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Biomedical subjects

C Di Rocco

Publications and source records attributed to C Di Rocco.

At least 55 records · Page 3Linked to original sources
PubMed2001

Intra-axial dermoid/epidermoid tumors of the brainstem in children.

BACKGROUND: Epidermoid and dermoid cysts are rare intracranial space-occupying lesions, which account for about 1% of all intracranial tumors; they are even rarer in the pediatric age group. Among the various locations, that inside the brain stem is quite exceptional (only 12 cases reported to date in the literature). We report two additional cases occurring in children, underlining their clinical characteristics, the difficulties faced in the diagnostic work-up, and the surgical treatment adopted (which consisted of removal of the cyst contents and partial resection of the cyst membrane, because of its adherence to the surrounding nervous and vascular structures). METHODS: We report two cases of intraaxial dermoid/epidermoid cysts observed within the last 5 years. Both patients complained of cervico-nuchal pain, with a remittent/intermittent character in one of them; this patient also exhibited transient 6th and 7th cranial nerve deficit. One lesion was approached through the floor of the fourth ventricle, the second one through the ventrolateral aspect of the brainstem. RESULTS: Subtotal tumor resection was achieved in both cases. Both patients had a smooth postoperative course without permanent neurological deficits. In the first patient residual tumor remains stable on follow-up MRI. In the second one, early tumor regrowth necessitated a second operation, after which the residual tumor has remained stable. CONCLUSIONS: Based on our experience we suggest that a cautious surgical approach ("conservative" resection) to these lesions is the best choice. In fact, attempts at radical removal carry unacceptably high morbidity and mortality rates.

Adolescent↗
PubMed2001

Dendritic anomalies in a freezing model of microgyria: a parametric study.

Despite easier recognition of focal developmental cortical anomalies with modern morphological and functional imaging techniques, mechanisms leading to refractory epilepsy are still poorly understood. Recent experimental studies have shown that not only the lesioned cortex, but also the apparently normal adjacent cortex undergoes morphological changes and alterations of its neuronal connections. To further investigate the modifications of the cortex surrounding a focal maldevelopmental lesion, we applied a freezing insult to newborn rat cortex, resulting in a focal cortical malformation similar to human microgyria. Corticocortical associative neurons were retrogradely labeled in a Golgi-like fashion using biotinylated dextran amine combined with NMDA. In addition to previously reported alterations, a considerable spine loss was observed in the basal dendrites of neurons located in the eulaminated cortex adjacent to the lesion. These data demonstrate profound maldevelopmental alterations which are not limited to the macroscopically abnormal lesion, but extend at a cellular level to the surrounding cortex. The observed alterations may contribute to the increased excitability of the cortex harboring a microgyric lesion as well as the frequently associated cognitive impairment.

Animals↗
PubMed2001

The influence of hemocoagulative disorders on the outcome of children with head injury.

BACKGROUND: Although disseminated intravascular coagulation (DIC) and other hemocoagulative abnormalities are severe complications of head injury, their effect on clinical outcome remains unclear, particularly among children. OBJECTIVES: To evaluate the frequency of hemocoagulative abnormalities and their influence on outcome among children with head injury. STUDY DESIGN: We conducted a prospective observational study among 60 children with head injury, immediately evaluating severity of head injury (Glasgow Coma Scale, GCS); cerebral axial tomography; prothrombin time; activated partial thromboplastin time (aPTT); fibrinogen level; concentration of fibrin-fibrinogen degradation products (FDP), and platelet count. Two months after injury, we applied the Glasgow Outcome Score (GOS). Associations with GOS were evaluated using univariate and multivariate logistic models. RESULTS: Among children with severe head injury, 22.2% (6/27) developed DIC, all of whom died and had shown severe brain edema. Among those with severe head injury yet without DIC, the mortality was only 14.2%. A low GOS was significantly and independently associated with a low GCS, multiple trauma, delayed aPTT, low fibrinogen level, elevated FDP and low platelet count. Brain edema was also associated with a low GOS, though not significantly. CONCLUSIONS: In addition to GCS, type of trauma, type of brain lesion and certain coagulation abnormalities are predictors of GOS.

Blood Coagulation Disorders↗
PubMed2001

Surgical treatment of moyamoya disease in pediatric age.

BACKGROUND: Moyamoya is a progressive occlusive cerebrovascular disease which affects the distal internal carotid artery and its main branches, and is characterized, from a clinical point of view, by a variety of ischemic manifestations ranging from transient ischemic attacks (TIA) to stroke. Although considered until recently as almost specific of Japanese population, it can also affect individuals of different origin, and there is an increasing interest for this rare disease in Western medical literature. Nonetheless papers dealing with moyamoya disease in pediatric age are still very rare and almost exclusively limited to isolated case reports. These considerations prompted us to review our experience with the diagnosis and treatment of such unusual disease. METHODS: Nine children (seven months to nine years of age) presented with clinical manifestations of occlusive cerebrovascular disease (TIAs or stroke), epilepsy, and/or psychomotor retardation. Neuroradiological investigation, and in particular cerebral angiography, demonstrated the classical pattern of moyamoya disease. Eight of these children underwent cerebral revascularization utilizing the encephalomyosynangiosis (EMS) technique, with a total of 14 operations (six on both sides, two on one side). RESULTS: There was no mortality neither major morbidity related to surgery. One patient with renovascular hypertension, died at home approximately six months after a successful operation, probably from cerebral hemorrhage. In all the cases postoperative angiography revealed a marked improvement in cerebral circulation. However, in spite of the apparently normalized cerebral blood flow, clinical manifestations were affected less significantly. In fact, there was only minimal if any improvement in children operated on with an already established hemiplegia; whereas improvement in motor ability was obtained in those presenting with hemiparesis or TIAs, as well as in epilepsy and psychomotor retardation. CONCLUSIONS: On the grounds of our results we conclude that cerebral revascularization (by means of a simple operation like the EMS) in children with moyamoya disease is worthwhile, provided that the operation be performed before irreversible cerebral damage is established; however, even under these circumstances the operation can at least stop the progression of the disease.

Age Factors↗
PubMed2000

Organization of cortico-cortical associative projections in a rat model of microgyria.

Microgyria was experimentally induced by focal freezing lesions of the frontal cortex in newborn rats. Adult microgyric animals received cortical injections of biotinylated dextran amine combined with NMDA, in order to obtain a Golgi-like retrograde labeling of cortico-cortical association neurons. Injections were performed either rostrally or caudally to the microgyric lesion. Results demonstrate that long-range association projections traveling across the zone of the microgyric lesion arise mainly from infragranular layers. In normal animals the same projections originate both from supragranular and infragranular layers. The analysis of single basal dendrites of layer 2/3 in microgyric animals demonstrates a simplified branching pattern, with a number of end points lower than in control animals. Potential implications for microgyria-associated epilepsy are discussed.

Animals↗
PubMed2000

Early post-traumatic seizures in children with head injury.

Post-traumatic seizures (PTS) can be a serious complication of head injury, because they can cause secondary brain damage through increased metabolic requirements, raised intracranial pressure, cerebral hypoxia, and/or excessive release of neurotransmitters. In children, early PTS are more frequent than late ones. In this retrospective study we conducted an epidemiological analysis and tried to identify potential risk factors for the onset of early PTS in children hospitalized for head injury in our Paediatric Intensive Care Unit. The severity of injury was assessed using the Glasgow Coma Scale (GCS), while the outcome of traumatized children was defined using the Glasgow Outcome Score (GOS). Early PTS were diagnosed in 15 out of the 125 children hospitalized (12%). Most of the children (73.3%), developed seizures within 24 h of the trauma (immediate PTS). Among the risk factors, a very important role was played by the severity of the injury; in fact, the incidence of early PTS among patients with GCS < or = 8 was ten times greater than that among children with GCS 13-15. Other risk factors that significantly influenced the onset of early PTS, were age (60% of children with early PTS were less than 3 years old) and severe cerebral edema. Overall, children with early PTS had a worse outcome than the other patients. In fact, 53% had a GOS of < or = 3 compared to 19.1% of those without early PTS (P<001). In particular, considering children with severe head injury, 80% of those with early PTS had a GOS of < or = 3, compared to 41% of those without early PTS (P<0.05). In conclusion, PTS can be a serious complication of head injury in children, because they can worsen secondary brain damage. Appropriate management of head-trauma patients must include suitable and immediate prophylaxis with anti-epileptic drugs.

Cerebral Hemorrhage↗
PubMed2000

Preemptive analgesia with tramadol and fentanyl in pediatric neurosurgery.

Preemptive analgesia is based on administration of an analgesic before a painful stimulus generates, so as to prevent the subsequent rebound mechanism. Tissue injury results in disruption of the processing mechanisms of noxious stimuli afferent to the CNS (central nervous system) by way of an increase of inputs in the spinal cord. These reactions may be reduced by the administration of opioids. Few studies on preemptive analgesia with opioids in children are available, and none of them is concerned with pediatric neurosurgery. Tramadol and fentanyl are synthetic opioids which are relatively new and act through the activation of pain-inhibitory mechanisms. We conducted a randomized, prospective trial on the preemptive effects in children of these two analgesic drugs, administered according to three different protocols: tramadol as a bolus (1 mg/kg); tramadol by continuous infusion (150 microg/kg per h); fentanyl by continuous infusion (2 microg/kg per h). In all, 42 children undergoing major neurosurgical operations were enrolled in the study, 14 in each treatment group. Each treatment was started at the induction of general anesthesia and continued throughout the entire duration of the operation. The postoperative pain evaluation was conducted in the Pediatric Intensive Care Unit at the end of the surgical operations and involved comparison of any changes in behavioral (AFS scale and CHEOPS score) and hemodynamic (heart rate, respiratory rate, systolic and diastolic arterial pressure, oxygen saturation, O(2) and CO(2) partial pressure) parameters. Only 2 children, both in group A, needed further drug administration postoperatively. No significant side effects were noticed in any of the three groups, except that in group A there was a higher incidence of nausea and vomiting. Tramadol efficacy seems to be better when it is administered in continuous infusion; this treatment modality also leads to fewer adverse effects. Fentanyl, in contrast, proved to be superior to tramadol in the treatment of postoperative pain. In conclusion, preemptive analgesia is a valid technique for the treatment of acute pain in children undergoing major neurosurgical operations.

Adolescent↗
PubMed2000

Cerebral arteriovenous malformations in children.

In spite of their congenital origin, only 18-20% of cerebral AVMs are diagnosed during infancy and childhood. Intracranial haemorrhage is the presenting clinical manifestation in 75-80% of paediatric patients and is associated with a high morbidity and mortality. The natural history of untreated cerebral AVMs in children is worse than in adults, in relation to a longer life expectation, a higher annual risk of AVM bleeding (3.2% vs. 2.2%) and a higher incidence of posterior fossa and basal ganglia AVMs, most of which present with massive haemorrhages. The surgical excision remains the treatment of choice for parenchymal AVMs in children; AVM complete removal is currently achieved in 70-90% of the patients. With the advent of new agents for endovascular management, preoperative AVM embolization has further improved surgical results. Stereotactic radiosurgery appears to be a successful treatment option in small or moderate sized AVMs. Recent studies have demonstrated low complication rates with this technique in paediatric patients. We reviewed our experience with 37 paediatric AVMs treated at the Section of Paediatric Neurosurgery of the Catholic University of Rome between 1980 and 1997. Twenty-three patients underwent surgery as the only treatment modality; endovascular embolization was combined with the surgical treatment in a further four cases. Radiosurgery was utilized as the only treatment in three patients and in combination with other techniques in an other three children (with surgery in one case and with AVM embolization in the remaining two subjects). No treatment was carried out in three patients because of excessively critical condition on admission; endovascular embolization failed in a further patient because of the anatomical complexity of the malformation. Previous studies have demonstrated a quite strict correlation between AVM complexity based on Spetzler and Martin's grading system and patients outcome. A less direct relationship has been observed in the present study. In our experience the factors which were more closely predictive of patients' outcome were the occurrence of an AVM bleeding and the neurological status on admission. In spite of the low number of cases in the single subsets of patients this study seems to support the role of AVM embolization and radiosurgery as effective adjuvant techniques in the management of cerebral AVMs in children.

Adolescent↗
PubMed2000

Surgical treatment of temporal tumors associated with epilepsy in children.

Seizures are a frequent sign of cerebral supratentorial tumors in children, especially when the location of the neoplasm is the temporal lobe. We report a series of 37 pediatric patients with temporal epileptogenic tumors. They represent 80.4% of children affected by temporal neoplasms, confirming the high incidence of seizures when neoplasms are located in this cerebral area. There was a slight male predominance. Epilepsy was the first symptom in all the patients of our series, as well as the only clinical manifestation present until surgery in 62% of patients. In the remaining children, hemiparesis, intracranial hypertension, psychosocial or neuropsychological disabilities, and delayed milestones arose before diagnosis and surgical treatment. The most frequent type of seizures was the partial complex (56%), but simple partial or generalized fits, as well as more than one type of seizures, were observed. The medial temporal structures were those more commonly involved, although seizures were observed also in cases where they were spared by tumors located exclusively in the temporal neocortex. On histology, most of the tumors showed a benign phenotype. Tumor resection was complete in 60% of cases; the excision of the tumor was incomplete in subjects whose lesion involved surgically inaccessible cerebral regions, as language areas, insular structures, and basal nuclei. As for epilepsy, 26 among the 32 long-term survivors can be classified in class I of Engel's classification; 4 of them did not receive any antiepileptic treatment. Four patients showed a significant reduction in the frequency of their fits. In 2 subjects, only the frequency of seizures was minimally reduced after tumor excision; in both, a partial removal of their tumor was performed. The relationship among the results on epilepsy and the extent of surgery removal of the tumor, brain tissue removal if any, frequency of seizures in the preoperative period and the time interval between the first epileptic manifestation and surgery show that the most significant prognostic element predictive of a good control of seizures is radical resection of the tumor.

Adolescent↗
PubMed2000

Safety and efficacy of remifentanil in craniosynostosis repair in children less than 1 year old.

Few studies on analgesia with remifentanil (Rf) in children are available, and there are no data on the use of this drug in pediatric neurosurgery. Rf is a new mu-receptor opioid agonist, acting through the activation of pain inhibitory mechanisms. We conducted a prospective trial on the analgesic effects of Rf in 20 children less than 1 year of age undergoing a neurosurgical procedure for craniosynostosis repair. Rf was administered at doses of 0.25 microgram/kg/min, by continuous infusion, 1 h after admission to the pediatric intensive care unit (PICU). The treatment was prolonged for 12 h after the operation. The postoperative pain was evaluated in our PICU, comparing the changing of behavioral (AFS and CHEOPS score) and hemodynamic (heart rate, respiratory rate, systolic and diastolic blood pressure, oxygen saturation, O(2) and CO(2) partial pressure) parameters, before and after treatment with Rf. This drug showed a satisfactory pain control in all the children treated. No significant side effects were noticed, except for one episode of urinary retention. In conclusion, Rf appears to be safe and effective for the treatment of acute pain in the very young child submitted to craniosynostosis repair.

Age Factors↗
PubMed2000

Hemimegalencephaly and intractable epilepsy: complications of hemispherectomy and their correlations with the surgical technique. A report on 15 cases.

Hemispherectomy is required in most cases of hemimegalencephaly in order to control epilepsy refractory to medical treatment. Although there is a general agreement on the effectiveness of the procedure in controlling the seizure disorder, the choice of the surgical technique is still a subject of debate. In particular, anatomical hemispherectomy is blamed to be associated with a higher incidence of surgical complications, namely hydrocephalus and hemosiderosis, than other less ablative operations such as functional hemispherectomies. A series of 15 children with hemimegalencephaly, who had undergone anatomical hemispherectomy (11 cases), functional hemispherectomy (2 cases), and hemidecortication (2 cases) at the Pediatric Neurosurgery Section, Catholic University Medical School, Rome, is reported. Twelve of these patients presented with one or more complications in their postoperative course. Temporary complications, which resolved spontaneously or following medical therapy, included fever, wound breakdown, worsening of preoperative motor deficit, unilateral third cranial nerve deficit, dystonia, and anemia. In 8 patients, postoperative complications led to a second surgical procedure. A CSF shunt was necessary in 5 children, to control a secondary hydrocephalus. Two subjects underwent a toilette of the residual cavity because of persisting chemical abnormalities in CSF parameters. In a child a cranioplasty procedure was necessary as a consequence of an infection of the hemicranial bone flap. There was no apparent correlation between the rate and the type of complications with a specific surgical procedure. On the other hand, the age factor appeared to play an important role in the occurrence of secondary hydrocephalus, as all 5 children with this complication were less than 9 months old at the time of the hemispherectomy. Surgical mortality was nil in this series.

Brain↗
PubMed1999

Fibroblast growth factor receptor mutational screening in newborns affected by metopic synostosis.

A number of craniosynostotic disorders have recently been ascribed to mutations in genes coding for the fibroblast growth factor receptors(FGFRs). The common feature of these FGFR-associated conditions is the unilateral or bilateral premature ossification of the coronal suture. One distinct craniosynostotic condition is trigonocephaly, which results from the premature fusion of the metopic suture. Trigonocephaly mostly occurs as isolated cranial defect; however, the premature closure of the metopic suture may represent a feature of more complex craniosynostotic conditions in which a progressive involvement of other cranial sutures with age is observed. The possible involvement of mutated FGFRs in trigonocephaly was investigated in nine newborns affected by isolated premature synostosis of the metopic suture. All except one of these cases carried no mutations in the FGFR1-3 domains indicated as hot spots for craniosynostosis-associated mutations. A T(978)C transition in the FGFR2 exon IIIa was found in a patient who had a phenotype that apparently fitted the trigonocephalic condition at birth, but showed additional facial anomalies, which worsened progressively with age towards a Crouzon-like profile. The present finding points out the importance, from both diagnostic and prognostic points of view, of early FGFR mutational screening in craniosynostotic conditions, even in forms that apparently do not involve closure of the coronal suture at birth.

Craniofacial Abnormalities↗
PubMed1999

Organization of callosal connections in rats with experimentally induced microgyria.

The anatomical organization of projections from the microgyric cortex to the contralateral hemisphere through the corpus callosum has been studied in the rat. Microgyria was induced on the first postnatal day by a freezing injury. Once adult, animals received injections of horseradish peroxidase in the cortex contralateral to the lesion. Callosally projecting neurons located in the normally layered cortex adjoining the focal lesion showed a different laminar distribution from that observed in control animals. The highest percentage of callosal neurons was found in layer 6 in lesioned animals, whereas layers 2/3 and 5 gave rise to most of interhemispheric projections in controls. Possible mechanisms leading to the establishment of aberrant callosal connections and potential implications for the human pathology are discussed.

Animals↗
PubMed1999

Blood salvage in craniosynostosis surgery.

In the history of surgery, every single step forward in the development of complex surgical techniques has been sustained by the acquisition of more reliable and effective methods for controlling hemostasis. For many years, in fact, uncontrolled hemorrhages, together with infections, represented the most deadly hazard of surgical procedures. In the last century, technical advances in surgical hardware and homologous blood transfusions have been utilized to counteract operative and postoperative anemia and hypovolemia. At the end of this millennium, however, new revelations about the infective and noninfective risks of allogeneic blood transfusions have led to a new acceleration in patients' and physicians' demands for autologous transfusions and more efficient blood conservation techniques. Specific surgical protocols, based on the preoperative administration of r-HuEPO, preoperative autologous blood donation, acute preoperative normovolemic hemodilution and intraoperative blood salvage techniques, have been designed by pediatric neurosurgeons to minimize the exposure of patients affected by craniosynostoses to allogeneic blood and blood components even when the surgical procedure is to be realized at an early age. In spite of the evolution expected in this area in the immediate future, the implementation of these blood concentration methodologies may prove to be highly effective only when associated with a concerned attitude of the surgeon toward blood-sparing intraoperative strategies.

Blood Substitutes↗
PubMed1999

Decreased proliferation and altered differentiation in osteoblasts from genetically and clinically distinct craniosynostotic disorders.

Craniosynostoses are a heterogeneous group of disorders characterized by premature fusion of cranial sutures. Mutations in fibroblast growth factor receptors (FGFRs) have been associated with a number of such conditions. Nevertheless, the cellular mechanism(s) involved remain unknown. We analyzed cell proliferation and differentiation in osteoblasts obtained from patients with three genetically and clinically distinct craniosynostoses: Pfeiffer syndrome carrying the FGFR2 C342R substitution, Apert syndrome with FGFR2 P253R change, and a nonsyndromic craniosynostosis without FGFR canonic mutations, as compared with control osteoblasts. Osteoblasts from craniosynostotic patients exhibited a lower proliferation rate than control osteoblasts. P253R and nonsyndromic craniosynostosis osteoblasts showed a marked differentiated phenotype, characterized by high alkaline phosphatase activity, increased mineralization and expression of noncollagenous matrix proteins, associated with high expression and activation of protein kinase Calpha and protein kinase Cepsilon isoenzymes. By contrast, the low proliferation rate of C342R osteoblasts was not associated with a differentiated phenotype. Although they showed higher alkaline phosphatase activity than control, C342R osteoblasts failed to mineralize and expressed low levels of osteopontin and osteonectin and high protein kinase Czeta levels. Stimulation of proliferation and inhibition of differentiation were observed in all cultures on FGF2 treatment. Our results suggest that an anticipated proliferative/differentiative switch, associated with alterations of the FGFR transduction pathways, could be the causative common feature in craniosynostosis and that mutations in distinct FGFR2 domains are associated with an in vitro heterogeneous differentiative phenotype.

Acrocephalosyndactylia↗
PubMed1999

Progressive disease in children with medulloblastoma/PNET during preradiation chemotherapy.

The overall prognosis in children with medulloblastoma/PNET has not significantly improved over the past decade. Intensive neoadjuvant chemotherapy has not yet adequately explored. We evaluated the short-term clinical results of an intensive chemotherapy regimen in high risk children with newly diagnosed MB/PNET, after surgery and before radiation. Twelve previously untreated patients with high-risk medulloblastoma/PNET, according to Chang's classification, were treated with the following chemotherapy regimen: high dose carboplatin 600 mg/m2/day on days 1 and 2; the same course was administered 4 weeks later. One month later, high dose cyclophosphamide 2 g/m2/day on days 1 and 2, followed by an identical course 4 weeks later. Vincristine 1, 5 mg/m2 i.v. was given on the first day of each course. Systemic evaluation of the disease included imaging of the entire neuraxis, including MRI of the entire spine. Out of 12 enrolled, 7 patients were able to be evaluated for a residual disease after surgery. After two cycles of high dose carboplatin, we noted 1 CR, 4 PR and 2 MR. After the subsequent two cycles of high dose cyclophosphamide we observed an additional response in 4 cases. On the other hand, 4 patients clearly showed evidence of PD immediately after the first course of cyclophosphamide (2 cases) or following the second course. Three of the 4 patients had shown respectively 1 CR and 2 PR after the second course of carboplatin. Whereas it was confirmed that 2 courses of high dose carboplatin is effective in high risk MB/PNET children, we observed an unacceptable number of PD during the subsequent high dose cyclophosphamide therapy. A review from the literature also suggests that, in general, the longer radiotherapy is delayed, the higher the incidence of PD. In the search for the optimal drug combination in "sandwich chemotherapy" for children with high risk MB/PNET, PD must be reduced to an acceptable incidence, since a high number of PD may significantly lower the probability of long-term survival.

Adolescent↗