Biomedical subjects
Publications and source records attributed to F Velardi.
Accessory lower limbs are rare anomalies that are associated with other severe malformations. The authors describe a case of this type and discuss the pathogenetic and classification problems surrounding these malformations in the light of the limited number of the other cases reported in the literature.
Early diagnosis of skull fibrous dysplasia in children is relatively easy, based on a history of painless progressive bony bulging. The therapeutic approach is still controversial, due to the benignity of the lesion. However, the clinical course may be unpredictable, with sudden appearance of symptoms, some of which can be important and irreversible. In pediatric patients, the possibility that an early surgical correction might positively interfere with the natural history of the lesion has to be evaluated by taking into account the obvious difficulties that will be encountered in reconstructing the skull after a wide excision of the pathologic bone. In the present report, we describe our personal experience on the surgical treatment of 9 children, ranging in age between 7 and 14 years. The patients were subdivided into two groups, according to the localization and extent of the disease. Patients in group 1 presented an involvement of the hair-covered cranium and/or the fronto-orbital region. Group 2 patients presented with a multizonal involvement of the skull, including the central cranial base (pterygoid, sphenoid, petrous and mastoid bone). The different surgical options, chosen for the two groups of patients, are discussed, together with the technical methodologies utilized, the cosmetic results and the long-term follow-up.
The introduction of the new diagnostic tools for neuroimaging has resulted in the early recognition of congenital brain tumors. In the present report we describe 39 children, in whom the diagnosis of an intracranial tumor has been obtained during the first 12 months of life. These patients represent 14.4% of a pediatric population of 271 children with brain tumors, observed in the same period of time (11 years) in the Neurosurgical Institute of the Catholic University of Rome. Most of the tumors were located within the supratentorial compartment. Midline tumors were common. Sixteen children underwent the total removal of the tumor. Four infants could not be operated either because their poor general condition or the extension of the tumor. In one case, treatment was refused by the parents. The remaining children underwent the partial removal of the tumor, which was followed by chemotherapy (malignant tumors) or by serial neuroradiological examination (benign tumors), with the aim of postponing radiotherapy at the end of 2 years of age at least. The total mortality (surgical deaths: 7 cases) and deaths during the follow-up period (11 cases) was 38.5%. Out of the 24 surviving children, 8 (20%) are normal: 7 (17%) exhibit only minimal neurosurgical deficits; 9 (22%) are seriously handicapped. One child has been lost for the follow-up observation.
One hundred-sixty-one cases of children operated on for craniostenosis in the first year of life at the Neurosurgical Department of the Catholic University of Rome in the period 1982-1986 are presented. The average age at diagnosis was 6 months and at operation 7.4 months. The patients considered in the present study were subdivided into two groups according to the prevalent involvement of sagittal suture or of the anterior sutural group (coronal metopic, spheno-frontal and spheno-palatine sutures). In fact, these two groups differ considerably not only in the characteristics of the pathological process of early fusion of the cranial sutures, but also in the technique required for surgical correction. Four children with premature fusion of all cranial sutures are considered separately. Congenital malformations of the bone structures or changes in ocular motility were presented in 7.9% of 63 patients with sagittal craniostenosis and in 14.7% of the group with premature fusion of the anterior cranial sutures. Surgical correction (linear craniectomies extended to the cranial base for the group with sagittal craniostenosis, craniectomies with advancement of the supraorbital margin and rotation of the bone operculum for the group with anterior craniostenosis) proved satisfactory in the great majority of cases. Postoperative morbidity was particularly low. Apart from postsurgical anaemia which required blood transfusion on the third day in 15.5% of operated patients, the only complications were transitory hyperpyretic states observed in 6.2% of cases. Only one child died for reasons related to the surgical procedure. The low postoperative mortality (0.6% of cases) and the low percentage of bone pathology recurrence (7.4%) compared with the results of other series involving older children, seem to indicate that the early surgical correction of bone malformations does not bear additional risk for the patient, permitting at the same time normal functional development of the brain and a more gradual morphological remodelling of the skull.
The clinical features, the diagnostic findings and the surgical results obtained on 35 children, under 1 year of age, operated on because of an early synostosis of the metopic suture are presented. In 79% of cases, the diagnosis was obvious at birth. A M/F ratio of 2.0 was observed in this series. Associated malformations were recorded in eleven patients (30.5%). The surgical procedure consisted of bilateral frontal craniectomy, opening of the roof of the orbit, advancement of the superior orbital margin, and repositioning of the frontal flaps after 180 degrees rotation. The cosmetical correction was satisfactory even in the immediate postoperative period. No mortality was recorded. The surgical correction was followed by a normal psychomotor development in 33 cases; delayed milestones were recorded in 2 children in whom the metopic synostosis was part of a complex malformative syndrome. On the grounds of our results, the young age of the patients at the operation should not be regarded as an obstacle to the immediate surgical correction; in our advice, the early correction results also in better functional and cosmetic long-term outcomes.
A classification of anterior plagiocephaly is proposed by the authors on the basis of the clinical and radiological findings analyzed in a series of 28 subjects less than 1 year old. Three main patterns of the anomaly have been identified. Type I anterior plagiocephaly is characterized by unilateral flattening of the frontal bone and elevation of the superior orbital ridge without deviation of the nasal pyramid. The vomer and petrous bones maintain a normal position. In type II anterior plagiocephaly, the frontal and orbital anomalies are accompanied by contralateral deviation of the nasal pyramid and homolateral anterior displacement of the petrous bone. A deviation of the sphenobasilar bone is associated with the above regard to the anomalies in type III anterior plagiocephaly. The three types of this particular form of craniosynostosis differ with difficulties involved in surgical management and outcome.
The authors describe their technique of dynamic CSF scanning utilizing the radionuclide material Tc-99m diethylentriamine-pentacetic acid (DTPA). Thirty-four infants with macrocephaly and enlarged intracranial CSF spaces were investigated with this technique. The resulting studies were graded according to severity. The grading scheme was of value in determining which patients would benefit from a CSF diversionary shunt.
The outcome of a light and electron microscopic study of a congenital choroid plexus papilloma removed from a lateral cerebral ventricle of a 10-day-old male presenting hypertensive hydrocephalus at birth is reported. The tumor was diagnosed by CT and cerebral angiography. The findings, compared with those obtained from choroid plexuses of controls subjected to hemispherectomy for epilepsy, highlight the intense metabolic activity of the papilloma and the relevance of this to the communicating hydrocephalus that is frequently associated with it.
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9 children, presenting with clinical symptoms of craniosynostosis, and 4 with microcrania underwent calvaria bone scanning and a lumbar subarachnoid constant-infusion manometric test as integrating procedures for the diagnosis and surgical indication. In all the patients with craniosynostosis, calvaria bone scanning revealed the pathological sutures as areas of diminished or absent radionuclide accumulation, while a normal isotope uptake was observed at all the cranial sutures in the children with microcrania. A biphasic response to the infusion test, with an early rapid increase in the intracranial pressure and a late compensation, characterized 8 of the 9 children with craniosynostosis. The response to the infusion test was normal in all the cases with microcrania and in 1 child regarded as affected with coronal synostosis on the grounds of clinical and calvaria bone scanning findings.
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In acute experiments on lambs, the effects of short-lasting induced modifications in arterial and venous systemic blood pressure on intracranial pressure have been studied. Haemodynamic changes have been provoked by increasing the cardiac venous return, by increasing or decreasing the arterial resistance and by stimulating the vagus nerve. Intracranial pressure modifications have been demonstrated to depend essentially on arterial influences. A direct venous influence is apparent only when the central venous pressure is increased, as occurs in the case of increased venous cardiac return.
Experimental hydrocephalus has been induced in lambs by artificial increase of the amplitude of intraventricular cerebrospinal fluid (CSF) oscillations related to arterial pulsations, without concomitant changes of the mean CSF-pressure. The characteristics of this hydrocephalus demonstrate that the intraventricular CSF-pulsations can play a role in the genesis of ventricular dilation. Such a method may be used to produce an original model of hydrocephalus independent of changes of CSF-circulation or absorption.
Out of 276 children with craniosynostosis operated on in a 9 year period, 231 were operated on under 1 year of age. According to the sutures involved in the process of early fusion, two groups of infants were individuated. Patients belonging to the first group were characterized by the involvement of the sagittal suture, while infants of group II presented with a synostosis of the anterior or posterior sutural complex. Two different techniques were required for the 2 groups of subjects, the first of them being mainly based on linear craniectomies, the second requiring more complex reconstructive surgical procedures. The morbidity, mortality and recurrence rates of the series were low, demonstrating that infants can tolerate the procedure as well as older children. The advantage of the early treatment consists of a better cosmetic result with a more physiological growth of faciocranial skeleton.
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