy.

Adolescent↗
PubMed2004

Diagnosis of Chiari I malformation and related syringomyelia: radiological and neurophysiological studies.

INTRODUCTION: The diagnosis of Chiari I malformation relies mainly on the anatomical demonstration of the abnormal position of the cerebellar tonsils below the foramen magnum, and of the associated bony anomalies of the posterior cranial fossa and craniocervical junction, as well as of the eventually-associated spinal cord cavitations. Consequently, the neuroradiological work-up plays a fundamental role in the definition of the malformation and in the follow-up of operated patients. REVIEW: The authors review the pertinent literature on the neuroradiology of the Chiari I malformation, with special regard to MRI, with the aim of providing the reader with an updated instrument for its diagnosis.

Arnold-Chiari Malformation↗
PubMed2004

Massive congenital intracranial teratoma: perioperative coagulation impairment.

Massive congenital intracranial teratoma is a rare neoplasm with a poor prognosis. Surgery may be curative only if complete resection can ben obtained. Several single case reports have been published in the pediatric literature, mostly focusing on prenatal diagnosis. The authors describe six patients with congenital intracranial teratoma treated at their institution in the past decade. Perioperatively, a marked hemostatic derangement was observed in three of them undergoing surgery: the pathophysiology of this complication is discussed. The surgical indication itself represents an ethical dilemma when treating a large intracranial tumor with partial destruction and replacement of brain structures.

Adult↗
PubMed2004

Intracranial midline dermoid and epidermoid cysts in children.

OBJECT: Dermoid and epidermoid cysts are rare space-occupying lesions of the central nervous system. Although characterized by a slow growth rate, they are often associated with serious complications. Surgery is the only effective treatment, and radical resection of the entire cyst, whenever possible, generally succeeds in achieving a cure. Authors of large series have only occasionally reported on pediatric cases, and these reports often lack a specific analysis of those cerebral midline lesions. METHODS: The authors report on the treatment of 19 patients (16 with intracranial intradural dermoid and three with epidermoid cysts located along the cerebral midline). All patients underwent surgery at the Catholic University Medical School in Rome. The patients ranged in age from 3 months to 16 years. Nine cysts were located in the posterior cranial fossa, six in the frontobasal subarachnoid spaces, two in the third ventricle, and two in the quadrigeminal plate cistern. In the cases presenting with dermal sinus tracts, attempts at resecting the dermoid cyst and the associated dermal sinus were made in a single stage to achieve an en bloc removal. In the cases without dermal sinus tracts, and in the three with epidermoid cysts, a standard craniotomy was performed to reach the lesion. There were no surgery-related deaths, and the morbidity rate was low. Total and subtotal resections were achieved in 15 and four cases, respectively; however, regrowth of the residual tumor was observed only in two of them. After repeated resection, tumor progression has not been observed. At a mean follow-up period of 92.7 months, excellent clinical results were achieved in 18 patients. CONCLUSIONS: Because surgery is the only effective treatment modality for these lesions, radical resection should be performed in all cases to avoid tumor recurrence; however, because the cyst capsule can adhere firmly to vital structures and attempts at its radical removal can be dangerous, subtotal resection may be a wise option in selected cases.

Adolescent↗
PubMed2003

Pharmacokinetics of temozolomide given three times a day in pediatric and adult patients.

PURPOSE: To characterize and compare pharmacokinetic parameters in children and adults treated with temozolomide (TMZ) administered for 5 days in three doses daily, and to evaluate the possible relationship between AUC values and hematologic toxicity. METHODS: TMZ pharmacokinetic parameters were characterized in pediatric and adult patients with primary central nervous system tumors treated with doses ranging from 120 to 200 mg/m2 per day, divided into three doses daily for 5 days. Plasma levels were measured over 8 h following oral administration in a fasting state. A total of 40 courses were studied in 22 children (mean age 10 years, range 3-16 years) and in 8 adults (mean age 30 years, range 19-54 years). RESULTS: In all patients, a linear relationship was found between systemic exposure (AUC) and increasing doses of TMZ. Time to peak concentration, elimination half-life, apparent clearance and volume of distribution were not related to TMZ dose. No differences were seen among TMZ C(max), t(1/2), V(d) or CL/F in children compared with adults. Intra- and interpatient variability of systemic exposure were limited in both children and adults. No statistically significant differences were found between the AUCs of children who experienced grade 4 hematologic toxicity and children who did not. CONCLUSIONS: No difference appears to exist between pharmacokinetic parameters in adults and children when TMZ is administered in three doses daily. Hematologic toxicity was not related to TMZ AUC. AUC measurement does not appear to be of any use in optimizing TMZ treatment.

Administration, Oral↗
PubMed2003

Prolonged ICP monitoring in Sylvian arachnoid cysts.

BACKGROUND: The decision making process for patients with Sylvian fissure arachnoid cysts still represents a challenge for the neurosurgeon. A high percentage of patients are indeed asymptomatic, despite neuroimaging signs of apparently increased intracranial pressure (ICP). The present study was conducted to evaluate the usefulness of prolonged ICP recording in the preoperative work-up. METHODS: Eleven children (10 M/1 F) harboring temporal arachnoid cysts were investigated (mean age: 5.9 years). According to Galassi classification they were subdivided in three groups. Group I was made up of three patients with Type I cysts; Group II comprised six children with Type II cysts; Group III consisted of two children with Type III cysts. An extensible silicone microprocessor (Codman), developed for continuous ICP recording, was implanted intraparenchymally, adjacent to the major extension of the cyst. The collected information was cable unloaded to a PC and stored. All the patients underwent a minimum of 48 hours to a maximum of 72 hours of ICP continuous check. In resting conditions, 10 mm Hg was arbitrarily chosen as the upper normal limit of ICP, and the patients were classified according to the percentile distribution of their ICP daily and nightly pressure values. RESULTS: ICP recordings were in the normal range in all the three children with Type I cysts (49-86%< 10 mm Hg during the entire recording), despite the fact that two of them were apparently symptomatic. Three of the six children with a Type II temporal cyst had elevated ICP values (69-99%> 10 mm Hg), even though they had an incidental (1 case) or prenatal (2 cases) diagnosis. The remaining three patients had normal ICP values for more than 70% of the recording time period. The two patients with type III temporal arachnoid cysts had almost constantly abnormal ICP values (95-99%> 10 mm Hg). Five patients were operated; in four of them the surgical indication was based on ICP recordings (two of the three children with a Type II cyst and increased ICP and the two patients with a Type III cyst). The last child, harboring a Type I cyst, was operated upon parents' request, as a preventive measure. At a mean follow-up of 9.3 months all the patients operated on are in excellent clinical condition; one of them (Type II cyst) initially undergoing a craniotomy and cystic membrane excision required a subduro-peritoneal shunt implant 3 months after surgery for a symptomatic subdural hygroma on the side of the cyst. Postoperative computed tomography showed signs of brain expansion in the two patients operated on for a Type III cyst, and in the patient operated on for a Type I cyst. CONCLUSIONS: With the limit of the relatively small series presented here, prolonged ICP recording appeared to be an important preoperative tool to rule out the necessity of operating on children with Type I cysts. Furthermore, the study confirmed that an increase of the ICP is almost constantly present in children with Type III cysts. In cases of Type II lesions, the study results were less discriminating, though the ICP monitoring contributed in identifying those patients in whom surgery was indicated despite the absence of symptoms.

Adolescent↗
PubMed2002

Ectopic recurrences of craniopharyngioma.

BACKGROUND: Ectopic recurrences of craniopharyngioma after total surgical removal are a rare event. Two possible mechanisms of seeding have been proposed: the dissemination of tumoral cells during the surgical procedure would account for tumor recurrence along the surgical path, whereas the migration of tumoral cells through the subarachnoid or Virchow-Robin spaces would explain ectopic recurrences far from the surgical bed or within the brain parenchyma. CASE REPORT: We report a unique case of metastatic craniopharyngioma recurring in a child after different intervals in which both the mechanisms could have taken place. The first ectopic seeding arose along the operative track of the primary surgical procedure in the frontal region while the second involved the deep cerebral nuclei and the brain stem. OUTCOME: The first recurrence was successfully operated upon. The following recurrences were not operated upon but remained stable for the last 4 years, thus showing dissociation between the capacity of seeding and invasivity.

Brain Neoplasms↗
PubMed2002

Chiari type III malformation.

BACKGROUND: Chiari type III is the rarest of the Chiari malformations, and it is usually associated with a dismal prognosis in terms of early death or severe disability in long-term survivors. Surgical treatment usually consists of primary closure of the occipital/cervical encephalocele, followed by delayed CSF shunting for the associated hydrocephalus. METHODS: In our case, we introduced a technical variant consisting in primary external drainage from the malformative sac, followed by closure of the encephalocele and, eventually, by CSF shunting.

Arnold-Chiari Malformation↗