Management of hydrocephalus in children with plasminogen deficiency.

Congenital plasminogen deficiency is an infrequent disorder, which usually becomes symptomatic as ligneous conjunctivitis. However, pseudomembranous lesions in the mucosa of the pharynx, tracheobronchial tree, and the peritoneum may likewise occur. An accompanying hydrocephalus is extremely rare; only 16 cases have been reported to date. The reports indicate that hydrocephalus, even if treated by ventriculoperitoneal (VP) cerebrospinal fluid (CSF) shunting, worsens the prognosis substantially. Thus, VP CSF shunting does not seem to be the optimal therapy for hydrocephalic children with plasminogen deficiency. We add two cases to the literature, and, on the base of our experience, we propose a management strategy for the hydrocephalus. We report the case history of two children with plasminogen deficiency and associated hydrocephalus. Both children initially were treated with VP shunts and had a very similar clinical course with multiple shunt malfunctions due to nonabsorption by the peritoneum. In the first child, the attempt to treat the hydrocephalus with a ventriculoatrial (VA) shunt failed due to catheter thrombosis. Finally, a ventriculocholecystic shunt was placed in both children, which worked well. In patients with plasminogem deficiency and associated hydrocephalus, special care must be taken in the management of hydrocephalus. The absorptive capacity of the peritoneum is reduced by pseudomembrane formation, which results in VP shunt malfunction. The plasminogen deficiency results in early thrombus formation if atrial catheters are used. Therefore, the authors believe that ventriculocholecystic shunting should be considered early on in the course of the disease.

Vascular events after spinal cord injury: contribution to secondary pathogenesis.

Traumatic spinal cord injury results in the disruption of neural and vascular structures (primary injury) and is characterized by an evolution of secondary pathogenic events that collectively define the extent of functional recovery. This article reviews the vascular responses to spinal cord injury, focusing on both early and delayed events, including intraparenchymal hemorrhage, inflammation, disruption of the blood-spinal cord barrier, and angiogenesis. These vascular-related events not only influence the evolution of secondary tissue damage but also define an environment that fosters neural plasticity in the chronically injured spinal cord.

Experience with a new concept to lower non-infectious complications in infants with programmable shunts.

In neonates and infants less than 1 year of age who are treated with a ventriculo-peritoneal shunt, non-infectious complications are almost as frequent and dangerous as infectious complications. While the incidence of infections can be reduced, using perioperative antibiotics, special surgical techniques and postoperative care non-infectious complications such as wound break-down, cerebrospinal fluid (CSF) fistula and subcutaneous CSF collection are preventable, but seem difficult to manage, especially in the group of patients at a susceptible age. The authors present their experience with the programmable Hakim valve in 40 neonates and infants less than 1 year of age, who were treated with de novo implantation of a ventriculo-peritoneal shunt due to various pathologies. The uneventful wound healing during the first weeks after shunt implantation, avoiding the above mentioned non-infectious complications, was supported by initial, temporary overdrainage and readjustment of the programmable valve after completed wound healing. All patients tolerated this procedure well and showed no pathological signs or symptoms of overdrainage like premature closure of cranial sutures, clinical low-pressure syndrome, slit ventricle syndrome (SVS), subdural fluid collection or brain collapse during an average follow-up period of 2.6 years (2 - 65 months). Infectious and other mechanical, non-infectious complications were analysed as well during the follow-up period. These results suggest that an initial, temporary overdrainage in infants and neonates with shunted hydrocephalus may contribute to further lower the incidence of non-infectious complications like wound break-down, CSF-fistula or subcutaneous CSF accumulation, without negative side effects. This technique could be a valuable option in the regimen of shunt-treatment of this age group in order to optimise the overall success rate and lower the general complication rate.

Value of cine phase contrast magnetic resonance imaging to predict obstructive hydrocephalus.

BACKGROUND: This is a prospective study to evaluate the role of CINE MRI to predict obstructive hydrocephalus in the preoperative work-up. PATIENTS/MATERIAL AND METHODS: A total of 16 patients with aqueductal obstruction demonstrated by CINE MRI who were undergoing ETV were included. MRI was performed preoperatively, at 3 months, at 12 months and at 24 months after surgery. Prior to the fenestration of the third ventricular floor aqueductal patency was evaluated using intraoperative ventriculography. A successful outcome was defined by using radiological and clinical criteria. RESULTS: In 8 patients with aqueductal obstruction on preoperative CINE MRI aqueductal patency was proven intraoperatively. ETV failed in all patients with intraoperatively proven aqueductal patency. Out of these 8 patients, 1 patient had no risk factors for ETV failure, 3 had 1 risk factor, 3 had 2 risk factors, and 1 had 3 risk factors. Most of the failure (6 out of 8 patients) occurred within 8 weeks of the initial procedure. A lumbar puncture was performed in these patients to avoid misinterpretation of the clinical course. CONCLUSION: The present study demonstrates that cine phase constrast MR may be a poorer choice to determine aqueductal patency compared to high resolution structural imaging. Interestingly, intraoperative ventriculography was an adjunct to better predict outcome after ETV in patients with obstructive hydrocephalus. In cases with non-conclusive preoperative imaging, postoperative decision making may be supported by the use of intraoperative ventriculography with the goal of reducing unnecessary tests and procedures. However, the analysis of the study data has to be considered as explorative. Therefore, findings should be validated with a larger patient population.

Combined motor and somatosensory evoked potentials for intraoperative monitoring: intra- and postoperative data in a series of 69 operations.

The primary objective of neurophysiologic monitoring during surgery is to avoid permanent neurological injury resulting from surgical manipulation. To prevent motor deficits, either somatosensory (SSEP) or transcranial motor evoked potentials (MEP) are applied. This prospective study was conducted to evaluate if the combined use of SSEP and MEP might be beneficial. Combined SSEP/MEP monitoring was attempted in 100 consecutive procedures, including intracranial and spinal operations. Repetitive transcranial electric motor cortex stimulation was used to elicit MEP from muscles of the upper and lower limb. Stimulation of the tibial and median nerves was performed to record SSEP. Critical SSEP/MEP changes were defined as decreases in amplitude of more than 50% or increases in latency of more than 10% of baseline values. The operation was paused or the surgical strategy was modified in every case of SSEP/MEP changes. Combined SSEP/MEP monitoring was possible in 69 out of 100 operations. In 49 of the 69 operations (71%), SSEP/ MEP were stable, and the patients remained neurologically intact. Critical SSEP/ MEP changes were seen in six operations. Critical MEP changes with stable SSEP occurred in 12 operations. Overall, critical MEP changes were recorded in 18 operations (26%). In 12 of the 18 operations, MEP recovered to some extent after modification of the surgical strategy, and the patients either showed no (n = 10) or only a transient motor deficit (n = 2). In the remaining six operations, MEP did not recover and the patients either had a transient (n = 3) or a permanent (n = 3) motor deficit. Critical SSEP changes with stable MEP were observed in two operations; both patients did not show a new motor deficit. Our data again confirm that MEP monitoring is superior to SSEP monitoring in detecting impending impairment of the functional integrity of cerebral and spinal cord motor pathways during surgery. Detection of MEP changes and adjustment of the surgical strategy might allow to prevent irreversible pyramidal tract damage. Stable SSEP/MEP recordings reassure the surgeon that motor function is still intact and surgery can be continued safely. The combined SSEP/ MEP monitoring becomes advantageous, if one modality is not recordable.

Extended pterional orbital decompression in severe orbital cellulitis.

BACKGROUND: Bacterial orbital cellulitis is a relatively uncommon infective process, which can threaten the function of orbital structures. Apart from antibiotic therapy, sinus surgery with or without abscess drainage via an orbito-otorhinolaryngological approach might be necessary. CASE DESCRIPTION: We present three cases of severe orbital cellulitis, leading to increasing loss of vision, proptosis, afferent pupillary disturbances and restriction of extra-ocular movements, despite antibiotic therapy. After extended pterional orbital decompression and reducing the orbital pressure by removal of the lateral and superolateral orbital walls, all patients showed distinct improvement of initial symptoms, without any complications related to the operation. INTERPRETATION: Extended pterional orbital decompression represents an effective treatment alternative and supplement in cases of a severe, threat to ocular function due to orbital cellulitis, where acute reduction of pressure on orbital, neural and vascular structures is intended.

Occurrence and therapy of space-occupying cystic lesions after brain tumor surgery.

OBJECTIVES: Space-occupying cystic lesions may develop in a variable time after resection of particular intracranial tumors, representing a small amount of complications of these procedures. We present our experience with the development and operative treatment of such postoperative cystic lesions in order to identify possible risk factors and to optimize the treatment. METHODS: The records and neuroradiological findings of patients, operated on either gliomas and meningeomas or craniopharyngeomas, who developed symptomatic cystic lesions in the former tumor resection area during the last ten years, were analyzed. RESULTS: 31 patients (2.5%) out of a total of 1240 corresponding tumor operations were identified. The mean age among the 20 female and 11 male patients was 47 years (12-74 years). In 17 patients (55%) the cystic lesion occured within 6 months after tumor resection (mean 5.6 weeks) and in 14 patients (45%) later than 6 months postoperatively (mean 3.6 years). 22 patients (71%) had malignant tumors and 16 patients (52%) had previous radiation therapy. 14 patients (45%) had more than one tumor resection at the same location and one patient had a postoperative meningitis as predisposing factor for the cyst-formation. All patients profited of the various definitive treatment modalities: repetitive percutaneous puncture/external drainage (5 patients), craniotomy for cyst-resection/-fenestration without (5 patients) and with Rickham-catheter implantation (10 patients), endoscopic cyst-fenestration with Rickham-catheter implantation (3 patients) and implantation of cysto-atrial or cysto-peritoneal shunts (8 patients). CONCLUSIONS: Symptomatic cystic lesions developing after brain tumor resection may occur as early - (5.6 weeks) or as late - (3.6 years) complications and though predisposing factors, like malignant primary tumor, preceding radiation therapy and multiple tumor resections can be identified, the reason for their occurrence remains unclear. A variety of effective therapy options is applicable but should consider the patients condition and prognosis.

MRI and intraoperative findings in cavernous haemangiomas of the spinal cord.

More sensitive imaging techniques, such as MRI, have led to an increase in the number of reported cases of spinal cord cavernous haemangioma (SCCH). Complete surgical resection has been performed with good outcomes. However, operative findings do not always confirm preoperative MRI as to the size and site (superficial or deep) of the lesion. We evaluated whether MRI can be used to predict whether or not SCCH reach the surface of the spinal cord, since this has an impact on surgical strategy. We reviewed the preoperative MRI, case-notes and video recordings of 12 patients who underwent surgery, at which five superficial and seven deep-seated lesions were identified. T1-weighted images correctly indicated the site of the lesion in ten, T2-weighted images in only eight. One deep lesion was thought to be superficial on both T1- and T2-weighted images. Intravenous contrast medium was not helpful in diagnosis or localisation. In no case was a surgically proven superficial lesion interpreted as deep in the spinal cord.