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A case of massive cerebral aspergillosis complicating a frontal encephalomeningocele is reported. The etiology and pathogenesis are discussed and the diagnosis, symptoms, and treatment possibilities reviewed.
The Currarino triad is a unique complex of congenital caudal anomalies including anorectal malformation, sacral bony abnormality, and presacral mass. The usual symptomatology is constipation due to anorectal stenosis. Contrast enema and computed tomographic myelography are the imaging modalities of choice for diagnostic confirmation and clarification of the anomalies. The clinical features, unique radiologic appearance, and importance of a correct diagnosis of the Currarino triad are reviewed.
The treatment of a boy is reported, who suffered from a combination of vesico-intestinal fissure and meningocystocele. Because five of seven patients known to us since 1969 had a similar combination, it should be analysed whether there exists an etiological or embryological connection. Therefore the pertinent literature and clinical data of known patients was collected and evaluated. It can be shown that also in the literature a coincidence of 75% of vesico-intestinal fissure and meningomyelocele is reported and both are caused during the 4th week of embryological development. Vesico-intestinal fissure is a disturbance of mesodermal inflow into the lower abdominal wall. The cause for meningocystocele is an abnormal cell proliferation of the neural anlage. Despite a number of possible clues an etiological connection could not be proved.
Currarino's triad is a rare complex of a congenital sacral bony abnormality, anorectal malformation and a presacral mass. Intractable constipation since birth is the leading symptom of this triad, which follows an autosomal dominant mode of heredity. We report conventional radiographic and MR findings in one family consisting of a mother and her two daughters. In all three cases, radiography revealed an abnormality of the os sacrum, the so-called scimitar sacrum. MR examination, undertaken next in our institution, was applied with T1-, T2- and proton density weighted sequences in all three orientations before and after i. v. application of gadolinium diethylene-triamine-pentaacetic acid (Gd-DTPA). In two patients we were able to diagnose the complete form of the triad and in one patient an incomplete form. In any case of a radiographically diagnosed scimitar sacrum in combination with constipation, Currarino's triad should be considered. MRI, as the method of choice, should be the next step to detect a presacral mass and any anomalies of the spinal canal. The importance of early recognition lies in the high morbidity and mortality rates resulting from this disorder.
Ten cases of the empty sella syndrome are presented. In four a pituitary adenoma had been treated by surgery with or without radiotherapy. In three women there was a close relation pregnancy, and two boys had congenital malformations. Five cases presented with appearances of pseudotomour, and the remainder had cerebrospinal fluid (CSF) fistula. Four fistulas were spontaneous, one was post-traumatic aggravated by the intrasellar implantation of YT-90. Two were occult CSF fistulas causing recurrent meningitis. Gamma cisternography and iodocisternography proved to be good diagnostic tests, both for the empty sella and for CSF fistulas. Nine cases were operated on. In four na intrasellar cyst was present. In the other five an empty sella with deficient or absent diaphragm was found. Treatment of most cases consisted of covering the floor of the sella with lyophilized dura, which was fixed in place with biological glue.
A case of an intrasacral meningocoele is described with typical radiological findings and obvious improvement after surgical treatment, but a somewhat unusual neurological picture.
Ten patients with postoperative collection of subcutaneous cerebrospinal fluid (pseudomeningocoele) refractory to nonsurgical treatment underwent lumboperitoneal (LP) shunt. In all patients rapid resolution of the subcutaneous collection was achieved, and no recurrence was observed during the follow-up period. Complications of the LP shunt included transient sciatica in one patient, and acute subdural haematoma after a mild head trauma in another patient who had had a subdural fluid collection. A LP shunt proved to be a less invasive and reliable method of treatment for this condition when it fails to resolve despite aspiration of cerebrospinal fluid combined with mechanical compression of the wound. However, in cases with subdural fluid collection, early withdrawal of the LP shunt may be recommended.
Ventricular shunting has dramatically improved the care of children with hydrocephalus. Yet shunt malfunctions are extremely common and cause significant morbidity. To document shunt problems in children with a neural tube defect and hydrocephalus, 67 children born since 1973 were studied via life-table analysis. 28% of the shunts failed within the first 6 months after insertion, 37% failed within the first year and 50% failed by 4 1/2 years after insertion. Shunt survival was similar in children whether or not they had previous shunt failures. The brand of shunt system and pressure rating, the level of neurological function, the interval between closure of the neural tube lesion and shunt insertion, and head circumference percentile at the time of insertion were also not correlated with shunt failure. However, shunts inserted in the first year of life were much more likely to fail than those inserted after one year of age (p less than 0.05). 68% of the revisions required replacement of the ventricular catheter. Shunt failure from all causes of hydrocephalus accounted for approximately 1% of paediatric admissions to Strong Memorial Hospital in 1982 with a mean cost of $4,543 and a mean length of stay of 9 days. Thus shunt problems remain both common and serious.
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Medical history and symptoms of a 37 year old woman showing progressive conus syndrome for nine months after a symptomless interval of 20 years are reported. NMR, myelography, and computed tomographic myelography verify a filum terminale with a paramedian fixation at the level of the lumbosacral angel and a further fixation at the caudal end of the dural sac at the lower end of the sacrum. The operative findings and procedures are described. The importance of control by neuro-stimulation of the filum terminale during surgery is stressed. The post-operative remission of most of the symptoms proves the success of the treatment.
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To date, four cases of ossified extradural pseudocyst have been described, the first being in 1951 by Verbiest who described unusual forms of compression of the cauda equina including a case of a lumbo-sacral extradural cyst and a case of 'knotting' of a caudal nerve root. We present an additional two cases.
Transtentorial herniation (TTH) of the fourth ventricle is the result of a progressive enlargement of an isolated fourth ventricle which herniates through the tentorial incisure into the middle cranial fossa. The characteristic CT-MR and neurosonographic findings are described.
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We present the clinical and neuroradiological features of tethered cord syndrome in a young woman and discuss the diagnostic role of CT scanning, performed before and after myelography.
We describe a newborn with multiple congenital anomalies due to the Amniotic Band Disruption Complex. Cerebral computerized tomography and plain films of the extremities corroborated the clinical diagnosis. The purpose of this case report is to alert the radiologist to the varied radiographic appearance of this little known entity and to provide a historical and embryologic background.
The most important spinal disorders in childhood are malformations and mass lesions. The sonographic appearance of the various kinds of dysraphism and their differentiation, of malformations at the cranio-cervical junction, and of mass lesions is demonstrated and illustrated. Based on our preliminary experience, spinal sonography appears to be useful as the first imaging device for the differentiation of complex malformations and as a screening method for occult dysraphism. In mass lesions spinal sonography is useful to diagnose and follow-up disease, with additional imaging procedures currently needed for confirmation of diagnosis.
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