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Spiros Sgouros and Bernard Williams

✓ Traumatic paraplegia is the most common cause of nonhindbrain—related syringomyelia. Fifty-seven patients with a mean age of 34.3 years at presentation were treated at the Midland Centre for Neurosurgery and Neurology between 1973 and 1993. A variety of treatment strategies have been used over the years, including syringosubarachnoid and syringopleural shunts, spinal cord transection, and pedicled omental graft transposition. More recently decompressive laminectomy, subarachnoid space reconstruction and formation of surgical meningocele have been used. A total of 81 operations were performed in these patients, 69 of them at the Syringomyelia Clinic. Combinations of strategies were often chosen; the use of one strategy such as drainage did not preclude another such as transection or augmentation of the cerebrospinal fluid pathways.

The overall postoperative complication rate was 12%. Problems specific to the operation type included dislodged, blocked, and infected drains (10 patients). Acute gastric dilation was seen following pedicled omental graft (one patient). At 6 years only 49% of the drains inserted still functioned. A higher than expected rate of cervical spondylotic myelopathy has been noted. Two patients developed Charcot's joints. Thirty-six patients were asked to score themselves with regard to limb function and performance of daily living activities and 30% reported improvement, particularly in arm function.

Since the use of magnetic resonance imaging has become widespread, it has become apparent that decompressive laminectomy with subarachnoid space reconstruction is effective in controlling the syrinx cavity. In complete paraplegia, spinal cord transection is an effective alternative. Pedicled omental grafting was associated with poor outcome and an increased complication rate and has been abandoned.

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Spiros Sgouros and Bernard Williams

✓ The use of drains in the treatment of syringomyelia has a simple and immediate appeal and has been practiced widely since the report of Abbe and Coley over 100 years ago. Good short-term results have been claimed in the past, but long-term outcome is largely unknown. An experience in Birmingham, England is reviewed in which 73 patients who had had some form of syrinx drainage procedure performed were subsequently followed up. In these cases, a total of 56 syringopleural and 14 syringosubarachnoid shunts had been inserted. Ten years after the operations, only 53.5% and 50% of the patients, respectively, continued to remain clinically stable. A 15.7% complication rate was recorded, including fatal hemorrhage, infection, and displacement of the drain from the pleural and syrinx cavities. At second operation or necropsy, at least 5% of shunts were discovered to be blocked. The effect of other drainage procedures that do not use artificial tubing, such as syringotomy and terminal ventriculostomy, was analyzed but found not to offer any substantial benefit. These results indicate that drainage procedures are not an effective solution to remedying the progressive, destructive nature of syringomyelia. It is suggested that, rather than attempting to drain the syrinx cavity, disabling the filling mechanism of the syrinx is more appropriate. Most forms of syringomyelia have a blockage at the level of the foramen magnum or in the subarachnoid space of the spine. Surgical measures that aim to reconstruct the continuity of the subarachnoid space at the site of the block are strongly recommended. Lowering the overall pressure of the cerebrospinal fluid is advocated when reestablishment of the pathways proves impossible.

Syrinx drainage as an adjuvant to more physiological surgery may have a place in the treatment of syringomyelia. If two procedures are done at the same time, however, it is difficult to ascribe with certainty a success or failure, and it is suggested that the drainage procedure be reserved for a later attempt if the elective first operation fails.

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Anne-Laure Boch, Éric Hermelin, Christian Sainte-Rose and Spiros Sgouros

Object. The authors studied new and calcified shunt catheters to identify the prevalence of failures caused by aging materials in the shunt. Complications associated with these devices have various origins. Among late complications, fracture or migration of the system is related to the subcutaneous adhesion of the distal tubing in a growing child. A review of a cohort of 64 children who underwent shunt placement in 1980 with barium-impregnated distal catheters showed that 10 of these patients underwent reoperation for complications related to aging of the shunt material. This group represents 15% of the whole series and 30% of those children who were followed for more than 3 years. The true impact of aging of materials on shunt function is probably underestimated.

Methods. The authors performed physical, chemical, and mechanical analyses of the retrieved aged catheters and also of new catheters, resulting in the following findings: 1) calcifications were observed only on the external surface of the catheter, predominantly in its subcutaneous segment at the level of the neck and anterior chest wall; 2) calcifications contained particles of free silicon and barium sulfate, signifying fragmentation of the polymer; 3) the microstructure of the silicone polymer was modified: microfractures and alteration of the polymeric network were observed; 4) silanol groups were observed on the external surface of the catheter; and 5) the mechanical properties of the silicone rubber were degraded, and the aged catheters were more brittle than the new ones, with ruptures at elongations and fracture energy much lower than that seen in new catheters.

Furthermore, in vitro testing with a metastable solution of simulated body fluid demonstrated the critical impact of pH variations in liquid media and surface degradation of the catheters on the precipitation of hydroxylapatite crystals.

Conclusions. Although most shunt complications can be addressed by better patient management and surgical technique, late complications appear to be partly related to aging of the material. Distal tubing calcifications have been observed in barium-impregnated catheters. The industry recently responded to these observations by introducing plain silicone-coated shunt tubing; further evaluation will show what improvement can be expected.

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Giuseppe Cinalli, Christian Sainte-Rose, Isabelle Simon, Guillaume Lot and Spiros Sgouros

Object

This study is a retrospective analysis of clinical data obtained in 28 patients affected by obstructive hydrocephalus who presented with signs of midbrain dysfunction during episodes of shunt malfunction.

Methods

All patients presented with an upward gaze palsy, sometimes associated with other signs of oculomotor dysfunction. In seven cases the ocular signs remained isolated and resolved rapidly after shunt revision. In 21 cases the ocular signs were variably associated with other clinical manifestations such as pyramidal and extrapyramidal deficits, memory disturbances, mutism, or alterations in consciousness. Resolution of these symptoms after shunt revision was usually slow. In four cases a transient paradoxical aggravation was observed at the time of shunt revision. In 11 cases ventriculocisternostomy allowed resolution of the symptoms and withdrawal of the shunt.

Simultaneous supratentorial and infratentorial intracranial pressure recordings performed in seven of the patients showed a pressure gradient between the supratentorial and infratentorial compartments with a higher supratentorial pressure before shunt revision. Inversion of this pressure gradient was observed after shunt revision and resolution of the gradient was observed in one case after third ventriculostomy. In six recent cases, a focal midbrain hyperintensity was evidenced on T2-weighted magnetic resonance imaging sequences at the time of shunt malfunction. This rapidly resolved after the patient underwent third ventriculostomy.

It is probable that in obstructive hydrocephalus at the time of shunt malfunction, the development of a transtentorial pressure gradient could initially induce a functional impairment of the upper midbrain, inducing upward gaze palsy. The persistence of the gradient could lead to a global dysfunction of the upper midbrain.

Conclusions

Third ventriculostomy contributes to equalization of cerebrospinal fluid pressure across the tentorium by restoring free communication between the infratentorial and supratentorial compartments, resulting in resolution of the patient's clinical symptoms.

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Giuseppe Cinalli, Christian Sainte-Rose, Isabelle Simon, Guillaume Lot and Spiros Sgouros

Object. This study is a retrospective analysis of clinical data obtained in 28 patients affected by obstructive hydrocephalus who presented with signs of midbrain dysfunction during episodes of shunt malfunction.

Methods. All patients presented with an upward gaze palsy, sometimes associated with other signs of oculomotor dysfunction. In seven cases the ocular signs remained isolated and resolved rapidly after shunt revision. In 21 cases the ocular signs were variably associated with other clinical manifestations such as pyramidal and extrapyramidal deficits, memory disturbances, mutism, or alterations in consciousness. Resolution of these symptoms after shunt revision was usually slow. In four cases a transient paradoxical aggravation was observed at the time of shunt revision. In 11 cases ventriculocisternostomy allowed resolution of the symptoms and withdrawal of the shunt.

Simultaneous supratentorial and infratentorial intracranial pressure recordings performed in seven of the patients showed a pressure gradient between the supratentorial and infratentorial compartments, with a higher supratentorial pressure before shunt revision. Inversion of this pressure gradient was observed after shunt revision and resolution of the gradient was observed in one case after third ventriculostomy. In six recent cases, a focal midbrain hyperintensity was evidenced on T2-weighted magnetic resonance imaging sequences at the time of shunt malfunction. This rapidly resolved after the patient underwent third ventriculostomy.

Conclusions. It is probable that in obstructive hydrocephalus, at the time of shunt malfunction, the development of a transtentorial pressure gradient could initially induce a functional impairment of the upper midbrain, inducing upward gaze palsy. The persistence of the gradient could lead to a global dysfunction of the upper midbrain. Third ventriculostomy contributes to equalization of cerebrospinal fluid pressure across the tentorium by restoring free communication between the infratentorial and supratentorial compartments, resulting in resolution of the patient's clinical symptoms.

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Spiros Sgouros, J. Henry Goldin, Anthony D. Hockley, Michael J. C. Wake and Kalyan Natarajan

Object. The goal of this study was to construct a model of normal changes in intracranial volume occurring throughout childhood from age 7 days to 15 years.

Methods. Using the technique of segmentation on magnetic resonance images obtained in healthy children, intracranial volume was measured and plotted against age.

Conclusions. Intracranial volume in the first few months of life is on average 900 cm3 in males and 600 cm3 in females. By the age of 15 years, it increases up to 1500 cm3 in males and 1300 cm3 in females, increased by factors of 1.6 and 2.1, respectively. By the time the child reaches 2 years of age, intracranial volume has reached 77% (1150 cm3 in males and 1000 cm3 in females) and, by 5 years, 90% (1350 cm3 in males and 1200 cm3 in females) of the volume observed at age 15 years. The change in intracranial volume that occurs with age is not linear, but there seems to be a segmental pattern. Three main periods can be distinguished, each lasting approximately 5 years (0–5, 5–10, and 10–15 years), during which the growth of intracranial volume is linear. Throughout childhood, males have higher intracranial volumes than females, with a similar growth pattern.

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Spiros Sgouros, Anthony D. Hockley, J. Henry Goldin, Michael J. C. Wake and Kalyan Natarajan

Object. There is still controversy regarding the optimum time to perform surgery for craniosynostosis. Some recommend surgery soon after birth and others delay until the age of 12 months. Intracranial pressure has been measured in an attempt to provide a scientific rationale, but many questions remain unanswered. To date, little attention has been given to intracranial volume and its changes during the first few years of life in children with craniosynostosis. The authors' goal was to focus on intracranial volume during this period and to compare measurements obtained in patients with craniosynostosis with measurements obtained in healthy individuals.

Methods. Using the technique of segmentation, the intracranial volume of 84 children with various forms of craniosynostosis was measured on preoperative computerized tomography scans. The change in average volume that occurs with increasing age was calculated and compared with a model of normal intracranial volume growth. The age at presentation for children with craniosynostosis was 1 to 39 months; 76% of the patients were younger than 12 months. In eight patients in whom only one cranial expansion procedure was performed, postoperative intracranial volumes were measured as well. Several interesting observations emerged. 1) There was little difference in head growth between boys and girls with craniosynostosis during the first few months of life. After the age of 12 months, however, the difference in intracranial volume normally seen between the two genders was observed in the craniosynostosis group as well. 2) Excluding children with complex pansynostosis, who have smaller heads, children with all other types of craniosynostosis have similar head growth after the 1st year of life, with no difference between the number of and type of suture affected. Children with Apert's syndrome develop greater than normal intracranial volumes after the 1st year of life. 3) Although children with craniosynostosis are born with a smaller intracranial volume, by the age of 6 months volume has reached normal levels, and from that point on volume follows the pattern of normal head growth. 4) Children who presented after the age of 6 months and later developed recurrent craniosynostosis after initial successful treatment had a small intracranial volume at their initial presentation. 5) Of the patients whose postoperative intracranial volumes were measured, all but one had preoperative volumes at or above normal values, and their postoperative volumes were considerably higher than normal for their age. These children all followed a growth curve parallel to that of healthy children but at higher volume value. One patient with a smaller-than-normal initial intracranial volume was surgically treated at a very young age and, despite cranial expansion surgery, postoperative volume did not reach normal levels. It is postulated that this was due to the fact that the operation was performed at a time when craniosynostosis was still active.

Conclusions. The results of this study indicate that the underlying mechanism leading to craniosynostosis and constriction of head volume “exhausts” its effect during the first few months of life. Measurement of intracranial volume in clinical practice could be used to “fine tune” the optimum time for surgery. In late-presenting children, this may be useful in predicting possible recurrence.

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Chris Xenos, Spiros Sgouros, Kalyan Natarajan, A. Richard Walsh and Anthony Hockley

Object. The goal of this study was twofold: to investigate the change in ventricular volume in children with hydrocephalus in response to shunt placement and to assess the effects of two different valve types (Medium Pressure [MP] cylindrical valve and Delta [model 1.5] valve).

Methods. Ventricular volume was measured using segmentation techniques on computerized tomography scans and magnetic resonance images obtained in 40 children with hydrocephalus who ranged in age from 4 days to 16 years. Imaging was performed preoperatively and at 5 days and 3, 6, and 12 months postoperatively. The results were compared with measurements obtained in 71 healthy children ranging in age from 1 month to 15 years. Each ventricular volume that was measured was divided by the corresponding sex and age—related mean normal volume to calculate the “× normal” ventricular volume, indicating how many times larger than normal the ventricle was.

The mean preoperative ventricular volume was 232 cm3 (range 50–992 cm3). The mean postoperative volumes were 147, 102, 68, and 61 cm3 at 5 days and at 3, 6, and 12 months posttreatment, respectively. The mean preoperative × normal ventricular volume was 14.5 (range 2.2–141.7), and the mean postoperative × normal volumes were 7.9, 5.6, 3.5, and 2.9 at 5 days and 3, 6, and 12 months postimplantation, respectively. The rate of volume reduction was consistently higher in patients who received the MP valve in comparison with those who received the Delta valve, both for new shunt insertions and for shunt revisions. The difference between the two valve groups did not reach statistical significance. Two patients in whom ventricular volumes increased during the study period experienced shunt obstruction at a later time.

Conclusions. Preoperative ventricular volume in children with hydrocephalus can be up to 14 times greater than normal. In response to shunt placement, the ventricular volume continues to fall during the first 6 months after operation. The effect is more profound in children who receive the MP valve than in those who receive the Delta valve, although in this study the authors did not demonstrate statistical significance in the difference between the two valves. Nevertheless, this may indicate that the MP valve produces overdrainage in comparison with the Delta valve, even within the first few months after insertion. There is some indication that sequential ventricular volume measurement may be used to identify impending shunt failure.

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Philippe Pencalet, Christian Sainte-Rose, Arielle Lellouch-Tubiana, Chantal Kalifa, Francis Brunelle, Spiros Sgouros, Philippe Meyer, Giuseppe Cinalli, Michel Zerah, Alain Pierre-Kahn and Dominique Renier

Object

Choroid plexus tumors are rare intraventricular tumors (1% of all intracranial tumors) that occur mainly in children. The physiopathological characteristics of associated hydrocephalus, surgical management, and oncological issues related to these tumors remain a matter of debate. To understand more about these tumors, the authors have reviewed their experience with the management of 38 children with choroid plexus tumors.

Methods

There were 25 cases of papilloma and 13 of carcinoma. The mean age of the patients at presentation was 22.5 months and one-half of the patients were younger than 2 years of age. Hydrocephalus was present in 33 patients and poorly correlated with the size, site, and pathological characteristics of the tumor. In nine children, a ventriculoperitoneal shunt was required after tumor excision, calling into question the notion that cerebrospinal fluid oversecretion is the only cause of hydrocephalus.

Complete excision was achieved in 96% of the cases of papilloma and 61.5% of the cases of carcinoma. These surgical procedures were complicated by the risks of perioperative hemorrhage, which proved to be fatal in two cases, and postoperative brain collapse, which led to subdural fluid collections requiring subdural shunt placement in six patients. Preoperative embolization was partially successful in four cases and significantly assisted surgery. Preoperative controlled drainage of excessively dilated ventricles and intraoperative gluing of the cortical incision have been used to address the problem of postoperative brain collapse.

Patients with carcinomas were treated postoperatively by chemotherapy alone (seven cases), radiotherapy (one case), or chemotherapy plus radiotherapy (one case). The overall 5-year survival rate was 100% for patients with papillomas and 40% for those with carcinomas.

Conclusions

Total surgical excision is curative in cases of papillomas. For carcinomas, the most effective treatment remains total surgical excision; however, adjuvant treatment in the form of chemotherapy in patients younger than 3 years, supplemented by radiation therapy in older children, can moderately reduce the risk of recurrence.