Carlo Mazza

Intracranial aneurysms and subarachnoid hemorrhage in children and adolescents

Thirty-eight cases of symptomatic cerebral aneurysms or spontaneous subarachnoid hemorrhage in children and adolescents were observed from 1965 to 1984; 33 cases were treated from 1970 to date. This group represents 2.6% of the total number of patients with subarachnoid hemorrhage treated at our institute in the same period. The cause of subarachnoid hemorrhage was unknown in 7 cases; an intracranial aneurysm had ruptured in 29 cases, and was unruptured but symptomatic in 2 remaining cases. Three aneurysms were mycotic. The most frequent aneurysmal locations were the internal carotid bifurcation and the anterior communicating artery; peripheral branches of the middle cerebral artery were also a relatively common location. Four patients were 3 years of age or younger: each presented peculiar clinical features, and 3 of the 4 had middle cerebral artery aneurysms. The remaining 34 patients were all above 9 years of age. Two groups were identified: (a) in 14 patients between 10 and 15 years of age, the aneurysm was most commonly at the internal carotid bifurcation (37%), and an intracerebral hematoma was observed in 50% of these cases; (b) in 20 patients between 16 and 20 years of age, the most common aneurysmal location was the anterior communicating artery (35%), and intracerebral hematomas were rare (10% of cases). Among patients with aneurysms, 19 underwent surgical exclusion by clip, with 10% morbidity and 5% mortality; 5 patients in moribund conditions were not operated on; 5 patients were conservatively treated; in 2 patients the aneurysm had disappeared at a second angiography. Ischemic deterioration from vasospasm was observed only in 3 patients, all above 17 years of age, and with a consistent or thick subarachnoid blood deposition on early CT scan. Hydrocephalus was also rarely observed (13% of cases), requiring a shunt in only 3 patients. Overall management results were significantly better than in adult patients, with 73% good results and 21% deaths. The better prognosis in the group under 20 years of age is probably accounted for (a) by the frequently observed reversibility of neural injury in young patients and (b) the very low incidence of ischemic disturbances in this age group.

Results of Treatment with Ventriculoatrial and Ventriculoperitoneal Shunt in Infantile Nontumoral Hydrocephalus

The authors present the results of surgical treatment in 165 children with nontumoral hydrocephalus, treated during a period of 11 years. The minimum period of follow-up was 1 year. The results in the group of children treated with a ventriculoatrial (VA) shunt were compared to those obtained in the group with a ventriculoperitoneal (VP) shunt. 45% of patients with VA shunts had one or more revisions, against 51% of patients with VP shunts. However, the incidence of revisions after 6 months from primary insertion was lower in the VP-shunted children. Most revisions were due to shunt malfunction: obstruction of the ventricular or abdominal catheter was the commonest finding. Among complications, the most serious was constituted by shunt infection (11% of cases). Most infections occurred at less than 2 months from surgery and were caused mainly by Staphylococci. 31% of infected patients died. Other frequent complications were due to shunt tubing lost in abdomen or in the ventricles and subdural hematoma. The overall mortality rate in the whole group was 18%, while the shunt-related mortality rate was 10.9%. Complications were more frequent in the VP-shunted patients, but were less than in VA-shunted patients and accounted for a lower mortality rate. Complications and mortality rate were prevalent in the patients presenting congenital communicating hydrocephalus or myelomeningocele. It is concluded that VP shunt is preferable to VA shunt in the treatment of infantile hydrocephalus.

Spontaneously healed vein of Galen aneurysms. Clinical radiological features.

Vein of Galen aneurysms demonstrate a high tendency to thrombose, as extensive thrombophlebitis of the dural sinuses and deep venous system occurs frequently. This may be explained by the frequent detection of venous anomalies and retention of embryonic venous patterns such as the median prosencephalic vein. Twentyone cases were reviewed (19 from the literature, 2 of our own cases): no typical syndrome for thrombosed aneurysm of the vein of Galen could be found; therefore, a preliminary diagnosis of clotted vein of Galen aneurysm was made in only half the cases. In all the remaining ones, pineal or III ventricle masses were suspected. Nevertheless, crescentic rimlike calcifications in the region of the vein of Galen can be found in 50% of cases. Only in half of the cases did angiography demonstrate the malformation filled by contrast medium, while in the remaining cases not only the aneurysm but also the deep Galenic venous system failed to opacify. Computed tomography and magnetic resonance scans demonstrated masses with high density/intensity values, with varying degrees of calcification. Knowledge of this pathological entity is important in order to avoid unnecessary surgical procedures.

Irreversible respiratory failure in an achondroplastic child: The importance of an early cervicomedullary decompression, and a review of the literature†

The authors report the case of a girl with achondroplasia suffering from a progressively worsening hypotonic quadriparesis. CT scan showed slight dilatation of ventricular and subarachnoid spaces, with well-defined evidence of cortical sulci and gyri. This aspect was compatible with the diagnosis of macrocrania and megalencephaly (CP being 51 cm). The foramen magnum was narrowed, the transverse diameter measuring 15 mm and the 50th percentile being, for age, 26 mm. Somatosensory evoked potentials (SEPs) revealed bilaterally prolonged interpeak latencies Erb-N13, slowing of central conduction time N13-N20 from right median nerve stimulation, and block from left median nerve. The suspicion of cervicomedullary compression was confirmed by MRI, showing a very marked stenosis with compression exerted by the odontoid process. Further, a stenotic cervical canal and optic nerves verticalization were manifest. The patient underwent neurosurgical decompression by suboccipital craniectomy and cervical-C1 laminectomy. In spite of treatment, both neurologic and respiratory problems (rapid, shallow and almost abdominal breathing) were unchanged. The girl died 4 1/2 months later. The authors emphasize the important role of SEPs in detection of cervicomedullary compression in achondroplastic children and also stress the necessity of an early surgical treatment as the only condition for possible clinical improvement and/or full recovery.

Transient focal leukoencephalopathy following intraventricular methotrexate and cytarabine : a complication of the Ommaya reservoir : case report and review of the literature

A 14-year-old boy, suffering from acute lymphoblastic leukemia with meningeal involvement, was treated with intraventricular methotrexate and cytosine arabinoside, administered via an Ommaya reservoir (OR). Three months later, right occipital headache, vomiting, and lethargy appeared. Cerebrospinal fluid specimens showed increased proteins and a right frontal slow-wave focus was evident on the EEG recording. The computed tomography scan revealed white matter hypodensity within the right frontal and rolandic regions. After injection of medium contrast, an abscesslike hyperdensity appeared, surrounding both a well-placed cannula tip and the right frontal horn of the lateral ventricle. Brain swelling and shift signs were also evident. Nine cases of focal methotrexate leukoencephalopathy have been previously reported, and in six of these there was a misplaced OR cannula tip. The focal meihotrexate leukoencephalopathy seems to be related to the neurotoxicity of the drugs administered, and may also exist with a well-placed OR cannula tip. Immediate removal of the catheter may be associated with a benign evolution.

Nonoperative treatment of cerebellar abscesses A case report and review of the literature

Abstract A case of a 5-year-old child presenting with a cerebellar abscess occupying the ponto-cerebellar region, who was treated with antibiotic therapy alone, is reported. Symptomatology, radiological findings, and antibiotic regimens are presented, and the literature is reviewed from the viewpoint of therapeutic management.

Childhood cerebrovascular diseases not associated with vascular malformations.

Of 76 patients in the pediatric age group suffering from cerebrovascular diseases treated in the years 1970–1983, 26 patients (34%) did not harbor intracranial vascular malformations (aneurysms or arterovenous malformations). Two groups of patients were identified: (a) those suffering from a spontaneous intracranial hemorrhage (16 cases); (b) those suffering from an ischemic stroke (10 cases). Of those with spontaneous intracranial hemorrhage, 10 patients underwent surgery and evacuation of the hematoma. In 2 cases the hematoma was located in the posterior fossa, in 1 case in the upper brain stem, and in 3 cases in the basal ganglia; in the remainder the hematoma was supratentorial. Two patients died soon after the hemorrhage. Eight of the surviving patients completely recovered. In those with ischemic stroke, none suffered from congenital heart disease, a well-known predisposing factor. In this second group 1 patient died and 9 survived. Only one patient showed complete recovery. The data indicate that a hemorrhagic stroke is more common than an ischemic stroke in a child presenting with acute onset of hemiparesis and/or loss of conciousness: thus the value of CT scan as the first diagnostic procedure is clear, owing to the possibility of emergency surgical treatment. In children with ischemic strokes, a complete laboratory/clinical evaluation should be undertaken in order to exclude preexisting heart disease, coagulation disorders or lipoprotein abnormalities, and less common systemic diseases.

Dr. Anthony David Hockley

Gentle...compassionate...a wonderful human being...a willing and helpful man...friend...educator...mentor...teacher... first class clinician...pioneer in paediatric and craniofacial surgery These are all the definitions widely utilised in the eulogies the Editor of CNS has received in honour of Mr Tony Hockley. Indeed, it is exceptional for a colleague to receive this kind of universal recognition as, in order to be paid such tributes, it is necessary to be a truly exceptional person, an exceptional teacher, and an exceptional surgeon.

The dramatic history of the plot against the Dominican friar, Peter from Verona: “a holy craniotomy”

During the Middle Ages, Verona was not only the city of Romeo and Juliet, but also the scene of the gruesome murder of the Dominican friar, Peter, from Verona. Peter is the copatron of Verona with Saint Zeno. He was canonized by Pope Innocent IV on March 1253 after an interval of only 337 days from his death, making him the fastest papally canonized saint in history. In the official iconography, he is always represented calm and with a gash across his head, something that should support our neurosurgical patients! Peter was born in the city of Verona in 1206 from a family, perhaps, sympathetic to the Cathars. This heresy held many adherents in the thirteenth century of Northern Italy. He went to a Catholic school and later to the University of Bologna, where at the age of fifteen, he met Saint Dominic, joined the Order of the Friars Preachers (Dominicans), and became a celebrated preacher throughout the northern and central Italy. In 1252, Pope Innocent IV appointed him Inquisitor in Lombardy. Peter evangelized nearly in the whole of Italy, preaching in Rome, Florence, Bologna, Genoa, and Como. The Cathars, against whom he preached, were a heretical group that had adopted elements of dualism, also called Manichaeism, rejecting the authority of the Pope and many Christian teachings. The hatred and frenzy of the Manicheans increased as they grew more and more obstinate. They knew well that unless they gave up their ungodliness, they could hope for no understanding with Peter whom they looked upon as the scourge and destruction of their sect. Accordingly, they conspired to kill him. The principals in this plot were Stefano Confalonieri, Tommaso da Giussano, a little village between Milan and Como, Guido Sacchella, and Giovanni della Chiusa. The price they agreed to pay the assassins was forty Milanese lire, which were placed in the hands of Tommaso da Giussano. For the execution of the crime, they chose Pietro Balsamone, commonly called Carino; and this man selected Albertino Porro for his assistant. From Milan, the friar went to Como, where he was prior. The conspirators let the Easter festivals pass. On the Saturday, within the octave of Easter, April 6, 1252, Peter left his convent before daybreak to return to Milan on foot.

The past examined by a modern eye, a prehistoric case?

It was a sunny day on Thursday, September 19, 1991 when at 1:30 P.M., two German hikers Erika and Helmut Simon descending from the Finail Peak to the Grawand, in the area of the Glacier of Schnalstal, Oetztal Alps, South Tyrol (Fig. 1), near the border with Austria, in the Tisenjoch area at a 3,210 m altitude (Photo 2), noticed a brownish object in a rocky gully filled with melted muddy water. It was a human corpse, the back of the head completely visible, the shoulders and part of his back jutting out of ice and water. The chest was lying against a flat rock. When they reached the Similaun lodge near the place of discovery, they informed the landlord Markus Pirpamer. Since the scene of the finding lies along the Italian– Austrian border, Pirpamer alerted both the Italian Carabinieri in Karthaus-Schnals and the Austrian Gendarmes in Soelden. Both responded that no person had been reported missing in that area for over 50 years!! At that time nobody could have imagined that the dead man was soon to become famous throughout the world; the story of the archaeological wonder of the twentieth century had just begun. On September 20, Markus Pirpamer and the Austrian Gendarm Anton Koler initiated the first attempt to remove the body from the ice encasement, causing some damages to the corpse’s left hip. With the weather worsening and the lack of necessary tools they were forced to abandon their work. Koler removed an axe that was laying on the edge of the gully ant took it to the gendarmerie post in Soelden. Rumors spread about the fact that several burns and a head wound were visible on the corpse and that he had even been tied up; this finally led to the opening of a criminal enquiry into the man’s identity and the possible involvement of a third party. The enquiry was filed by the Public Prosecutor under the number ST 13 UT 6407/91. On September 21, the famous mountaineers Reinhold Messner and Hans Kammerlander (Fig. 2) reached the Similaun mountain refuge while on a tour of the South Tyrol Mountains along the Austrian–Italian border. They attempted to release the body from ice (Kammerlander used a piece of wood which turned out to be a piece of the Iceman’s backpack frame!). Kurt Fritz, a mountain guide, lifted the dead man’s head to reveal his face for the first time. A journalist urged Messner not to estimate the age of the corpse at more than 500 years. Plenty of hypotheses were formulated: a soldier from World War I, a shepherd, a mercenary after a military debacle during the fifteenth century. On September 22, Alois Pirpamer and Franz Gurschler made their way to the Tisenjoch to prepare the corpse for its recovery chipping it free with ice picks. On September 23, the corpse could finally be extracted from ice. It was packed into a body bag and transferred on a helicopter to Vent in the Austrian Oetz Valley, under the supervision of Rainer Henn, Professor of Forensic Medicine at University of Innsbruck. The Public Prosecutor ordered the corpse to be placed in a wooden coffin and taken in a hearse to the University of Innsbruck Institute of Forensic Medicine. On September 24, the corpse was finally inspected by an archaeologist, Konrad Spindler, Professor of Ancient and Early history at the University of Innsbruck. He immediately dated the whole finding to be “at least 4,000 years old”. Up until this discovery, such a well-preserved ancient finding of a human in complete clothing with numerous Childs Nerv Syst (2008) 24:1083–1088 DOI 10.1007/s00381-008-0702-9