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C Di Rocco

Publications and source records attributed to C Di Rocco.

At least 37 records · Page 2Linked to original sources
PubMed2004

Craniopagus: the Thessaloniki-Rome experience.

OBJECT: A case of two female Greek twins conjoined at the left frontal and orbital regions is described. These conjoined twins were transferred to the Pediatric Neurosurgical Unit of the Gemelli Hospital, Catholic University Medical School, at the age of 4 months for possible surgical division. METHOD: Multiple diagnostic investigations, which included in particular advanced imaging techniques, demonstrated an independent cerebral vascularization of the two babies and excluded significant intracranial malformations. Such an accurate evaluation of the anatomy of the craniopagus at the level of the cranial junction allowed the feasibility of the surgical correction to be established. CONCLUSIONS: The operation was carried out by a multidisciplinary team in a single stage. The operating team included two distinct teams of neurosurgeons and anesthesiologists as well as a plastic surgeons' team. Both babies survived the operation. The postoperative course was uneventful. Both twins were discharged in good clinical and neurological condition after a 3-week period of hospitalization.

Brain↗
PubMed2004

Muenke syndrome.

BACKGROUND: Muenke syndrome is a genetically determined craniosynostosis that involves one or both coronal sutures. In some patients it is associated with skeletal abnormalities such as thimble-like middle phalanges, coned epiphysis, and/or neurological impairment, namely sensorineural hearing loss or mental retardation. In spite of a variable phenotype, Muenke syndrome has been related to a unique mutation on the FGFR3 gene, Pro 250 to Arg, which is characteristic of this disease. Because of the incomplete penetrance of this anomaly, the suspicion of Muenke syndrome must be raised in any child with uni- or bilateral coronal craniosynostosis, and the genetic analysis propounded even in the absence of extracranial features. ILLUSTRATIVE CASES: We report the cases of two sisters who presented with Muenke syndrome and whose affected mother did not have any form of craniosynostosis.

Arginine↗
PubMed2004

Hemispherectomy procedures in children: haematological issues.

OBJECTS: In literature, excessive perioperative haemorrhage and related haemodynamic instability have been described as major risk factors in hemispherectomy. In this report we analyse the impact of neurosurgical operation on both the haematological and coagulative patterns of these children, especially focusing on younger patients. METHODS: From 1993 to 2003, 18 consecutive children suffering from intractable epilepsia and treated by hemispherectomy were admitted to the Pediatric Intensive Care Unit (PICU) of Catholic University Medical School, Policlinico Gemelli, Rome. Eight children had an entire hemisphere removed (anatomical hemispherectomy), whereas the remaining 10 underwent disconnective procedures (functional hemispherectomy) or cerebral cortex ablations (e.g. hemicorticectomy). Eleven out of these 18 children underwent hemispherectomy because of hemimegalencephaly (HME): their mean age was 14.5 months (range 3-56 months); non-HME patients underwent surgery for epileptogenic lesions involving the cerebral hemisphere to a great extent or diffusely. Data have been compared with an historical cohort of 13 children operated on before 1992 at the same institution comparable for age, aetiology of epilepsy and the modalities of surgical operation. CONCLUSIONS: Blood losses and haemotransfusions showed a profound influence on the haematologic/coagulative status of the children operated upon. A strict correlation was demonstrated between estimated red cell volume (ERCV) loss and haemostatic impairment in this series. Recent surgical techniques appear to reduce blood losses and related haemocoagulative risks even in younger patients.

Blood Coagulation Disorders↗
PubMed2004

Blood sparing in craniosynostosis surgery.

One of the main risks of craniosynostosis surgery is the possible need for an allogenic blood transfusion (ABT). Most patients are operated on in the first months of life, when physiological conditions are particularly sensitive to even limited blood losses. Furthermore, most surgical techniques proposed in the past were based on extensive craniectomies and cranial remodeling. Because of the known infective and immunologic risks of ABT, in recent years more attention has been dedicated to factors that might help reduce the risk of ABT. We review recent preoperative (ie, erythropoietin administration), intraoperative (ie, acute normovolemic hemodilution, intraoperative blood salvage), and postoperative (ie, clinical monitoring, postoperative blood salvage) anesthesiologic procedures developed with this aim in mind. We also consider operative techniques and technical apparatus that reduce surgical invasiveness, particularly preoperative planning, age selection, and the role of endoscopic assistance and gradual distraction devices.

Blood Loss, Surgical↗
PubMed2004

Nerve growth factor and brain-derived neurotrophic factor in human paediatric hemimegalencephaly.

Paediatric hemimegalencephaly (HME) is a congenital central nervous system (CNS) disorder, characterized by monolateral cerebral hemisphere enlargement, intractable seizures starting in the post-neonatal period, and mental retardation associated with neuropathological anomalies (mainly cortical thickness and lack of lamination). Nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF) are two neurotrophic factors produced in the mammalian CNS that are involved in the survival, development, and function of a variety of brain cells. In the present study, we found increased cerebral tissue levels of NGF and BDNF in 4 infants with HME; these changes appear to be also associated with abnormal NGF-receptor expression in subcortical blood vessels. Moreover, the marked reduction of cortical choline acetyltransferase immunoreactivity is strongly suggestive of a dysregulation in the NGF differentiative activity in this site that could lead to the pathogenesis of HME.

Antibodies↗
PubMed2004

[Endoscopic third ventriculostomy: complications and failures].

Endoscopic third-ventriculostomy (ETV) became the treatment of choice for non-communicating hydrocephalus and its effectiveness is largely reported. On the contrary, specific articles on complications and failures of this technique are very rare and this review aims at supplying further information about it. Therefore, an analysis of the main and up-to-date series is made and exhaustive data about complications and failures of ETV and about their incidence are obtained. The overall frequency rate of complications is 6-20%. Their severity may vary either because of the length of the damage (transient or permanent) or the value of the involved structures (basilar artery, areas of the CNS, hypothalamus) or the importance of the injury (from subclinical sequelae to fatal complications). Moreover, this study showed both the risk that the endoscopic procedure must be suspended (intra-operative failure) and the risk of an early (before 1 month) or delayed (even some years after the intervention) narrowing of the ventriculostomy. The rate of and the reasons for failure have been analysed and the success of a second ETV has been estimated. The results of this analysis suggest that the children proposed for ETV are carefully selected and meticulously studied during the follow-up.

Blood Loss, Surgical↗
PubMed2003

Desmoplastic infantile ganglioglioma.

BACKGROUND: Desmoplastic infantile gangliogliomas are rare intracranial tumors that mostly occur in the first 2 years of life. They are exclusively supratentorial, generally have a voluminous size, and are partially cystic. Histologically they are characterized by a divergent astrocytic and ganglionic differentiation and a prominent desmoplastic stroma; more primitive cells may be observed, which present a higher number of mitoses and these areas can mimic the features of malignant astrocytomas. Surgery is the treatment of choice. Data available from the literature suggest that no complimentary treatment is needed in cases of complete tumor resection. Chemotherapy is an option in infants with infiltration of eloquent CNS structures and progressive disease after surgery. DIGs have generally a good prognosis: recurrence-free intervals of up to 14 years have been reported and spontaneous disappearance of tumor residuals has also been described. CASE REPORTS ND DISCUSSION:We report two cases of DIGs. The first child underwent a staged partial removal of a huge right fronto-temporo-parietal tumor when she was 2 months old. At that time histological diagnosis was anaplastic astrocytoma and on these grounds she underwent six chemotherapy cycles, with a partial reduction of the tumor residual. When she was 16 months old a new operation and complete removal of the tumor residual was performed; histological diagnosis was DIG. A review of the initial histological samples confirmed this diagnosis. Twenty-two months after surgery no tumor recurrence has been documented. This case is an example of a difficult differential diagnosis, which can lead to incorrect management choices. The second patient was operated on when he was 9 months old for a mostly cystic right temporo-parieto-occipital DIG. At surgery a deep nodule, strictly adherent to the Galen and internal cerebral veins, was not removed. MRI control 9 months after surgery showed the disappearance of the tumor residual. Eleven years after surgery no tumor recurrence has been documented. The history of this patient confirms that tumor residuals do not need complimentary treatment; indeed they do not usually grow and, as in our patient, they can spontaneously disappear.

Astrocytes↗
PubMed2003

Infantile myofibromatosis of the central nervous system.

BACKGROUND: Infantile myofibromatosis is the most common fibrous disorder of infancy and childhood. It may occur in two distinct forms: multicentric and solitary. In both cases involvement of the central nervous system (CNS) is unusual: brain myofibromas are usually intracranial in proximity of the dura mater, with infiltration of the calvarial bones and secondary brain compression. Spine myofibromas are exceptional and most of the cases reported in the literature represent secondary locations of visceral lesions. The natural history of myofibromas of the CNS in infantile myofibromatosis is characterized by a period of rapid growth, subsequent stabilization, and spontaneous regression in many cases. On these grounds surgical treatment is reserved for lesions that compress the brain or spinal cord and show an obvious progression. Small lesions or tumor residuals in asymptomatic children without visceral involvement should be closely observed as first choice. CASE REPORT: We report the case of a 10-month-old child with a solitary intramedullary myofibroma, extended from C7 to the conus. The patient underwent partial surgical removal of this lesion. Control MRI has shown the spontaneous slow reduction of the tumor residual and a progressive improvement of preoperative neurological conditions has been observed. To our knowledge this is the first case in which a solitary myofibroma was localized within the spinal cord.

Adolescent↗
PubMed2003

Subdural hygroma: an unwanted result of Sylvian arachnoid cyst marsupialization.

INTRODUCTION: Subdural hygroma is a known complication of Sylvian fissure arachnoid cysts (SACs). However, most of the data in the literature refer to spontaneous or posttraumatic occurrence of subdural hygromas, regarded as either a favorable or an unfavorable event. Little is known about this phenomenon as a consequence of the surgical management of SACs. The present study was carried out to evaluate the significance of postoperative subdural hygromas in children with temporal arachnoid cysts, who have been treated with craniotomy and wide marsupialization of the cystic membrane. CLINICAL MATERIAL AND RESULTS: Between 1980 and March 2002, 104 children were operated on for a SAC at the Pediatric Neurosurgical Unit of the Catholic University Medical School in Rome. Six patients (5.8%; boys/girls=4/2; mean age 3.28 years) developed a subdural hygroma postoperatively, which required further treatment. According to Galassi's classification 4 children had a Type III cyst and 2 children a Type II cyst. All the patients had previously been submitted to open marsupialization of their arachnoid cyst and extensive removal of the cyst wall. In 5 patients (Type III cyst: 4 patients; Type II cyst: 1 patient), acute or subacute symptoms and signs of increased intracranial pressure (ICP) developed at a temporal distance, varying from 5 days to 1 month (mean interval: 14.3 days). All these children needed surgical treatment for the hygroma. A subduro-peritoneal (SDP) shunt was implanted as the first step in 3 patients (Type III cyst: 2 patients; Type II cyst: 1 patient). In the remaining 2 patients of this group (Type III cysts) medical therapy was initially attempted (acetazolamide: 24 mg/kg) without any improvement in clinical manifestations; on these grounds an external spinal subarachnoid drainage was implanted, but led only to the transient clearance of symptoms. The direct drainage of the subdural collection (SDP shunt in 1 patient and temporary external subdural drainage in the other), was followed by complete clinical recovery in both cases. The last patient in this series showed a pathologic increase in head circumference during the 1st month after surgery for his arachnoid cyst, followed by a subsequent stabilization. A CT scan documented a subdural hygroma, which first of all increased in size, but stabilized 2 months after surgery. No surgical treatment was performed in this case. At a mean follow-up of 2.38 years (minimum: 1 month; maximum: 5 years) all the patients are in excellent clinical conditions. Complete neuroradiological resolution of the hygroma was observed in 2 of the 4 shunted patients, who have both been submitted to shunt removal (2 and 3 years after the implant respectively). CONCLUSIONS: It is our opinion that a wide opening of the external membrane of SACs may predispose the CSF fluid to accumulate within the subdural space, where its absorption is insufficient. A ball mechanism created by CSF pulsation may further increase the subdural fluid accumulation. Secondary distortion and occlusion at the level of the basal cisterns may also contribute to the persistence of the subdural fluid collection. Clinical manifestations may be transient, but frequently have a progressive course and the drainage of the subdural collection is, though transient, required in most cases. On these grounds we suggest the selective opening of the basal cisterns associated with a limited resection of the outer cyst walls in order to limit CSF access to the subdural space.

Arachnoid Cysts↗
PubMed2003

[Prolonged ICP monitoring in children with sylvian fissure arachnoid cysts].

BACKGROUND: The decision making process for patients with sylvian fissure arachnoid cysts still represents a challenge for the neurosurgeon. A high proportion of the patients is indeed asymptomatic, in spite of neuroimaging signs of apparently increased intracranial pressure (ICP). The aim of the present study was to evaluate the usefulness of prolonged ICP recording in the preoperative work-out. METHODS: Twelve children (11 M/1 F) harboring temporal arachnoid cysts were investigated (men age 6.3 years). According to Galassi classification they were subdivided into 3 groups. Group I included 3 patients with Type I cysts; group II comprised 6 children with Type II cysts; group III consisted of 3 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 ICP continuous check. Ten mmHg was arbitrarily chosen as the upper normal limit of ICP in resting conditions 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 3 children with Type I cysts (49-86% <10 mmHg during the entire recording), in spite of the fact that 2 of them were apparently symptomatic. Three of the 6 children with a Type II temporal cyst had elevated ICP values (69-99% >10 mmHG), even though they had an incidental (1 case) or prenatal (2 cases) diagnosis. The remaining 3 patients had normal ICP values for more than 70% of the recording time period. Two of the 3 patients with type III temporal arachnoid cysts had almost constantly abnormal ICP values (95=99% >10 mmHg); conversely the third patient showed normal ICP values for more than 80% of the recording time period. Five patients were operated on; in 4 of them the surgical indication was based on ICP recordings (2 of the 3 children with a Type II cyst and increased ICP and the 2 patients with a Type III cyst and increased ICP). The last child, harboring a Type I cyst, was operated on under parents request, as a preventive measure. At a mean follow-up of 10.1 months all the patients operated on are in excellent clinical conditions; 1 of them (Type II cyst) initially submitted to craniotomy and cystic membrane excision needed a subdural-peritoneal shunt implant 3 months after surgery for the appearance of a symptomatic subdural hygroma on the side of the cyst. Postoperative CT showed signs of brain expansion in the 2 patients operated on for a Type III cyst, and in the patients operated on for a Type I cyst. CONCLUSIONS: With the limit of the relatively small series here presented, prolonged ICP recording appeared to be an important preoperative tool to rule out the necessity of operating on children with Type I cysts. Furthermore, it was particularly useful in the decision-making process for children with Type III cysts. In cases of Type II lesions, the investigation resulted less discriminating, though the ICP monitoring contributed to find out those patients in whom surgery was indicated in spite of the absence of symptoms.

Adolescent↗
PubMed2002

Cerebral hemorrhage in Henoch-Schoenlein syndrome.

BACKGROUND: Henoch-Schoenlein syndrome (HSS) is the most common form of vasculitis seen in childhood. The clinical diagnosis is based on the association of nonthrombocytopenic purpura, arthritis and abdominal pain. Nephropathy is the most common complication. Hemorrhages can occur in the respiratory, gastrointestinal and urinary tracts. Neurological complications are rare, though they may be particularly severe. CASE REPORT AND DISCUSSION: Intracranial hemorrhage is an extremely rare complication of the disease; we report the case of a child with cerebral hemorrhage in HSS and review the literature.

Cerebral Hemorrhage↗
PubMed2002

Transsphenoidal meningoencephalocele.

BACKGROUND: Encephaloceles are rare cranio-facial developmental anomalies. CASE REPORT: In this paper, we discuss the case of an 8-month-old girl affected by a transsphenoidal meningoencephalocele and try to give landmarks of the state of art in the surgical treatment of this rare pathology.

Abnormalities, Multiple↗
PubMed2002

Surgical treatment of chronic subdural hygromas in infants and children.

BACKGROUND: Extra-axial chronic fluid collections are pathological conditions occurring more frequently in the paediatric age, particularly in children less than two-year-old. Although recognised for many years and repeatedly reported in the literature, some aspects of their treatment are still under debate. This review of the management of chronic subdural collections is based on our experience of 72 cases treated between January 1984 and December 2000. METHOD: Patients were divided into four groups according to aetiology. Thirty-four cases (47%) occurred following various surgical procedures; 14 cases (20%) occurred as complications of leptomeningeal infections; 13 cases (18%) were post-traumatic, whereas no obvious aetiology could be detected in the last 11 cases (15%). The collections were unilateral in 34 patients (47%) and bilateral in the other 38 (53%). FINDINGS: Thirty-five patients (48%) were treated by means of a temporary subdural external drainage (SED) (for a total of 38 SED procedures), which was maintained for a mean of 5.8 (+/-3.4) days; it was effective in 26 cases, whereas in the other 9 it was necessary to perform a subdural-peritoneostomy (SPS). Three of these 38 SEDs were complicated by infection. In one more child the external drainage was complicated by a chronic subdural haematoma. A SPS was performed in 44 cases (61%), 9 being failed external drainages. Only in 16 (38%) of these 44 patients the SPS was removed after an average of 27.0 (+/-16.6) months. Three patients (4%) were treated by craniotomy and resection of the neomembranes lining the subdural fluid collection. Obstruction of the SPS occurred in 6 children, infection in 4. Good results were obtained with either external or internal drainage. In fact post-operative neuroradiological investigation disclosed in all the cases complete or almost complete cerebral re-expansion; also the clinical outcome was generally very good, although largely dependent upon the basic clinical condition. Unilateral subdural-peritoneal and/or external drainage was effective also in case of bilateral collections. Only 2 patients required temporary bilateral external drainage.

Adolescent↗
PubMed2001

Rapid detection by fluorescent multiplex PCR of exon deletions and duplications in the C1 inhibitor gene of hereditary angioedema patients.

Hereditary angioedema (HAE) is due to a variety of defects in the C1 inhibitor gene (C1NH gene), including approximately 20% of partial deletions/duplications whose boundaries are usually within Alu repeats. To ensure complete molecular characterization of C1 inhibitor deficiencies a fluorescent multiplex assay was constructed to amplify simultaneously five exons of C1NH and an exon of the BRCA1 gene. PCR protocols were optimized for these amplicons (size range between 300 and 700 bp). Forward and reverse chimeric primers that carry strand-specific 5' tags of 16 nucleotides were used to ensure similar levels of PCR products for each amplicon in the multiplex. Data were analyzed by superposing fluorescent profiles of test and control DNA and by visually comparing the normalized peak levels of corresponding amplicons, rather than by calculating the ratios of peak areas. Tests on a collection of known defects, including five different Alu-mediated deletions and a partial duplication have validated this approach. In a study of 19 sporadic cases of HAE, of which four had failed to reveal mutations upon screening all exons by fluorescent chemical cleavage, three de novo deletions were diagnosed by using this multiplex PCR approach: a deletion of exon 4, a deletion of exons 5 and 6, and an apparently complete gene deletion. Besides being suitable for the initial DNA screening of the C1NH gene in HAE patients prior to screening for point mutations, this method can be easily adapted to complex genes for the screening of rearrangements.

Angioedema↗