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Biomedical subjects

M Poza

Publications and source records attributed to M Poza.

At least 55 records · Page 3Linked to original sources
PubMed1996

Congenital desmoid tumor of the scalp: a histologically benign lesion with aggressive clinical behavior.

Desmoid tumors are uncommon benign neoplasms of fibroblastic origin, frequently presenting in the abdomen or abdominal wall of adults. This tumor is well known to general surgeons and pathologists, but it has rarely been reported in the neurosurgical literature. We describe a neonate who presented with a right temporal subscalp tumor at birth. In spite of initial apparent total removal, the tumor recurred twice. We discuss the diagnostic pitfalls encountered in assessing this tumor and the difficulties in distinguishing, in the excised specimens, between desmoid and reactive fibrosis. We suggest that desmoid tumor should be considered in the differential diagnosis of scalp and calvarial masses in children. Complete excision is required to prevent recurrences, and sometimes a multidisciplinary team is needed to secure margin-free resection.

Adult↗
PubMed1996

Craniosynostosis in neural tube defects: a theory on its pathogenesis.

BACKGROUND: In most cases, craniosynostosis is a sporadic occurrence of unknown etiology. Efforts to ascertain the exact, cause of this deformation often fall to reveal its origin and pathogenetic mechanism. Some previous reports have described isolated instances of the association of neural tube defects (NTD) and craniosynostosis, but in none of them has a causal relationship been established to explain this coincidence, apart from a fortuitous sequence of congenital defects. METHODS: We have reviewed the clinical records of 40 patients diagnosed with craniosynostosis who were studied at our hospital over a 22-year period, for the purpose of determining the possible etiologies of this cranial deformation. RESULTS: We found that three children with unilateral coronal synostosis had associated NTD-an incidence of 7.5%. Two children had cephaloceles and the third harbored a lumbosacral myelomeningocele. In these three cases, the craniosynostosis was noted immediately after birth and before the development or treatment of hydrocephalus. CONCLUSION: We think that there must be an alternative explanation for the association of craniosynostosis and NTD other than overdrained hydrocephalus or chance association. We advance the hypothesis that in these cases the cause of the premature coronal synostosis was the lack of cerebral expansion. Since brain expansion and intracranial pulse pressure determine the growth and shape of the overlying cranial vault, the dampening effect of the malformation sac (myelomeningocele or cephalocele) on the intracranial pressure and on the pulse pressure may lead to close contact of the affected bones and to the premature closure of the coronal suture. The possibility of fetal head constraint because of diminished fetal activity due to the NTD as a contributory factor is also suggested.

Craniosynostoses↗
PubMed1996

Familial occurrence of atretic cephaloceles.

In this article we report the cases of an 11-year-old girl who presented with an occipital atretic cephalocele and 2 of her siblings who had similar occipital lesions. Neuroimaging studies in these three instances showed a spectrum of posterior fossa cystic malformations. The girl's parents and a further sibling were also investigated by neuroimaging studies that proved to be normal. The familial occurrence of cephaloceles in general, and of atretic cephaloceles in particular, seems to be very rare in the absence of a known syndrome. Current literature on the familial presentation of cephaloceles and of Dandy-Walker complex is briefly reviewed. To the best of our knowledge this is the first description of the familial presentation of atretic cephaloceles not associated with a recognized syndrome.

Adolescent↗
PubMed1995

Congenital dermal sinus associated with an abscessed intramedullary epidermoid cyst in a child: case report and review of the literature.

A 16-month-old male infant presented with paraparesis and a high thoracic skin dimple. After myelography the child underwent complete surgical removal of the dermal sinus and of an abscessed tumor located within the cervicothoracic cord. Histological study showed that the lesion was an intramedullary epidermoid cyst. Only four descriptions have previously been published of dermal sinuses associated with intramedullary epidermoids, three of them complicated by intramedullary abscesses. This appears to be the first report in the current literature of the association of a dermal sinus and an infected intramedullary epidermoid occurring out of the confines of the lumbosacral region.

Epidermal Cyst↗
PubMed1995

Neurosurgical management of Walker-Warburg syndrome.

The Walker-Warburg syndrome (WWS) is a lethal complex of the central nervous system and the eyes. At present its cause is unknown, but clinical evidence strongly suggests that it is an autosomal-recessively inherited disorder. We report a series of nine children with WWS. The diagnosis was established by the detection of lissencephaly, hydrocephalus, and cerebellar malformation on computed tomography. All children exhibited profound psychomotor retardation and ocular abnormalities (in their anterior or posterior eye chambers). The existence of an occipital encephalocele in eight cases was the main diagnostic clue to WWS. Six patients were investigated for the presence for congenital muscular dystrophy, which was confirmed in only four of them. There were no patients with a cleft lip or palate. We studied the incidence of WWS in Spain and estimated it at 0.21 cases per 10,000 live-born children. In our series, WWS was prevalent in the Spanish gypsy population. Consanguinity was present in five of seven affected families. In a case of pregnancy with twins, one of the siblings was unaffected. Eight patients were treated with ventriculoperitoneal shunts and seven underwent encephalocele repair. Histological study of the excised encephaloceles demonstrated two different patterns. Interestingly, one of the infants showed coronal craniosynostosis. Finally, we include in the appendix, for completeness, a report of the case of the sibling of a WWS patient with acrania-exencephaly.

Brain Diseases↗
PubMed1994

Macrocephaly, dystonia, and bilateral temporal arachnoid cysts: glutaric aciduria type 1.

Two siblings presented with macrocephaly, psychomotor delay, and progressive dystonia. The initial diagnosis was of hydrocephalus and bilateral temporal cerebrospinal fluid collections. Following ventriculoperitoneal shunting, the patients showed only modest neurological improvement. Metabolic investigations performed later in the course of the disease disclosed increased levels of glutaric acid in the urine and decreased levels of serum carnitine, which were confirmatory of glutaric aciduria type 1. The association of macrocephaly, dystonia, and bilateral temporal arachnoid cysts, shown either by computed tomography or magnetic resonance imaging, seems to be diagnostic of glutaric aciduria type 1. The authors report these two cases as they think they might be of interest to neurosurgeons.

Arachnoid Cysts↗
PubMed1994

Accidental transmission of Creutzfeldt-Jakob disease by dural cadaveric grafts.

Four patients who received dural grafts of cadaveric origin in the course of posterior fossa procedures subsequently developed Creutzfeldt-Jakob disease (CJD). The interval from dural placement to clinical onset of CJD ranged from 16 months to nine years. Initial clinical presentation consisted of cerebellar symptoms, with dementia and myoclonus developing in later stages of the disease. EEGs showed diffuse slowing that evolved to a periodic activity pattern. CT and MRI were unremarkable in the early stages but pronounced cerebral and cerebellar atrophy with widened sulci and collections of fluid over the convexities were seen in the late stages of disease. The diagnosis was histologically proved by brain biopsy in all four cases. Molecular genetic analysis showed that the four patients were homozygous for methionine at codon 129 of the PrP gene. From this experience, and from six previous descriptions of this occurrence in the literature, it is manifest that awareness of the means of iatrogenic transmission of CJD, and the adoption of preventive measures, constitute the only effective way to stop the spread of CJD among patients who have neurosurgery.

Adolescent↗
PubMed1993

Pediatric Creutzfeldt-Jakob disease: probable transmission by a dural graft.

A 10-year-old boy underwent a posterior fossa craniectomy for removal of a grade 2 cerebellar astrocytoma. Dural closure was achieved by the placement of a dural graft. Eight years later the patient developed dementia and myoclonus. Electroencephalography demonstrated generalized slow activity that evolved into a pattern of periodic triphasic waves. Computed tomography scan and magnetic resonance imaging were unremarkable. Brain biopsy confirmed spongiform encephalopathy of the Creutzfeldt-Jakob type. In the light of previous reports of four similar occurrences, and of our own experience with two further cases of this disease, we believe that the cadaveric dura was the source of transmission of Creutzfeld-Jakob disease in our patient. The authors remark the importance of the awareness of this late complication of dural substitutes, both for the diagnosis of possible future cases and for taking preventive measures to stop the spread of the disease.

Astrocytoma↗
PubMed1993

Giant meningioma of the III ventricle in a child: case report and review of the literature.

We report the case of an 11-year-old girl with a giant meningioma of the III ventricle who presented with signs and symptoms of raised intracranial pressure. The tumor was removed through a transcallosal approach. A review of current literature yielded 16 previous cases of meningiomas of the III ventricle in the pediatric age group. The usual form of presentation is that of a midline syndrome with no localizing signs. In the cases that were operated on, surgical intervention was rewarding and mortality was low. A very unusual finding in the meningioma that we report was its origin in the floor of the III ventricle, a circumstance that has only once been described previously.

Cerebral Ventricle Neoplasms↗
PubMed1993

Bilateral temporal arachnoid cysts in neurofibromatosis.

The occurrence of bilateral temporal arachnoid cysts has been considered as a rare event. Unilateral arachnoid pouches have been reported in a few instances associated with neurofibromatosis. The authors describe a 5-year-old girl with bilateral temporal arachnoid cysts who presented obvious stigmata of van Recklinghausen's disease. To the best of our knowledge, this is the first time that this association has been described in the literature.

Arachnoid Cysts↗
PubMed1992

Congenital arachnoid cyst of the lateral ventricles in children.

The authors report a series of three children with symptomatic congenital arachnoid cyst of the lateral ventricles. Presenting symptoms consisted of macrocephally, delay in psychomotor development, and seizures. CT findings were of a well-defined cystic lesion placed in the atrium of the lateral ventricle. One child was treated by direct cyst exposure and cysto-peritoneal shunt. The other two were treated with ventriculo-cysto-peritoneal shunts; in one of these, we used a ventriculoscope both for cyst fenestration and for accurate shunt placement. The origin of intraventricular arachnoid cysts seems to be secondary to the displacement of arachnoid cells by the vascular mesenchyma, through the choroid fissure, during the process of choroid plexus development.

Arachnoid Cysts↗
PubMed1992

The child with a mass on its head: diagnostic and surgical strategies.

A series of 65 pediatric patients with scalp or calvarial masses is reported on. The majority of children presented with a disfiguring or painful mass on the head. Clinical findings suggested the correct diagnosis in 39/65 cases, skull radiographs in 46/65, and CT in 49/65. Taking the combined results of clinical and radiological studies, 54/65 of the lesions were accurately diagnosed. Tumor excision was curative in 43 of 48 patients who were operated on. Most scalp and calvarial neoplasms were benign; only 5/65 children harboured a malignant lesion. There was no mortality related to surgery in the series. Surgical intervention seems to be indicated in most cases, both for diagnosis and for treatment.

Adolescent↗