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Biomedical subjects

A Iannelli

Publications and source records attributed to A Iannelli.

At least 55 records · Page 3Linked to original sources

Isolated agenesis of the gallbladder. An intraoperative problem.

Agenesis of the gallbladder and cystic duct is a rare congenital malformation. In 40-70% of cases this anomaly is associated with other gastrointestinal, skeletal, cardiovascular and genitourinary malformations. Lithiasis of the common bile duct is present in 25-50% of cases. In the majority of cases patients are asymptomatic or have symptoms compatible with a biliary disorder. A preoperative diagnosis is extremely difficult and the absence of the gallbladder is often an intraoperative finding. The authors report a case of isolated agenesis of the gallbladder. The relative embryology, development, diagnostic pitfalls, intraoperative behaviour and therapeutic strategies are discussed.

Adult↗

Combined management of intracranial vascular malformations in children.

UNLABELLED: We have reviewed 39 cases of intracranial vascular malformations, observed in the Section of Paediatric Neurosurgery of the Catholic University of Rome between 1980 and 1995 (age 1-15 years). Diagnosis was of parenchimal AVM in 26 cases, in 7 cases of AVM of the Galen region, in 5 cases of dural AVM and in 1 case of venous angioma. RESULTS AND CONCLUSIONS: Haemorrhage was the prevalent clinical onset in patients with parenchimal AVMs (57.7% of cases). Only one patient with a Galen region AVM presented cardiovascular symptoms, while 2/5 children observed for a dural AVM complained a congestive heart failure. Total surgical excision was performed in 17 of the 26 patients with parenchimal AVMs; moreover surgery was combined with preoperative endovascular embolization in 2 cases and with radiosurgery in 1 patient; we think that surgery is still the treatment of choice in patients with parenchimal AVMs. Endovascular treatment and radiosurgery have mainly an adjuvant role; nevertheless we agree that a preoperative endovascular management of parenchimal AVMs reduces the surgical risk, especially in cases of huge AVMs or AVMs localized in eloquent areas of the brain. Radiosurgery may be an alternative choice for complex or deeply sited AVMs; however in most cases, a combination with surgery or endovascular treatment is necessary to reduce the "target" size. AVM embolization was the only treatment in 2 of the 6 cases of dural AVMs; dural AVM embolization was combined with surgery in 1 case. Endovascular AVM exclusion is the treatment of choice in these patients. Surgery may be combined with endovascular treatment in case of a partial persistence of the AVM, or if signs of revascularization are present on control angiography. A conservative management is justified in patients with carotido-cavernous fistulae; in fact a complete spontaneous thrombosis is very frequent in these cases. A high rate of cardiovascular symptoms is reported in patients with Galen region AVMs diagnosed in the 1st year of life, but only 1 of our patients complained of congestive cardiac symptoms. Surgical treatment of Galen region AVMs is associated with a high morbidity and mortality. Actually a transarterial embolization of afferent vessels represents the treatment of choice in these patients; it can be combined with transvenous approach in case of complex AVMs and/or a non complete AVM exclusion.

Adolescent↗

Surgical management of paediatric cerebral cavernomas.

Over the past 15 years (1981-1995) 24 children with cerebral cavernous angiomas have been treated surgically at the Section of Pediatric Neurosurgery of the Catholic University of Rome. The most common symptoms at presentation were epilepsy (13 children) and intracranial hypertension (9 children). None of these patients had a family history of cerebro-vascular malformations. Multiple lesions were detected in two subjects. In 19 cases, radiological signs of significant acute and subacute hemorrhage were present at the radiological examinations. The supratentorial compartment was the most frequent localization of the lesions, with only four cavernomas observed in the subtentorial space. The post-surgical results were good: only nine children had a persistence of the presurgical neurological signs, although ameliorated by the treatment; no patient showed a progression of pre-operative neurological signs, neither the onset of new deficits; only one child died, but his clinical conditions were very critical before surgery. Our results suggest that the youngest children present with evidence of significant hemorrhages more frequently than their older counterpart. In our series, the removal of cavernomas allowed to control the seizure disorders in all the case, thus suggesting that in this type of malformative lesion, lesionectomy alone may be sufficient to resolve epilepsy.

Adolescent↗

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↗

Minilaparotomy with rectus muscle sparing: a personal technique for cholecystectomy.

The present paper describes a minilaparotomy technique characterized by an horizontal incision of the skin and two successive vertical incisions which run along distinct sagittal as well frontal planes, sparing, thus, the rectus muscle. This approach, in comparison to other minilaparotomy techniques, ensures a reduced incidence of incisional hernia, decreased postoperative pain and optimal cosmetic results. The technique is useful when laparoscopic cholecystectomy is contraindicated or the laparoscopic apparatus is not available.

Cholecystectomy↗

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↗

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↗

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↗

Inhibition of c-ras reactivates thyroglobulin synthesis in middle-T transformed thyroid cells.

Differentiated thyroid cells expressing polyoma Middle-T became transformed and tumorigenic when injected into syngenic animals. The expression of thyroglobulin was greatly reduced and no longer responsive to thyrotropin (TSH) and to cAMP. Inhibition of endogenous c-ras by the expression of two transdominant negative mutant H-ras genes, Asn17 and Leu61-Ser186, reactivated thyroglobulin synthesis. Reactivation of thyroglobulin synthesis by c-ras inhibition was not observed in the absence of TSH. These findings indicate that MT elicits dedifferentiation of thyroid cells by activating endogenous c-ras and that c-ras interferes with TSH or cAMP signaling.

Animals↗

[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↗

[Surgical treatment of epileptogenic hemimegalencephaly].

Thirteen children affected by hemimegalencephaly were observed in the Pediatric Section of the Institute of Neurosurgery of the Catholic University of Rome in the last six years. Nine of them were operated because of an intractable epilepsy. Seven were males and 2 females; the age at operation ranged between 7 months and 11 years (mean: three years and five months); the follow-up period varied between 1 and 6 years (mean 3 years and 10 months). All the patients had a clinical history of daily epileptic seizures not responsive to medical treatment; all of them presented with severely delayed psychomotor development. At neurological examination, six children showed a motor deficit of variable severity contralateral to the affected hemisphere and two patients a severe tetraparesis. The remaining child did not present with motor deficit. In all the cases the diagnosis had been obtained by CT scan and MRI. One of the cerebral hemispheres was abnormally enlarged with associated dilation of the lateral cerebral ventricle. The cortical architecture was obviously deranged with several areas of heterotopia of the gray substance suggesting an alteration of the neuronal cell migration. All the children underwent an extrathalamocaudato hemispherectomy. A post-operative ventriculo-peritoneal shunt was required in two cases. There were neither operative, nor late deaths. A dramatic reduction in the frequency and severity of epileptic seizures was observed in all but one of the patients of the series.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain↗

Spinal epidural meningiomas in childhood: a case report.

A case of a girl affected by an extradural spinal meningioma is described. This lesion is rare in childhood, and the prognosis may be not favorable. In our case a complete removal of the neoplasm with exeresis of the involved dura resulted in a very good clinical and radiological recovery.

Adolescent↗

Intracranial tumours in the first year of life. A report on 51 cases.

The authors report on 51 infants with intracranial tumours treated in an eleven-year period; these infants represent 13% of the total population of children with intracranial tumours who have been operated on in the same institution during the same period of time. Males (28 cases) were slightly more frequent. Astrocytomas (17 cases), medulloblastomas (12 cases), and ependymal tumours (5 cases) were the commonest histologic types. Signs and symptoms of increased intracranial pressure were by far the most frequent clinical manifestations, followed by seizure disorders. Thirty tumours were localized within the supratentorial, and 21 within the subtentorial compartment. The parasellar region (10 cases) and the lateral cerebral ventricles (8 cases) for the supratentorial tumours, the inferior cerebellar vermis and fourth ventricle (13 cases) for the infratentorial tumours appeared to be the preferred topographic locations. Craniotomies were carried out in 44 infants, with a total or radical removal of the tumour in 19 cases, a subtotal removal in 6 cases, and a partial removal in 17 cases. In 3 cases only a biopsy procedure was performed. Twenty-nine of these patients required an ancillary procedure such as CSF shunting. Three subjects underwent a biopsy procedure and 1 infant the insertion of a CSF shunting device only. Surgery was not performed in 5 cases. Overall, there were two surgical deaths. Two infants died before any surgical treatment could be performed. Radiation therapy was administered to 9 patients when they had reached three years of age. Chemotherapy was given to 21 infants, according to various chemotherapeutic protocols. During the postoperative period 20 deaths (39%) were recorded. Two patients were lost to follow-up. From 1 to 10 years after the operation, 29 patients are still alive, 14 of them (28%) with a normal psychomotor development, 10 (20%) with some neurological or mental deficits, and 5 (10%) with severe psychomotor retardation. There was no apparent correlation in this series between late outcomes and the histological type of the tumour.

Astrocytoma↗

Epilepsy and low grade gliomas in pediatric neurosurgery.

A series of 33 children affected by low grade gliomas and epilepsy is reported. Seizures are often the only symptom of these patients; intracranial hypertension and focal neurological signs may be present too. All children were operated on; tumor removal was performed in 21 cases, tumor removal associated with corticectomy and/or lobectomy was performed in 12 patients. The treatment of epilepsy has been more effective in the first group of patients with only lesionectomy than in the second one, in which neoplasm removal was coupled with nervous system tissue asportation.

Adolescent↗

[Spinal lipomas in childhood].

Spinal lipomas account for 5% of the tumors of the spinal cord, frequently present already at birth. Most commonly they are associated with forms of dysraphism, but lipomas without bony involvement are considered dysembriogenetic lesions too. Children with lipoma frequently have intact neurological functions, but may become symptomatic later on. Diagnosis is possible also in neurologically intact patients because of skin lesions or subcutaneous masses. Many surgeons suggest early surgery to prevent injury to neural structures from traction due to cord tethering; others prefer to wait for the rise of any symptom before considering surgery. However, neurological recovery after surgery is rarely observed, and, when present, is always partial; the primary goal of surgery is to stop the clinical progression through the detethering of the cord.

Child↗

[Non-tumoral neonatal hydrocephalus. Results of surgical treatment in the first month of life].

Two hundred eight patients with non-tumoral congenital hydrocephalus underwent CSF shunting below the age of one month. Ultrasonography was the most frequently utilized tool; hydrocephalus was recognized during pregnancy in 52% of the cases. Hydrocephalus was associated with myelomeningocele in 97 infants; in 38 subjects ventricular dilation was secondary to aqueductal stenosis. Post-hemorrhagic and post-infective hydrocephalus accounted for only 20 and 13 cases respectively. At the follow-up observation, a normal psychomotor development was recorded in 67.3% of the cases. Seventeen patients died. CSF infection was the most common cause of death (41%); in 6 patients the exitus was determined by the natural evolution of congenital associated malformations. Shunt revision did not influence the morbidity and the mortality significantly. On the other hand, CSF infections appeared to influence the prognosis negatively. In our experience, the prognosis of hydrocephalus operated on in the first month of life does not differ from that of hydrocephalus operated on late in life.

Cerebrospinal Fluid Shunts↗