Complications of lumboperitoneal shunts. A retrospective study of a series of 195 patients (214 procedures)

BACKGROUND AND PURPOSE: We report our experience with the lumboperitoneal shunt (LPS) in 195 patients. The aim of this retrospective study was to assess and compare the rate of complications and to discuss 4 indications. MATERIAL AND METHOD: Between January 1983 and July 1994, 195 patients including 14 pediatric cases were treated with a LPS. Sex ratio was 1.24. The mean age at insertion was 59.5 years (from 6 months to 88 years) and the follow-up in this series was from 6 months to 12.5 years. The indications for a LPS were: chronic idiopathic hydrocephalus (115 cases), post-hemorrhagic hydrocephalus (37 cases), cerebrospinal fluid fistula (11 cases), post-traumatic hydrocephalus (9 cases), post-surgical hydrocephalus (8 cases), hydrocephalus of the child (6 cases), post-meningitis hydrocephalus (4 cases), benign intracranial hypertension (4 cases), post-radiotherapy hydrocephalus (1). Forty patients (20.5%) presented with at least one complication. A total of 47 complications were observed: chronic subdural effusion (8 cases), meningitis (10 cases), mechanical failures (28 cases), acquired Chiari abnormality (1 case). Mechanical complications varied with the type of shunt. CONCLUSION: Complications of LPS in adults are less frequent than is usually reported after ventricular atrial or peritoneal shunting. In adults, LPS can be used as the first valuable treatment in case of chronic communicating hydrocephalus. LPS is also valuable in the treatment of benign intracranial hypertension or recurrent CSF fistulae. Conversely, in the pediatric cases general and specific complications are frequent, so an indication for LPS must be strictly discussed.     

Hydrocephalus in mice infected with a Theiler's murine encephalomyelitis virus variant

The etiology of hydrocephalus is never established in the majority of clinical cases, while various agents, nutritional deficiencies, and genetic factors have been shown to play a role. Viral infection has been recognized as one of the causative factors in the development of hydrocephalus. The wild-type DA strain of Theiler's murine encephalomyelitis virus (TMEV), which belongs to the family Picornaviridae, causes a chronic demyelinating disease in mice with viral persistence that resembles multiple sclerosis. We found that a DA virus variant, hydrocephalus 101 virus (H101 virus), caused hydrocephalus in mice, a condition previously never described for TMEV. To clarify the relationship between DA virus infection and hydrocephalus, we compared H101 virus and wild-type DA virus infection in mice. Using immunohistochemistry and terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick-end labeling (TUNEL), we found that during the acute phase of infection, H101 virus caused macrocephaly and meningitis with the presence of apoptosis, while parenchymal involvement was not evident. In contrast, wild-type DA virus caused an acute polioencephalomyelitis with parenchymal infection and apoptosis. During the chronic phase, H101 virus infection caused communicating hydrocephalus without viral persistence. No demyelination and little or no anti-TMEV antibodies were observed in H101 virus-infected mice. Sequence analysis revealed that H101 virus had mutations in the 5'UTR and capsid protein coding region. Characterization of this new hydrocephalus model gives insight into the possible viral involvement in human hydrocephalus cases of obscure etiology.     

Extralobar pulmonary sequestration presenting as a mediastinal malignancy

OBJECTIVE: We intended to characterize the CT patterns of hemorrhage associated with ruptured posterior inferior cerebellar artery (PICA) aneurysms. MATERIALS AND METHODS: CT scans of 44 cases of angiographically confirmed ruptured saccular PICA aneurysms (4) aneurysms at the junction of the vertebral artery and the PICA and three distal PICA aneurysms) were retrospectively reviewed. All scans had been obtained within 2 days of the subarachnoid hemorrhage (SAH) (day 0 [less than 24 hr], 35 patients; day 1, eight patients; day 2, one patient). Presence or absence of hemorrhage in specific subarachnoid, intraventricular, and intraparenchymal locations was noted, as were the presence and degree of hydrocephalus. RESULTS: Posterior fossa SAH was present in 95% of cases. Isolated posterior fossa SAH was present in 30% of cases, but in no case was isolated supratentorial SAH present. Supratentorial SAH was present in 70% of cases. SAH involving the sylvian fissure or the interhemispheric region was present in 25% and 23% of cases, respectively. SAH along the convexity was present in 2% of cases. Intraventricular hemorrhage (IVH) with or without associated SAH was seen in 95% of cases, whereas isolated IVH was seen in 5% of cases. Hydrocephalus was present in 95% of cases and was moderate to marked in 70%. Both IVH and hydrocephalus were present in 93% of cases. CONCLUSION: Ruptured PICA aneurysms almost always coexist with hydrocephalus and IVH, as seen in 93% of cases, and almost never coexist with SAH along the convexity. The most common pattern of hemorrhage associated with such aneurysms includes IVH and posterior fossa hemorrhage. Extensive supratentorial SAH, in conjunction with posterior fossa SAH, is a common finding in patients with ruptured PICA aneurysms. SAH isolated to the posterior fossa is present in a sizeable minority of cases.     

Inhibition of cytochrome P-450 3A (CYP3A) in human intestinal and liver microsomes: comparison of Ki values and impact of CYP3A5 expression

Seckel syndrome has been described as the prototype of the primordial bird-headed type of dwarfism. Since Seckel originally defined the disorder, less than 60 cases have been reported. In addition to the characteristic craniofacial dysmorphism and skeletal defects, abnormalities have been described in the cardiovascular, hematopoietic, endocrine, and central nervous systems. This pleiotropy has implied genetic heterogeneity and prompted reviews of previously reported cases of Seckel syndrome. As a result, the characteristic diagnostic features of Seckel syndrome have been highly debated. Although deletions in chromosome 2q have been described, to date, no genetic defect has been defined. We report three cases of Seckel-like syndrome in siblings from nonconsanguinous Caucasian parents. In addition to the typical Seckel phenotypic features, all three cases were characterized by severe hydrocephalus. We review the literature and propose that there is a spectrum of Seckel conditions that share some common key features, but also demonstrate a wide range of phenotypic features.     

Acquired neutrophil dysfunction and diseases with an inflammatory component

We describe nine children with a similar pattern of features including macrocephaly and cutis marmorata telangiectatica congenita. All were large at birth and had a distinctive capillary haemangioma involving the philtrum and upper lip. The seven who survived all developed hydrocephalus and had developmental delay. Six developed body asymmetry and three had internal arteriovenous malformations. Syndactyly of the second and third toes and/or the third and fourth fingers or toes was commonly seen. All of the cases were sporadic. This condition is easily recognizable and should be considered in the differential diagnosis of patients presenting with overgrowth and macrocephaly.     

Nephritis following shunt operation for hydrocephalus

The ventriculo-atrial (VA) and ventriculo-peritoneal (VP) shunts implanted due to hypertensive hydrocephalus might get infected and be complicated by shunt nephritis. We reviewed the morphological changes in kidney retrospectively in the autopsy materials of our institute for the last 15 years after shunt implantation. Histological examination of the kidney was performed in 26 of these cases having 7 cases with glomerular morphological changes. Thus, the shunt nephritis is a relatively frequent complication of VA or VP shunts implanted due to hydrocephalus. Monitorization and follow-up of kidney function in case of any dysfunction of the shunt may suggest the possibility of shunt infection, and the removal of the infected shunt may protect the development of more severe, septic complications.     

Retropharyngeal pseudomeningocele after atlanto-occipital dislocation: report of two cases

OBJECTIVE AND IMPORTANCE: We report two cases of retropharyngeal pseudomeningocele after atlanto-occipital dislocation. This is rare, with only one other reported case in the literature. CLINICAL PRESENTATION: We report two patients who presented after blunt cervical and head trauma. Plain films revealed that each patient had atlanto-occipital dislocation. Subsequent magnetic resonance imaging revealed the delayed development of retropharyngeal pseudomeningocele. Concomitant hydrocephalus was noted in both patients. INTERVENTION: The surviving patient showed marked neurological improvement and resolution of his pseudomeningocele after ventriculoperitoneal shunting. CONCLUSION: For patients with closed head injuries who develop posttraumatic pseudomeningocele, we recommend cranial computed tomography to assess for the presence of hydrocephalus. In patients with atlanto-occipital dislocation, delayed neurological deterioration warrants magnetic resonance imaging of the craniocervical junction to rule out posttraumatic pseudomeningocele.     

Cephalocranial disproportion. A complication of the treatment of hydrocephalus in children

Eight children with hydrocephalus showed signs of posterior fossa tonsillar impaction despite arrest of the hydrocephalus and patency of their CSF shunts. Contrast studies performed because of their clinical deterioration demonstrated cerebellar tonsillar herniation, and at posterior fossa decompression, this was confirmed in all cases. Improvement followed decompression. The pathogenesis of a state of cephalocranial disproportion following successful treatment of hydrocephalus resulting in symptomatic tonsillar herniation, is discussed.     

Frontal foramina in pediatric skull in cases of congenital hydrocephalus

PURPOSE: To describe a new observation, frontal calvarial foramina, in pediatric patients with congenital hydrocephalus secondary to central nervous system malformation. MATERIALS AND METHODS: Frontal foramina were initially identified in three female patients with Chiari II malformation. Subsequently, head computed tomographic (CT) scans in 99 patients with congenital hydrocephalus were retrospectively reviewed. CT scans in a control group of 116 patients without hydrocephalus were also retrospectively reviewed. RESULTS: Frontal foramina were found in eight of 61 (13%) patients with Chiari II malformation, in one child with Dandy-Walker malformation, and in one child with occipital horn dilatation (colpocephaly), but not in control patients. Sequential CT examinations in three patients with frontal foramina depicted gradual closure after ventriculoperitoneal shunt placement. CONCLUSION: Frontal foramina may represent an abnormality variably expressed in certain central nervous system malformations that cause congenital hydrocephalus. The presence of frontal foramina palpated or visualized on plain radiographs may help in the diagnosis of congenital hydrocephalus and central nervous system malformation.     

Severe fetomaternal alloimmune thrombocytopenia presenting with fetal hydrocephalus

We report two patients where the finding of isolated fetal hydrocephalus led to the detection of severe fetal thrombocytopenia, using fetal blood sampling. Serological investigation led to the diagnosis of fetomaternal alloimmune thrombocytopenia (FMAIT) due to anti-HPA-1a. Both women had had previous unsuccessful pregnancies probably due to FMAIT; one had had four miscarriages at 17-18 weeks' gestation. The other had had one previous pregnancy complicated by severe fetal anaemia, and eventually hydrocephalus developed and the fetus died without the diagnosis of FMAIT being considered. Subsequent pregnancies in the two women were also affected by FMAIT, but prenatal treatment, predominantly with serial fetal platelet transfusions, resulted in a successful outcome in both cases. These observations suggest that FMAIT should be suspected if there is isolated fetal hydrocephalus, unexplained fetal anaemia, or recurrent miscarriages. The accurate diagnosis of FMAIT is important because recent advances in prenatal management can improve the outcome of subsequently affected pregnancies.     

合并梗阻性脑积水的松果体区脑膜瘤的手术治疗

Objective:The aim of our study was to find out the best operative therapeutic regimen by reviewing the comprehension of operation, the improvement of operative method and the selection of operative approach concerning pineal region meningioma combining obstructive hydrocephalus. Methods:We retrospected the clinic data, comprehension of operation and prognosis of 6 cases of pineal region meningioma. Results:Six tumors were all removed, after that the back wall of the up, the manifestation of hydrocephalus disappeared, other symptoms were relieved more or less without any complication with one patient dismissing. The regular MRI examination indicated that the lesions had not reoccur and the cerebral ventricles had been normal. Conclusion:We identify the nourish arteries of the tumor and anatomy relationship between the tumor and its peripheral blood vessels through modern imaging devices, to select reasonable operative method are the premier therapy of pineal region meningioma. The back wall of the third ventricle becomes thin and the suprapineal recess develops shallow and wide, so the third ventricle-quadrigeminal cistern fistulation can effectively eliminate the obstructive hydrocephalus due to the long-term compression on the aqueduct after removing the tumor.     

Focal dilation and paradoxical collapse of cortical fissures and sulci in patients with normal-pressure hydrocephalus

OBJECT: The authors describe a subgroup of patients with shunt-proven normal-pressure hydrocephalus (NPH) who presented with focal fissural and sulcal dilation on imaging studies. The specific radiological features and methods of differentiating this condition from cortical atrophy are delineated. METHODS: Normal-pressure hydrocephalus has been described as dilation of the ventricles that is out of proportion to the sulci. Sulcal dilation has been taken as evidence of cortical atrophy and has even been used as a criterion to exclude patients from undergoing a shunting procedure. The authors describe five cases of patients with shunt-proven NPH who presented with focal dilation of cortical fissures and sulci. In three of the cases, there was a paradoxical decrease in the size of the dilated fissures and sulci that paralleled the decrease in the size of the lateral ventricles following successful shunting. CONCLUSIONS: This study demonstrates that focal fissural and sulcal dilation may represent reservoirs of cerebrospinal fluid analogous to the ventricular system. Patients should not be denied a shunting procedure solely on the basis of focally dilated fissures of sulci.     

Intramedullary brain stem cyst and trapped IV ventricle after infection with Listeria monocytogenes

We present the rare case of a girl surviving intrauterine listeria brain stem meningoencephalitis, who subsequently developed hydrocephalus, a trapped IV ventricle and an intramedullary cyst. Such cases have been reported only infrequently, and in earlier cases modern imaging studies were not available. Magnetic resonance imaging has been helpful in our patient to delineate the lesions and plan further treatment.     

MOS-SF-36 in evaluating health-related quality of life in alcohol-dependent patients

OBJECT: It is possible to diagnose hydrocephalus prenatally based on the morphological appearance of the fetus on neurodiagnostic images; however, the prognosis of this disease shows wide variation. The authors previously proposed a classification system for the prediction of postnatal outcome based on progression of hydrocephalus and affected brain development, known as the "Perspective Classification of Congenital Hydrocephalus (PCCH)." In this study the authors have used their classification system to analyze long-term follow-up results obtained in each clinicoembryological stage of fetal hydrocephalus. METHODS: Sixty-one fetuses with hydrocephalus were examined to predict postnatal outcome by using this newly developed classification. The authors' recently developed method of using heavily T2-weighted imaging with a superconducting magnet clearly delineated the cerebrospinal fluid (CSF) space and the malformed brain and spinal cord. Imaging was achieved in less than 1 second per slice and required no sedation of the fetus. The technique appears to be simple and good at delineating intrauterine anatomy. Hydrocephalus was diagnosed in two fetuses at PCCH embryological Stage I (8-21 gestational weeks), in 28 fetuses at Stage II (22-31 weeks), and in 31 fetuses at Stage III (32-40 weeks). Among these 61 fetuses, clinicopathological typing showed that 19 had primary hydrocephalus (nine in Stage II and 10 in Stage III), 34 had dysgenetic hydrocephalus (two in Stage I, 16 in Stage II, and 16 in Stage III), and eight had secondary hydrocephalus (three in Stage II and five in Stage III). When the hydrocephalic state developed during PCCH Stage I or II, the prognosis was very poor, and only one of 18 fetuses with dysgenetic hydrocephalus and none of three fetuses with secondary hydrocephalus had an acceptable postnatal outcome. Even within the same category or subtype of fetal hydrocephalus, such as primary hydrocephalus in its simple form, or hydrocephalus with spina bifida aperta (myeloschisis), the postnatal outcomes differed depending on the time of onset of hydrocephalus. When the diagnosis of hydrocephalus was made during PCCH Stage II, the fetuses had a poorer postnatal outcome compared with those at Stage III (p < 0.05). CONCLUSIONS: It is emphasized that postnatal prognosis is not simply a function of the form of the diagnosis but is also dependent on the progression of hydrocephalus and the degree to which that process affects neuronal development. Early decompressive procedures, conventionally performed after but, hopefully, performed before birth, are indicated to obtain the optimal postnatal prognosis of fetuses with hydrocephalus diagnosed at PCCH Stage II.     

Hydrocephalus in cerebral cysticercosis. Pathogenic and therapeutic considerations

The cases of 11 patients with hydrocephalus secondary to cerebral cysticercosis are analyzed. Most of the patients had suffered from epilepsy before they developed hydrocephalic symptoms, and computerized tomography showed that infestation of the parenchyma coexisted with ventricular or cisternal colonization. In four cases, the parasitic vesicles compromised cerebrospinal fluid (CSF) flow in the ventricular system, resulting in internal hydrocephalus. Communicating hydrocephalus, caused by the presence of Cysticercus larvae in the basal cisterns (Cysticercus racemosus), or by the occurrence of a chronic basal meningitis, or both, developed in seven more patients. Changes in CSF pressure were related to the number and location of the cysts and to the leptomeningeal inflammatory reactions evoked by them. The majority of patients presented with a chronic and relatively normotensive hydrocephalus. All patients except one had identifiable ventricular or cisternal Cysticercus larvae; these patients were treated with open removal of the cysts, and did well. However, most of them had impairment of CSF flow through the basal cisterns and required permanent CSF shunting. Communicating hydrocephalus due to leptomeningeal scarring was also successfully managed with extracranial shunting. Epilepsy was controlled with anticonvulsant therapy. Although good lasting results may be obtained with aggressive treatment of neurocysticercosis, patients are liable to relapse because surgery is only palliative in most instances.     

Management of 50 spontaneous cerebellar haemorrhages. Importance of obstructive hydrocephalus

The records of 50 cerebellar haemorrhages were reviewed retrospectively. In this series the most important factor for clinical development, management and mortality was the presence of obstructive hydrocephalus (p < 0.01). Slowly progressive (type 1) and abruptly developing (type 2) deterioration of consciousness was significantly related to high mortality; this holds also true for the combination of hydrocephalus with an haematoma diameter > 3 cm. Larger haematomas had a higher mortality but this relation, analyzed alone, did not reach statistical significance (p > 0.05). In cases with hydrocephalus mortality could significantly be reduced by surgical evacuation of the haematoma (p < 0.01). The treatment of cerebellar haemorrhages must be directed at resolving obstructive hydrocephalus.     

Chromosome 1p terminal deletion: report of new findings and confirmation of two characteristic phenotypes

We report three unrelated patients with small terminal deletions involving 1p36.22-->pter that occurred de novo and compare our patients to the 10 previously reported cases. Although our patients have an identical cytogenetic deletion, patients 1 and 2 share similar clinical features that differ substantially from patient 3. Our patients confirm the existence of two characteristic phenotypes in 1p36.22-->pter deletion. Both phenotypes share some dysmorphic features, but are differentiated by characteristics of growth failure versus macrosomia. In addition, we report the new finding of cardiomyopathy and hydrocephalus in the phenotype associated with growth failure. It is possible that different phenotypic subgroups may exist because of differences in the parental origins of the deleted chromosome or of variations in undetectable amounts of genetic material.     

The site of a missense mutation in the extracellular Ig or FN domains of L1CAM influences infant mortality and the severity of X linked hydrocephalus

The L1 cell adhesion molecule (L1CAM) plays an important role in axon growth, fasciculation, and neural migration. Mutations in the L1CAM gene produce a phenotype characterised by X linked hydrocephalus, mental retardation, spastic paraplegia, adducted thumbs, and agenesis of the corpus callosum. We have conducted a detailed analysis of the phenotypic effects of missense mutations in the extracellular portion of L1CAM, following a study that differentiated between "key" amino acid residues critical for maintaining the conformation of the extracellular immunoglobulin type C-like (Ig) or fibronectin type III-like (FN) domains and surface residues of less certain significance. We have analysed the data from 71 published cases and seven patients whose mutations were detected in our laboratory to determine if the site of a missense mutation in the Ig or FN domains correlated with the severity of hydrocephalus, presence of adducted thumbs, or survival past infancy. Mutations affecting the key residues in either type of domain were more likely to produce a phenotype with severe hydrocephalus, adducted thumbs, and lifespan less than one year than were mutations affecting surface residues. In addition, mutations affecting the FN domains were more likely than those affecting Ig domains to produce a phenotype with severe hydrocephalus, with less certain effects on adducted thumbs and lifespan. Mutations in key residues of the FN domains were particularly deleterious to infant survival. These data provide information that may be useful in predicting some aspects of the phenotypic effects of certain L1CAM mutations.     

The role of endoscopic third ventriculostomy in the management of shunt malfunction

OBJECTIVE: To evaluate the effectiveness of third ventriculostomy as an alternative to shunt revision in the management of shunt malfunction and infection in obstructive hydrocephalus. METHODS: All of the clinical notes of 30 patients treated with third ventriculostomy for malfunctioning or infected shunts between January 1, 1974, and December 31, 1996, were retrospectively reviewed. Third ventriculostomy was performed under fluoroscopic control in the first seven patients and endoscopically in the remainder. A successful outcome was achieved if further shunt revision surgery was avoided. The median follow-up duration was 8.7 years RESULTS: Twenty-three patients (76.7%) experienced successful outcomes, resulting in shunt independence. Of the seven failures, three were technical failures at the time of surgery and the remaining four were manifest within a median of 10 days, resulting in shunt revision. There have been no delayed failures. CONCLUSION: Third ventriculostomy is a valuable alternative to shunt revision in patients affected by obstructive hydrocephalus presenting with shunt malfunction or infection. It should be considered in all suitable cases as the first-line treatment for obstructive hydrocephalus of all causes. Because all failures were manifest within a short time, it is likely that these successes will be durable.