[Nontumoral infantile hydrocephalus. Long-term catamnestic study of 346 cases with cerebrospinal fluid shunt].
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Biomedical subjects
Publications and source records attributed to R Giuffrè.
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A surgical series of 346 children with nontumoral hydrocephalus shunted in the period January 1957 to December 1973 is presented. The etiological factors, preoperative clinical and anatomical status of the patients, their surgical career, the reasons for revision operations and causes of death, are analyzed and discussed in relation to each of the four groups constituting the series: tetraventricular communicating hydrocephalus, triventricular hydrocephalus, obstructive hydrocephalus from ventriculocisternal block and postmeningocele or postmyelomeningocele hydrocephalus. 141 patients required 218 revision operations. 16 patients died after the first operation, 27 on revision and 38 sometime later. 41 patients were lost to follow-up. A recent clinical and instrumental review of the physical, neurological and mental status of the remaining 224 patients showed that 136 (60.7%) are well or very well, 68 (30.3%) have residual deficits likely to regress with treatment and 20 (8.9%) are severely handicapped. These long-term results and the surgical aspects of the problem are discussed in the light of the literature.
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A case of intrasellar teratoma with a germinal structure in a 10-year-old girl is described. A few months after intracranial surgery the tumor differentiated into a choriocarcinoma and finally spread to multiple cerebral, pulmonary, and renal metastases. In the course of choriocarcinomatous evolution, very high urinary levels of luteinizing gonadotropin (HCG) developed, but there was no clinical or anatomical evidence of precocious puberty.
A child with hydrocephalus had two rare complications of a ventriculo-peritoneal shunt: exposure of the abdominal catheter through the surgical incision and perforation of the intestine by a new Raimoundi catheter and its emergence from the anus. No infection occurred. The catheter was replaced with a new one on each occasion, without incident.
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The standard surgical procedures on either peripheral or central nervous system in the treatment of primary spasmodic torticollis are: myotomy, neurotomy, cervical rhizotomy, cortical topectomy, prefrontal lobotomy and a variety of stereotaxic nucleotomies and tractotomies (globus pallidus, thalamus, subthalamus, midbrain). These procedures are briefly reviewed. Some newly proposed operations are than discussed (Svien's labyrinthine suppression and Mazar's interruption of cortico-capsular head-turning pathways). The former surgical intervention is based on physiological and experimental evidence than an altered influence of the reticular substance of the midbrain on the "central" vestibular circuits may be relevant in the genesis of primary spasmodic torticollis. The results are reported of 20 personal cases treated with various surgical procedures with a maximum follow-up of 14.4 years (mean = 6.7 years). It is concluded that at present the most effective and reliable treatment is bilateral anterior rhizotomy of C1-C3 combined with unilateral or bilateral neurotomy (or spinal rhizotomy) of the 11th cranial nerve. The results were rated clinically as useful in 66.6% of the 15 patients treated with this procedure.
Clustering of arachnoid villi along the sagittal sinus gives rise to the so-called "Pacchionian Granulations". These structures were originally described in 1705 by Antonio Pacchioni, an Italian scientist. Born in Reggio Emilia in 1665, he graduated in Medicine in his hometown and later moved to Rome were he built up a successful career by dedicating to medical practice as well as to research and teaching. He became friend and often collaborated with some of the leading scientists of his age: Lancisi, Malpighi and Morgagni among the others. Moreover he carefully followed research developments in Europe as testified by frequent quoting of foreign authors in his works. He devoted himself to the elucidation of structure and function of Dura Mater, often by using new techniques of maceration of anatomic specimens in various fluids. Among Pacchioni's works, the "Dissertatio epistolaris de glandulis conglobatis Durae Meningis humanae" (1705) is particularly well known and contains the first description of arachnoidal granulations. He compared Dura to cardiac muscle and attributed to its "glandulae" (glands) the faculty of secreting lymph for lubrification of the sliding movements between meninges and brain during contractions. He died in Rome in 1726. Three centuries after Pacchioni's death fine structure of arachnoid villi hasn't been fully elucidated; moreover many questions on mechanisms underlying CSF absorption remain unanswered.
The genesis and development of a CSDH are presently explained in terms of a) neoformation of a subdural membrane; b) abnormal vascular permeability; c) defective local hemostasis (hyperfibrinolysis) and chronic rebleeding. Many etiological factors are involved, mechanical, hematogenic and vasogenic in nature. Both clinical and experimental evidence supports the hypothesis that hormonal factors, in addition to the above mentioned ones, could play a role in the pathogenesis of CSDHs.
A surgical series of 144 infantile triventricular hydrocephalus (from nontumoral aqueductal stenosis) operated on in the period 1957-1982 is presented. The etiological factors, preoperative clinical and anatomical status of the patients are summarized. Their surgical career, the reasons, for revision operations and causes of death are analyzed in relation to each of the four operations performed: ventriculocisternostomies according to Stookey-Scharff or Torkildsen, extrathecal shunts (VA or VP). 46 patients required 72 revision operations, but 23 more patients were lost to follow-up after the first operation. 3 patients (2.0%) died after the first operation, 7 (4.8%) on revision and 18 (12.5%) sometime later. A recent clinical and instrumental review of the physical, neurological and mental status of the remaining 93 patients (64.5% of the total series) has showed that 50 (34.8%) are well or very well; 35 (24.3%) have residual deficits, and 8 (5.4%) are severely handicapped.
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