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PubMed1995

[Malformation of the internal ear and recurrent meningitis: radio-surgical correlations].

Authors report one case of bilateral inner ear malformation in a 3 years-old child with congenital sensorineural hearing loss, recurrent meningitis and cerebro-spinal fluid leakage. This anomaly is a pseudo-Mondini's malformation with a meningocele through the oval window and a fistula of C.S.F. of the round window. A close radiological and surgical correlation support some physiopathological hypothesis about fistula between sub arachnoid space and middle ear. Authors discuss the place of high resolution computed tomography in case of congenital sensorineural learning loss.

Child, Preschool↗

PubMed1995

'Membranous aplasia cutis' with hair collars. Congenital absence of skin or neuroectodermal defect?

BACKGROUND: The skin and the nervous system are both derived from ectoderm. Separation of neural ectoderm from epithelial ectoderm occurs concurrently with the closure of the neural tube. This chronologic association may explain the cutaneous abnormalities often found overlying neural tube defects. A ring of dark long hair encircling a congenital scalp lesion (the hair collar sign) is one such marker and is often associated with encephaloceles, meningoceles, and heterotopic brain tissue. OBSERVATIONS: We describe six children with aplasia cutis who displayed the hair collar sign. Aplasia cutis is a relatively heterogeneous disorder; however, these lesions had a unique and strikingly similar appearance. This subtype of aplasia cutis, which we have termed membranous aplasia cutis, shares several clinical and histologic features with cranial neural tube defects. CONCLUSIONS: We propose that membranous aplasia cutis is a form fruste of a neural tube defect and may be derived from a similar embryological defect. Recent advances in the understanding of cranial neural tube closure may provide support for this hypothesis.

Atrophy↗

PubMed1994

The perioperative care of the paediatric neurosurgical patient.

The Queen's Medical Centre, University of Nottingham, is one of the largest neuro-science centres in Britain and serves a population of five million people. Its catchment area covers the counties of Nottinghamshire, Leicestershire, Derbyshire and Lincolnshire though patients are often referred from other parts of Britain and from abroad. The total number of neuro-paediatric beds is 20 plus access to 12 beds in the paediatric intensive care unit and 18 beds of the neonatal unit. In the year 1992 the total number of operations was 300, a number which increased by 25% the following year and included such operations as craniostenosis, craniotomies, spinal surgery, repair of meningocele, shunts, implanting and endoscopic and stereotactic procedures.

Child↗

PubMed1995

[Treatment of anal incompetence in children by free graft of denervated muscle].

Transplantation of a denervated muscle imitating a levator loop allows pathogenetic correction of anal incompetence. Its results depend on the patient's participation in the rehabilitation process to a lesser extent than the results of plastics of the external sphincter ani by the traditional methods. This is of certain significance in pediatric practice and makes it possible to operate on younger children. This method produces satisfactory results in the so-called hopeless patients suffering from meningocele and concomitant developmental anomalies of the spine. This makes it possible to widen significantly the indications for surgical treatment of children with fecal incontinence.

Adolescent↗

PubMed1976

[A case of paravertebral lumbar meningomyelocele (author's transl)].

A case reported here is one year-old girl, who was admitted to our neurosurgical service because of gradually increasing swelling in the left lumbar region, and maldevelopment and paresis of the left leg since birth. From the level of L-2 to S-1, there was a 8 X 7 xm subcutaneous swelling of lipomatous consistency with well-defined margins. Of particular characteristics was that the swelling didn't cross the midline and was entirely located in the left paravertebral region (Fig. 1). In the spine no gap could be felt between the spinous processes. There was no evidence of hydrocephalus, but she bad marked right sided scoliotic deformity of the lumbar region, and dislocation of hip joint and club-foot on the left side. Rectum-bladder-vaginal fistula with defect of anus and hypoplasia of kidney on the left side were also detected. Skiagram of the lumbosacral spine revealed marked right-sided scoliosis at the level of L-1 and hypoplasia of left pedicles, accompanied with marked dilation of transverse diameter of spinal canal between the level of L-1 to S-2. Defect of laminae of lumbosacral spine was not definately determined. Conray myelogram showed presence of cyst protruding laterally to the left paravertebral region probably through defect of lamina on the left side at the level of L-5 (Fig 2). At operation, it was confirmed that meningomyelocele protruded out laterally through the defect of half of lamina at the level of L-5. Massive lipoma was noted inside as well as outside the dura mater. Neural elements were replaced inside the spinal canal and the dura repaired. The postoperative period was uneventful. Diagram of defect of lamina at the level of L-5 and its relationships to the meningomyelocele sac is shown in Fig. 3. Meningocele or meningomyelocele which lays in the paravertebral lumbar region is very rare (Table 2), but the possibility of this disease should always be considered when we examine the patients with lipomatous or cystic swelling in the paravertebral lumbar region, especially when the patients present the neurological symptoms or signs.

Female↗

PubMed1994

[Clinico-epidemiologic study of infantile hydrocephalus in Japan].

A nation-wide survey on congenital hydrocephalus, including secondary hydrocephalus occurring within one year after birth, was carried out in 1988. The number of patients with congenital hydrocephalus treated during the previous one year was estimated to be 3,200-3,500 throughout Japan. The number of new cases of this disease which occurred during the year was calculated to be 800. This indicates a rate of 0.58 per 1,000 live births. Clinico-epidemiologic features were compared with regard to three subjects listed below. (1) The difference between the epidemiologic profile of term and preterm infants with hydrocephalus was investigated. As for etiology, the incidences of primary hydrocephalus and hydrocephalus complicated by Arnold-Chiari malformation type II and meningocele were significantly higher in term infants than in preterm infants. It was estimated that about 30% of the hydrocephalus in preterm infants and only 7.7% in term infants occurred as a consequence of intracranial hemorrhage. The degree of neurological impairment and the mortality rate were higher in preterm infants than term infants. (2) The changing panorama of infantile hydrocephalus in Japan was investigated comparing the epidemiologic data of four groups according to the birth year. As for pregnancy period, preterm infants with hydrocephalus were gradually increasing recently. Hydrocephalus secondary to intracranial hemorrhage was increasing and that secondary to intracranial infection was decreasing. The rate of shunt malfunction was decreasing significantly. (3) Clinico-epidemiologic features of intractable hydrocephalus based on the diagnostic criteria of the research committee was investigated comparing intractable with non-intractable groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Humans↗

PubMedDate not supplied

[Craniofacial dysraphism and dacryocystitis].

Two clinical observations with craniofacial dysraphism and affecting of the lacrimal ways are presented. The first observation was done on a 34-years-old patient, with medical craniofacial perforation (3-12), with telorbitism, right operated meningocele and left suprainfected lacrimal mucocele. The accentuated craniofacial dysraphism didn't permit dacryocystorhinostomy and imposed the extirpation of the left lacrimal mucocele. The second observation was done on a 6-years-old child with paramedian craniofacial perforation (3-11) and left suppurated dacryocystitis. The extirpation of the lacrimal bag was followed by purulent meningitis through continuity solution existent at the craniofacial level. It was insisted on effecting a through examination in craniofacial perforations for finding a craniocerebral continuity solution in the interventions done on lacrimal ways.

Adult↗

PubMed1993

Dural defects of the temporal bone.

Cerebrospinal fluid leakage (otorrhea or otorhinorrhea) from the temporal bone is the end result of rupture of the arachnoid membrane or herniation of the brain through a defect in the protective dura mater and calvarium. The rupture may be small, admitting only a herniation of arachnoid (meningocele), or be large enough to accommodate brain tissue (encephalocele). Flow of cerebrospinal fluid through either type of fistula may be a trickle or profuse, chronic or intermittent, and usually ceases temporarily for a few weeks following an attack of meningitis. The etiology, anatomy, signs and symptoms, and various methods of treatment for cerebrospinal fluid otorrhea and otorhinorrhea are discussed.

Brain Diseases↗

PubMed1993

The management of depressed skull fractures in children at Muhimbili Medical Centre, Dar es Salaam, Tanzania.

Thirty eight consecutive children with depressed fracture of the skull admitted to the Paediatric Surgical Unit (PSU) of Muhimbili Medical Centre (MMC), Dar es Salaam between January 1986 to May 1992 were studied. There were 22 boys and 16 girls. Age range was from birth to 10 years. A fall of one form or another accounted for 25 (65.00%) patients. Falling from a sister's or mother's back occurred in 4 (10.5%) patients, while falling from trees was a cause in 8 (21%) patients. Objects hitting on the head accounted for 6 (15.8%) patients. The objects included stones in 3 and falling coconut fruits in three. Traffic motor accidents accounted for 5 (13.2%) patients. In 35 (92.1%) patients, the fractures were closed while in 3 (7.9%) they were open. The fractures were located in the frontal or parietal bone in 27 (76.3%) patients. The 3 coconut fractures were located at the sagittal area. Only 4 (10.5%) patients had focal neurological signs. Convulsions were recorded in 3 patients while loss of consciousness was observed in 4 patients. Surgery was performed on 22 (57.9%) patients while conservative approach was practised in 16 (42.1%). The indications for surgery were cosmetics in 15 patients, compound fracture in 3 patients, focal neurological deficit in 2 patients, and torn dura presenting with Pseudo-meningocele in 2 patients.

Academic Medical Centers↗

PubMed1996

Immediate skin expansion: an old concept by a novel and inexpensive technique.

Intraoperative skin expansion is not a new concept. We have developed a technique using readily available inexpensive material to achieve wound closure. In this prospective study, 15 patients (age range, 1-72 years) with melanoma, giant nevi, scars, meningocele, gastroschisis, alopecia, thrombosed renal dialysis fistula, calcified nodule and trauma, with defects on back, limbs, abdomen, scalp, hand, calcaneum, and sole of foot were treated. Only hypodermic needles and dental wire, with an overall cost of less than a dollar, are needed for the immediate skin expansion technique. In contrast, the numerous other commercial devices currently in use are costly and must be ordered in advance. Using our technique, the wound can be gradually approximated and subcutaneous sutures placed with the device in situ. An intradermal or simple running suture is then placed to approximate the skin edges under little or no tension and the device is removed. All patients had a good result with minimal morbidity. The wounds treated by this technique healed by primary intention except in 3 patients, who developed minor dehiscence of the wounds.

Adolescent↗

PubMed1995

[Analysis of 110 cases of sacrococcygeal lesions in children].

110 cases of sacrococcygeal lesions seen in the Guangzhou Children's hospital were reviewed. 42 cases were anomalies, 66 cases were tumors and 2 were inflammatory granulomas. As compared to data in western literatures, the morbidity of anomalies in this series was lower than that of tumors, whereas in western reports the morbidity of anomalies was much higher than that of tumors. Another interesting point was that the morbidity of meningocele (MC) in this series was much higher than that of myelomeningocele (MMC) of spina bifida (7:1). According to a study in Australia, the MC:MMC was 1:5. We suggest that endodermal sinus tumor should be considered as an independent entity of there was no other tissue component (organlike component) found in tumor section. We also suggest that if neuroblastoma and/or other immature neuroectodermal component is observed in a teratoma, it should be classified as malignant rather than benign.

Child, Preschool↗

PubMed1996

The prevalence of genetic disorders, birth defects and syndromes in central and eastern Kentucky.

Without an operative Birth Defects Registry, the state of Kentucky does not have a means of determining which of the nearly 6,000 syndromes and birth defects are the most common or the most rare, nor is there an ability to compare and contrast these data with data from other states. The authors reviewed 4,212 charts of patients evaluated between July 1981 and February 1995 by the Division of Genetics and Dysmorphology at the University of Kentucky Chandler Medical Center. Each patient's chart was categorized by diagnosis, and tables were generated to determine the most common diagnoses in the following groups: (1) multiple congenital anomaly syndromes, (2) teratogenic embryopathies, (3) chromosome anomalies, (4) isolated malformations, and (5) bone dysplasias. The most common multiple congenital anomaly syndromes were Down syndrome, Marfan syndrome, and trisomy 18. Fetal alcohol syndrome and infants of diabetic mothers were the most common embryopathies. Spina bifida (meningocele and myelomeningocele) was by far the most common isolated birth defect, followed by cleft lip/ palate and microcephaly. Achondroplasia was the most common bone dysplasia. These data support a number of previous assumptions including the universally high frequency of syndromes like Down syndrome and trisomy 18. The data also give credence to what was previously thought, but unproven, to be a high incidence of diabetic and alcohol embryopathies. The latter (fetal alcohol syndrome) has increased in frequency tremendously over the past 7 years. This is undoubtedly due in part to the overall increased awareness of the diagnosis in the medical community. However, it may also be due to the increased use of alcohol among Kentucky women. Other "rare" disorders, like diastrophic dysplasia, seem to be unusually the diagnosis in the medical community. However, it may also be due to the increased use of alcohol among Kentucky women. Other "rare" disorders, like diastrophic dysplasia, seem to be unusually the diagnosis in the medical community. However, it may also be due to the increased use of alcohol among Kentucky women. Other "rare" disorders, like diastrophic dysplasia, seem to be unusually

Chromosome Aberrations↗

PubMed1996

Cutaneous neural heterotopias and related tumors relevant for the dermatopathologist.

Heterotopic neural tumors are rare in the skin; however, when encountered, they often pose a diagnostic problem. While the clinicopathologic features of most neuroaxis abnormalities are well-documented in the neuropathologic literature, their significance in cutaneous pathology as well as the associated nonneural dermatopathologic findings have not received enough attention. This report offers a comprehensive review of the most common cutaneous neural heterotopic abnormalities and their related tumors. The clinicopathologic features of the following entities along with their histogenetic considerations are discussed: classical and rudimentary meningocele, primary cutaneous meningioma, heterotopic brain tissue (nasal glioma), peripheral primitive neuroectodermal tumor, metastatic neuroblastoma, and ganglioneuroma. Familiarity with the associated dermatopathologic changes and with the differential diagnosis should assist in arriving at the correct diagnosis even without special training in dermatopathology or neuropathology.

Choristoma↗

PubMed1996

[Prevalence, clinical aspects and prognosis of neural tube defects in The Netherlands].

OBJECTIVE: To determine the live birth prevalence of neural tube defects (NTD) in the Netherlands and describe the clinical picture. DESIGN: Descriptive. SETTING: TNO Prevention and Health, Leiden, the Netherlands. METHOD: Data collected through active surveillance of NTD on a monthly basis by paediatricians throughout the country. RESULTS: From October 1993 to September 1995, 203 infants with NTD were registered. Six were diagnosed with anencephaly, 10 with encephalocele and 157 with spina bifida. On 30 children no further information was available. Seventy-nine per cent of children with spina bifida had a myelomeningocele and 11% had a meningocele. Hydrocephalus was found in 75% of all registered infants and in 81% of infants with myelomeningocele. In 18% of the latter group a kyphosis was diagnosed. In 45% of 131 children prognosis of ambulation fair: they were expected to learn to walk. In 32% prognosis for motor disorder was very poor, and unsupported sitting was considered to be unlikely. At the time of registration, when most children were less than a month old, 55 (27%) had died; in infants with a myelomeningocele this was 35%. In many children treatment had not been started, probably because the prognosis was poor. Some had not been referred to a multidisciplinary team and only one of them received a hydrocephalus valve. CONCLUSION: The prevalence of spina bifida in the Netherlands is at least 100 live-born children per year among 195,000 total births. Mortality in the first month is high and for many surviving infants a severe handicap can be expected. Preventive strategies (such as folic acid supplementation) are urgently needed.

Activities of Daily Living↗

PubMed1996

[Management of breach of the tegmen isolated or associated with cephalocele].

This is a retrospective report of 41 tegmen breaches observed in 2 different populations : 27 patients among a series of 252 cases of operated chronic otitis (6.7%) and 14 patients admitted for management of tegmen breach and dura mater involvement. The most frequent cause was chronic otitis (36 cases, including 6 due to iatrogenic causes); there were also 2 cases of petrus fracture, 2 cases of intracranial hypertension and 1 idiopathic case. Computed tomography revealed a bony breach in 97% of the cases. Encephalo-meningocele was associated in 11 cases (31%). In these cases, MRI confirmed the preoperative diagnosis. Treatment via the mastoid route allowed removal of the infectious process and via the suprapetrous route allowed closure of the breaches in the bone and dura mater as well as treatment of associated cephaloceles, except in case of major infection where the cephalocele was closed in a second operation.

Adult↗

PubMed1997

Neurosurgery in Rwanda during a United Nations peace-keeping mission.

An analysis of the neurosurgical component of the medical support provided by a United Nations peace-keeping mission in Rwanda is presented. The Australian Defence Force contingent provided medical support to the United Nations and the civilian population. Eight hundred thirty-eight procedures were performed during 12 months. A wide range of surgery was encompassed, with neurosurgery accounting for 17 (2%) of the total operations: compound depressed fractured skull, 5; intracranial pressure monitor, 2; burr holes for acute head injury and chronic subdural hematoma, 2; skull osteomyelitis debridement, 1; rib-graft cranioplasty, 2; scalp rotation flap, 1; congenital myelomeningocele, 2; occipital meningocele, 1; craniofacial approach to Le Fort III fracture, 1. A broad range of neurosurgical procedures have been performed. The overall numbers of neurosurgical operations were small, but they were successfully performed by general surgeons. Familiarity with neurosurgery is necessary in predeployment training of military surgeons working in a remote location with limited resources.

Adult↗

PubMedDate not supplied

Dural ectasia of the optic nerve sheath in neurofibromatosis type 1: MRI manifestations.

Patients with neurofibromatosis type 1 (NF-1) have an increased incidence of optic glioma. Although spinal dural ectasia or meningocele is well represented in the NF-1 literature, radiologists are not as familiar with dural ectasia of the optic nerve sheath as spinal dural ectasia. This is a report of a pediatric patient with NF-1 with dural ectasia of the optic nerve sheath. This is the second reported case of dural ectasia of the optic nerve sheath demonstrated by magnetic resonance imaging.

Cheek↗

PubMed1979

Prevention of neural tube malformation by genetic counselling, and prenatal diagnostic surveillance.

The aetiology and genetics of the spectrum of dysraphic neural tube malformations from anencephaly through encephalocele, myelocele and meningocele to complicated spina bifida occulta, is outlined. The risk of recurrence, the methods and problems of genetic counselling are discussed. All women known to be at risk for neural tube malformation should be counselled carefully and offered prenatal diagnostic surveillance for any future pregnancy. There is no longer any need for a couple able to accept termination, to go through with a pregnancy in fear of recurrence.

Amniotic Fluid↗