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PubMedDate not supplied

[Prevalence and geographic distribution of spina bifida aperta in West Germany].

In a national study the prevalence of open meningocele and myelomeningocele as well as the geographical distribution in the Federal Republic of Germany was determined among the life-born infants during 1982 and 1983. The prevalence rates of the single districts and states and the federal territory were established based upon standardized reports of nearly all Children's Hospitals, Departments of Child Surgery and the major Centers of Neurosurgery. In Hamburg the study was extended by establishing the number of abortions because of medical indications after the prenatal diagnosis of a myelomeningocele. In 1982 and 1983 there were 354 resp. 342 infants born with an open myelomeningocele, thus the prevalence rate was 0.569 and 0.575/1000 live births. The average prevalence rate was 0.57/1000 live births. Compared with international studies our data are corresponding with reports from countries with a relative low frequency. The prevalence rates of the states in the federal territory differ between 0.42/1000 live births in Hamburg and 0.78/1000 in Bremen; these differences are not statistically significant. In 9 from 247 districts, the reported rates were significantly higher than the average rate. Because of the small number of children born with this malformation in the single districts, any conclusions from these results are not warranted. Quite different from reports of other countries (Great Britain, United States of America) this study did not show essential geographic differences in the federal territory. Prenatal diagnosis and subsequent termination of pregnancy lowered the prevalence rate of open meningomyelocele at birth in Hamburg from 0.61/1000 to 0.42/1000.

Abortion, Eugenic↗
PubMedDate not supplied

[Clinical and radiologic comparison of "idiopathic" and "neurogenic" pes cavus].

41 patients with 66 cavus feet were reviewed clinically and radiologically according to the parameters of Dennemann at an average age of 41.3 years. The etiology was a so-called "idiopathic pes cavus" in 51 per cent of the cases, in the others, a neurological basic disease (15 per cent condition after poliomyelitis, 3 per cent meningocele, 6 per cent Friedreich's ataxia, 10.5 per cent other neurological disorders) or condition after congenital club foot or trauma. Exact neurological and electrodiagnostic examination of the cavus feet primarily staged as idiopathic revealed sensory or motor loss in 74 per cent of the cases. A comparison of the "idiopathic cavus feet" with the cavus feet in neurological basic diseases with respect to the radiological parameters showed no significant differences concerning progression and osteoarthritis. Conservative and surgical measures were carried out with roughly the same success. In 36 cases (55 per cent), however, complaints on the part of the ankle joint and the talo-calcaneonavicular joint and the metatarsal head were noted.

Adolescent↗
PubMed1990

Spina bifida: a chick embryo experimental model.

Neural Tube Defects (NTD) can be induced in the chick embryo with relative ease in order to provide an experimental tool for investigation of such disabling malformations. Domestic hen (Gallus gallus) eggs were incubated at 37.5 degrees C and 80% humidity for 24 h. At that moment, 5 ml of albumen were aspirated by sterile puncture of the shell, and the incubation was resumed. The embryos were recovered and studied at the 8th, 10th and 14th days. Almost half (45%) of the 602 treated embryos survived and 73 of them (12%) had various malformations. Thirty-six (6%) suffered NTD of which 30 were open myelomeningocele, 2 meningocele and 4 encephalocele. The anatomy of the defects was astonishingly similar to that of the human malformation. Whether these experimental NTD are induced by mechanical or nutritional modifications of the internal environment of the egg is unknown, but the similarity of the lesion with those in humans make them suitable for further investigation of these issues. We believe that this relatively simple and inexpensive model is a suitable tool for research on spina bifida.

Animals↗
PubMed1989

[The clinical problem of the tethered cord syndrome--a report of 3 personal cases].

Tethered cord syndrome is defined as a low state of the conus medullaris below the lumbar vertebra 2 after the neonatal period. Possible causes are: short thickened filum terminale, fibrotic ligaments, intradural lipomas. The clinical course is characterised by motoric and sensory deficits, disturbances of balance, neurogenic bladder disturbances, foot deformities and backache. A 3-year-old boy after operation of a lipomyelomeningocele at 2 months showed a one-sided leg shortening and a progressive neurogenic bladder and intestinal disturbance. The cause was a low state of the conus with a shortened filum terminale and a remainder of a lipoma. A 7-year-old boy after operation of a thoraco-lumbar meningomyelocele developed a progressive asymmetric paraspastic state with contractures. The neuro-radiological diagnosis of an intradural dermoid was verified on operation. A 10-year-old boy after operation of a covered lumbosacral meningocele showed a progressive backache which was connected with flexion. We found a low state of the conus through adhesions caused by scars which could be removed operatively. A collaborative treatment by the paediatric surgeon and the neuro-paediatrician of patients with dysraphic disturbances can prevent the severe consequences of the tethered cord problems with the co-operation and early intervention of the neuropaediatric surgeon.

Cauda Equina↗
PubMed1984

The tethered spinal cord syndrome.

The results of surgical treatment of the tethered spinal cord syndrome in twenty-five children in the age range six weeks to nineteen years are reported. After a description of the symptomatology, the importance of neuroradiologic investigations including myelography and CT-scanning as well as urodynamic studies are explained. The operative techniques are described in cases of tight filum terminale, lipoma of the filum terminale, diastematomyelia, lipomyelomeningocele and meningocele. Early operation is essential to avoid the neurological damage which results from awaiting the onset of symptoms. The aim of the operation is to free the conus medullaris and cauda equina before mechanical traction or compression damages function in lumbosacral cord.

Adolescent↗
PubMed1987

Neurenteric cyst of medulla oblongata--a curiosity.

A case of a newborn infant with a complex cerebral malformation, including a neurenteric cyst of medulla oblongata is presented. This localization is extremely rare, only one such case has been reported in the world literature. Other cerebral malformations were spina bifida posterior with cervico-dorsal dysraphia, cerebellar hypoplasy, cervico-dorsal cystic meningocele and hydrocephalus. The etiology of this complex malformation remained unknown.

Female↗
PubMed1996

The benefits of team treatment and control of adult patients with spinal dysraphism.

We investigated the outcome of newly referred patients to our spina bifida team in the period 1989-1994. 12 of 42 patients had spina bifida occulta, 8 had a meningocele and 22 a myelomeningocele. More than half of the patients underwent an operation which was beneficial to them. 13 operations for a tethered cord syndrome were performed with good results. In 15 patients referral delay caused unnecessary and mostly irreversible harm. Life-long regular control by members of a spina bifida team is, therefore, highly recommended.

Adult↗
PubMed2000

Type 1-primary cutaneous meningioma of the scalp.

Type 1-primary cutaneous meningioma is a rare and often clinically unsuspected lesion of the scalp, forehead or paravertebral region which occurs at birth or during childhood. The pathogenesis of these lesions still remains uncertain. Several authors have emphasized that type 1-cutaneous meningiomas are not real tumors but sequestrated meningoceles or heterotopic meningeal nodules of the skin. Nevertheless, the search for an intracranial or intravertebral connection should be carried out. We describe the clinical and pathological features of a congenital type 1-meningioma of the vertex. No cranial defect or intracranial tumor was found. However, the cutaneous lesion was directly linked to a large cranial vein. The purpose of this study was to illustrate this rare lesion and to indicate the possible surgical risks and the pathological characteristics.

Child↗
PubMed1994

Outcome after maternal varicella infection in the first 20 weeks of pregnancy.

BACKGROUND: Infection with the varicella-zoster virus during pregnancy can produce an embryopathy characterized by limb hypoplasia, eye and brain damage, and skin lesions. The risk is greatest when infection occurs during the first 20 weeks of pregnancy, but the magnitude of the risk is uncertain. METHODS: We studied 106 women with clinically diagnosed varicella infection in the first 20 weeks of pregnancy and compared the outcomes with those in 106 age-matched, nonexposed controls. RESULTS: Among the women with varicella, there was a trend toward more elective terminations of pregnancy (14 percent, vs. 7.5 percent among the controls; P = 0.1), corresponding to a significantly higher perception of teratogenic risk (P = 0.03). The proportions of miscarriages and live births and the mean birth weights were similar in the two study groups; there were more premature births (< or = 37 weeks) among the women with varicella infection (14.3 percent vs. 5.6 percent, P = 0.05). Congenital defects occurred in four infants born to the women with varicella (varicella embryopathy, hydrocephalus, meningocele and clubfeet, and hammer toe) and two infants born to the controls (ventricular septal defect and hip dislocation). The risk of varicella embryopathy after infection in the first 20 weeks was 1.2 percent (95 percent confidence interval, 0 to 2.4 percent). When we pooled our results with those from other prospective studies, the mean risk of embryopathy after infection with varicella-zoster virus in the first trimester was 2.2 percent (95 percent confidence interval, 0 to 4.6 percent). CONCLUSIONS: The absolute risk of embryopathy after maternal varicella infection in the first 20 weeks of pregnancy is about 2 percent.

Chickenpox↗
PubMed2003

Bullous aplasia cutis congenita.

A tense yellow vesicle was noted on the back parietal scalp of a female newborn. This proved to be a bullous variant of aplasia cutis congenita. Only 16 cases of this apparently rare disorder have been previously reported. Histologic evaluation of such lesions reveals a distinct pattern containing fibrovascular stromas, edematous stroma, or both. Identical histologic findings are found in encephaloceles and meningoceles, supporting the recently proposed hypothesis that this variant of aplasia cutis may represent the form fruste of a neural tube closure defect. This disorder should be included in the differential diagnosis of vesicobullous lesions in the neonate. Bullous aplasia cutis congenita is a rare clinical subtype of aplasia cutis congenita with distinctive histologic findings. We present a new case, and summarize the clinical and histologic findings of the 16 cases previously reported in the English-language literature. Bullous or membranous aplasia cutis congenita may represent a form fruste of a neural tube defect.

Ectodermal Dysplasia↗
PubMed2001

Congenital malformations in newborns in a teaching hospital in eastern Saudi Arabia.

A review of 14762 singleton live-born babies during a period of 6 years at the King Fahad Hospital, Al-Khobar, Saudi Arabia is made with a view to study the clinical pattern of easily identifiable congenital malformations among neonates. The incidence of congenital malformations in the study was 17.0/1000 live births. Major abnormalities were present in 74.4% and minor ones in 25.6% of cases. The anatomical organs most commonly affected were the central nervous system (CNS), musculoskeletal and renal defects followed by gastrointestinal and chromosomal defects. CNS anomalies accounted for 48.8% of the total defects, hydrocephaly, anencephaly and meningocele being the predominant lesions. The incidence of malformed babies in diabetic mothers was 7.8%. Multiple anomalies were present in 16.7% infants. Of the total 251 malformed infants, 38 died within the first week of life with a mortality rate of 15.1%, compared with the overall perinatal mortality rate of 12.2/1000 live births in the hospital during the period of study.

Journal Article↗
PubMed1989

Congenital lumbosacral lesions with late onset in adult life.

Seven adult patients with congenital lumbosacral lesions and clinical onset in adult life are reported. All also had an abnormally low and tethered spinal cord. A thickened filum terminale and a spinal lipoma are the most common lesions in adults, whereas others, including diastematomyelia, fibrous adhesions, previous meningocele repair and dermal sinus are more rare. Although water-soluble myelography visualizes the abnormally low conus well and may diagnose a diastematomyelia and a thickened filum, computerized tomography, CT myelography and magnetic resonance may provide a better diagnostic definition of the other abnormalities. Surgical treatment should be performed in all adult patients with progressive symptoms. Transection of the thickened filum or of fibrous adhesions and the removal of a spinal lipoma, diastematomyelia septum or dermal sinus tract, result in remission of the pain and improvement of the sensorimotor deficits, or at least prevent the progression of the neurological troubles.

Adult↗
PubMed1976

Cerebrospinal fluid pressure changes in response to coughing.

CSF pressure recordings have been taken from the lumbar region and the cisterna magna of 16 patients during coughing in the sitting position. Isolated coughs of low amplitude have been studied. The lumbar pressure waves occur sooner and lower. Thus there is a phase during which the lumbar pressure exceeds the cisternal, followed by one in which the cisternal exceeds the lumbar. These phenomena may be conveniently displayed on a differential trace. The phase during which the cisternal pressure exceeds the lumbar may be protracted. It is suggested that Froin's syndrome, central subarachnoid pouches and syringobulbia may be associated with upward pressure waves. Cough headache, the filling stages of communicating syringomyelia and tonsillar herniation may be associated with valve-like blockage at the foramen magnum which produces craniospinal pressure dissociation by interfering with downward or rebound pulsation. Decompensation of hydrocephalus after birth may be related to pulsation in association with crying; also after removal of a meningocele sac decompensation may be related to the effects of similar pulsation modified by changes in capacitance following operation. The cord destruction of syringomyelia, and the mechanisms which fill spinal subarachnoid cysts may be related to pressure waves directed both upwards and downwards. The merits and limitations of cough impulse as a clinical test for spinal blockage are discussed, and the suggestion is made that after further evaluation they may provide a more sensitive indication of spinal blockage than Queckenstedt's test.

Cisterna Magna↗
PubMed1992

Neural tube defects in the middle belt of Nigeria.

Forty-two babies were found to have neural tube defects during a 3-year prospective study in a large cosmopolitan West African city. An incidence of 7/1000 deliveries was noted. The commonest defect was meningomyelocele, in 45 per cent of cases. Other defects comprised anencephaly (5 per cent), Arnold-Chiari malformation (7 per cent), encephalocele (19 per cent), occipital meningocele (14 per cent) and spina bifida occulta (10 per cent). No case of consanguinity was encountered. More defects were found among females and low birth weight infants among those whose mothers were aged between 20 and 30 years, and in mothers with parity of four and above. The commonest associated defect was talipes equinus in 15/42 (36 per cent) infants. Developmental assessments (neurological follow-up status) done at the age of 18 months on five surviving cases of meningomyelocele showed very poor development.

Female↗
PubMed1992

Continuous intrathecal baclofen in spinal cord spasticity. A prospective study.

Continuous intrathecal infusion of the well known antispastic medication baclofen was evaluated in ten consecutive patients. One year after pump implantation the average Ashworth scale for muscle tone decreased, compared with before treatment, 2.32 points (P < 0.0001), reflexes decreased 2.22 points (P < 0.0001) and the spasm score decreased 1.65 points (P < 0.0001). The average dose increased from 92.22 to 290.95 micrograms (P < 0.0001) between the 1st month of treatment and 1 yr of treatment. The dosage for all patients more than doubled (P < 0.0022) between 3 months and 1 yr postimplantation. There was no significant difference for muscle tone, reflexes or spasms at 3 months v 1 yr. Complications were not unusual and included temporary atelectasis, orthostatic hypotension with escalation of baclofen dose, loss of penile erections, postsurgical pseudo-meningoceles, catheter disruptions and exhausted pump reservoirs. One patient suffered a seizure apparently related to a rapid withdrawal from intrathecal baclofen as a result of catheter sequestration. All patients required a period of intensive inpatient rehabilitation to benefit functionally from the decreased motor tone and/or increased voluntary motor control. The procedure is expensive and close follow-up is necessary for assessing efficacy and refilling the pump. Intrathecal baclofen infusion by subcutaneous pump is useful in treating the effects of spinal spasticity resistant to oral medications. However, there appears to be accommodation to intrathecal baclofen necessitating escalating doses to maintain clinical effects.

Adult↗
PubMed2001

Magnetic resonance imaging of spinal dysraphism.

Spinal cord development occurs through three consecutive periods. Gastrulation (weeks 2-3) is characterized by conversion of the embryonic disk from a bilaminar to a trilaminar arrangement and establishment of a notochord. Primary neurulation (weeks 3-4) produces the uppermost nine tenths of the spinal cord. Secondary neurulation and retrogressive differentiation (weeks 5-6) result in formation of the conus tip and filum terminale. Defects in these early embryonic stages produce spinal dysraphisms, which are characterized by anomalous differentiation and fusion of dorsal midline structures. Spinal dysraphisms may be categorized clinically into two subsets. In open spinal dysraphisms, the placode (non-neurulated neural tissue) is exposed to the environment. These disorders include myelomeningocele, myeloschisis, hemimyelomeningocele, and hemimyelocele, and are always associated with a Chiari II malformation. Closed spinal dysraphisms are covered by intact skin, although cutaneous stigmata usually indicate their presence. Two subsets may be identified based on whether a subcutaneous mass is present in the low back. Closed spinal dysraphisms with mass comprise lipomyeloschisis, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise complex dysraphic states (ranging from complete dorsal enteric fistula to neurenteric cysts, split cord malformations, dermal sinuses, caudal regression, and spinal segmental dysgenesis), bony spina bifida, tight filum terminale, filar and intradural lipomas, and persistent terminal ventricle. Magnetic resonance imaging is the imaging method of choice for investigation of this complex group of disorders.

Adolescent↗
PubMed1998

Spinal-cerebrospinal fluid leak demonstrated by radiopharmaceutical cisternography.

Cerebrospinal fluid (CSF) leaks are known to occur under several conditions: lumbar puncture performed for contrast myelography, spinal surgery, spinal stab wounds, fracture of the thoracic spine, inadvertent spinal puncture during epidural anesthesia, traumatic lumbar meningocele, and bronchopleural subarachnoid fistula due to bronchogenic carcinoma. Spontaneous spinal leaks are uncommonly encountered in neurosurgical practice, but they are increasingly recognized as a cause of spontaneous intracranial hypotension. Most CSF leaks are located at the cervicothoracic junction or in the thoracic spine. The disease is often self-limiting. A CSF leak can be detected directly by accumulation of radioactivity outside the subarachnoid space or suggested indirectly by the rapid disappearance of tracer from the subarachnoid space and early appearance in the urinary bladder. In this paper we present two unusual cases of CSF leak identified by radiopharmaceutical cisternogram.

Adult↗