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Cervico-thoracic neurenteric cyst: clinicoradiological correlation with embryogenesis.

Two cases of cervico-dorsal neurenteric cysts are presented, one located dorsal and the other ventral to the spinal cord, with associated Klippel-Feil anomaly, meningocele, spinal dysraphism and possibly spinal cord malformation. One patient was operated on as a neonate for a cervico-thoracic meningocele and presented during adolescence with spastic quadriparesis. Imaging revealed spina bifida, a dorsally located neurenteric cyst and a tendency towards split cord malformation. The other child presented with cervico-thoracic kyphoscoliosis and paraparesis. Imaging demonstrated persistence of a cervico-thoracic remnant of the primitive neurenteric canal through the upper thoracic spine.

Child↗

Antenatal diagnosis of spinal lipomas.

OBJECT: The purpose of this study was to highlight the key prenatal diagnostic features of fetal spinal lipomas. METHODS AND RESULTS: All fetuses in whom a spinal lipoma was suspected prenatally or diagnosed postnatally were included in the study. Lumbosacral lipoma was diagnosed in 16 fetuses (group 1), but at birth 3 of these were found to have other forms of occult dysraphism. In 2 other fetuses (group 2) a meningocele was suspected but at birth these lesions were found to be lumbosacral lipomas. The lipomas were covering large meningoceles in 13 fetuses and filling the cul-de-sac in 2 others. Three sets of parents decided on termination. Of the 13 children born with a spinal lipoma, 9 were operated on and 4 were managed conservatively. At the last follow-up, 7 were neurologically intact. CONCLUSION: The distinction between meningomyelocele and spinal lipoma is possible antenatally. The differential diagnosis between lipoma and the other forms of occult dysraphism is more difficult.

Diagnosis, Differential↗

Cutaneous lesions in occult spinal dysraphism--correlation with intraspinal findings.

OBJECTS: This study was conducted to investigate the frequency and type of cutaneous stigmata in different forms of occult spinal dysraphism (OSD) and their correlation to the underlying malformation. METHODS: Fourteen different forms of spinal malformations were identified in 358 operated patients with OSD. Most frequent findings (isolated or in combinations) were spinal lipoma, split cord malformation, pathologic filum terminale, dermal sinus, meningocele manqué, myelocystocele and caudal regression. Stigmata were present in 86.3% of patients, often in various combinations. Using a binary logistic regression analysis, significant correlations with distinct malformations were found for subcutaneous lipomas, skin tags, vascular nevi, pori, hairy patches, hypertrichosis, meningoceles and "cigarette burn" marks. CONCLUSIONS: Cutaneous markers in a high percentage accompany spinal malformations. Due to the correlations of different stigmata to distinct malformations, they can aid the clinician in further diagnostic and therapeutic work.

Adolescent↗

Cystic spinal dysraphism of the cervical and upper thoracic region.

BACKGROUND: Cystic dysraphic lesions of the cervical and upper thoracic region are rare and only a few series have been published about the topic. These malformations can be divided into categories that include both myelocystoceles and the so-called cervical meningoceles or myelomeningoceles. METHODS: A retrospective study of 18 patients was conducted. RESULTS: In 17 patients a squamous or a cicatricial epithelium of variable thickness covered the dome of the lesions, while the base was covered with full-thickness skin. In one case the skin was entirely normal. Four patients displayed associated CNS malformations and three more had systemic congenital anomalies. All patients underwent surgical exploration and the length of time between birth and surgery ranged from 6 h to 9 months. The most frequent surgical finding, seen in 14 patients, was a stalk connecting the dorsal surface of the spinal cord to the cyst. In three patients the findings were consistent with myelocystocele. Only in one case was a true meningocele found. Hydrocephalus and Chiari II malformation were not as consistently associated as in myelomeningoceles. Neurological signs and symptoms were not so marked as in myelomeningoceles and were found in the follow-up of four patients. In two of them there was a non-progressive deficit, probably expressing an imperceptible involvement of the nervous system in the first year of life. The histopathological findings were of three types: neuroglial stalks, fibrovascular stalks and myelocystoceles. CONCLUSIONS: Cystic dysraphisms of the cervical and upper thoracic region differ clinically and structurally from meningomyelocele and have a more favorable outcome. We believe that these malformations have not been properly labeled and propose a classification based on the structures found inside the cyst.

Abnormalities, Multiple↗

Meningeal nodules in teratoma of the testis.

A case of a 52-year-old man with a mature adult teratoma is reported. Beside histologically mature tissues, this teratoma contained large areas of a meningiomatous proliferation in close proximity of a peripheral nerve and glial tissue. These meningiomatous proliferations were mostly seen in the peripheral parts of the teratoma surrounding the rest of teratomatous elements and were immunohistochemically EMA-positive and S-100 protein- and cytokeratin-negative. Identical meningothelial proliferations are well known in the skin and adjacent soft tissues of the scalp, where they have variously been called sequestrated meningoceles, meningeal hamartoma, cutaneous meningiomas, rudimentary meningocele, hamartoma of the scalp with ectopic meningothelial elements, or cutaneous heterotopic meningeal nodules.

Biomarkers, Tumor↗

Transsphenoidal cephaloceles in adults. A report of two cases and review of the literature.

Transsphenoidal cephalocele rarely occurs in adults. We describe two adult cases with transsphenoidal cephaloceles. The first case was a 53-year-old female who presented with spontaneous cerebrospinal fluid (CSF) rhinorrhea. Magnetic resonance (MR) imaging demonstrated a transsphenoidal meningocele. Surgical repair was attempted via the transsphenoidal route. Rhinorrhea recurred at one month and also two years later. Transsphenoidal surgical repairs were repeated. There has been no evidence of CSF leakage in the four years of follow-up after the last surgery. Transsphenoidal repair seems to be the most suitable approach in this case. The second case was a 26-year-old female with secondary amenorrhea, diabetes insipidus, bitemporal hemianopsia, and see-saw nystagmus. MR imaging demonstrated a transsphenoidal encephalocele with the optic chiasma and infundibular recesses descending into the meningocele and an agenesis of the corpus callosum. The first operation was performed via the transnasal route and the second by sublabial transmaxillary transsphenoidal approach six months later. Postoperative MR imaging revealed reduction of the encephalocele. There was neither worsening nor noticeable improvement in the neurological or endocrinological function. However, in this type, complete repair is often impossible, and non-radical surgery such as transsphenoidal repair may be indicated as most suitable. They should be selected with careful assessment referring to MR findings and clinical symptoms and signs.

Adult↗

Hereditary sacrococcygeal teratomas.

Hereditary presacral teratoma and its associated anomalies have been described in six kindreds. We report on a similar family where the father presents with a presumed meningocele and his two children with sacrococcygeal teratomas, associated with anterior meningocele in one.

Adult↗

A split cord malformation with paresis of the unilateral lower limb: case report.

BACKGROUND: We report a rare case of split cord malformation. CASE DESCRIPTION: A female neonate presented with split cord malformation (SCM) manifesting as right lower limb paresis. Myelomeningocele and meningocele were found in the lumbosacral region at birth. Magnetic resonance imaging (MRI) demonstrated division of the spinal cord into two hemicords below the T5 level. The right hemicord formed a hemimyelomeningocele and the left hemicord terminated in the low-lying conus. Three-dimensional computed tomography (3D CT) showed extensive vertebral body abnormalities. The hemimyelomeningocele was repaired 1 day after birth, and septectomy and the repair of the meningocele were performed when the patient was 1 year old. The patient has been followed up as an outpatient, and has residual right lower limb paresis. CONCLUSION: SCM can be associated with multiple spinal abnormalities. MRI and 3D-CT are useful for identifying such abnormalities and planning the surgical treatment.

Abnormalities, Multiple↗

Lumbar peritoneal shunting with video-laparoscopic assistance: a useful technique for the management of refractory postoperative lumbar CSF leaks.

BACKGROUND: Cerebrospinal fistulas and pseudomeningoceles can occur after lumbar spinal surgery, and are sometimes refractory to direct repair, external drainage, and blood patches. The authors report a technique for cerebrospinal fluid (CSF) diversion from the lumbar spine to the peritoneum to assist with the management of these difficult situations. METHODS: Using video-laparoscopic assistance, two shunts are placed from the lumbar region into the peritoneal cavity: first, a lumbar subarachnoid space to peritoneum shunt; and second, a meningocele cavity to peritoneum shunt. Patients are ambulated immediately after the procedure. External drains are not used. RESULTS: Four patients with refractory CSF leaks were successfully managed with this technique. Complications associated with prolonged bedrest and external drains were avoided. Ancillary procedures were minimized, and hospital stay was shortened. Laparoscopic assistance offered verification of accurate placement of the peritoneal catheter and shortened operative times. CONCLUSIONS: Dual lumbar peritoneal shunts (intrathecal-peritoneal and meningocele cavity-peritoneal), placed with laparoscopic assistance, proved effective in the management of four patients with postoperative lumbar CSF leaks, who had failed to respond to conventional treatment.

Aged↗

[Complications of endonasal paranasal sinus surgery--diagnostic and therapeutic consequences].

BACKGROUND: Functional endoscopic sinus surgery has been proven the therapeutic method of choice in surgical therapy of chronic sinusitis. On the other hand, endonasal sinus surgery may cause severe complications even when performed by a skilled surgeon. This is easily explained by the close vicinity of many functionally important structures to the operative site. CASE REPORTS: Three histories are reported that involve possible complications even in apparently simple cases. Diagnostic and therapeutic consequences are discussed. In a case previously diagnosed histologically as chronic unspecific sinusitis, an endonasal biopsy resulted in endocranial bleeding requiring neurosurgical intervention. Midline granuloma was found to be the correct diagnosis. Another patient was seen with a normal X-ray of the sinuses and solitary polypoid structure in his left nose. Polypectomy was planned and a CT scan was performed, which demonstrated a meningocele. Transfacial surgery was then performed to remove the meningocele. Another patient presented with a traumatic impression of the frontal sinus, and open reposition by transfacial surgery of the frontal and ethmoid sinus was planned. When CT scans revealed an uncovered optic nerve in the sphenoid sinus of the fractured side, we abandoned ethmodectomy and performed reposition of the frontal sinus as the only surgical procedure. RESULTS AND CONCLUSIONS: In this paper, we show typical complications of endonasal sinus surgery and strategies for avoiding them. If any complication occur, prompt treatment is required. Three groups of complications can be defined: perforation of frontobasal dura resulting in cerebrospinal fluid (CSF) fistula, severe bleeding, and orbital or optic nerve injury. When the surgeon discovers an intraoperative complication, possible consequences must be considered immediately to minimize side effects for the patient. A CSF fistula should be closed in the same procedure, and transfacial surgery may be necessary. Hemorrhage resulting from an ethmoidal artery may require frontoorbital surgery and ligation of this vessel. If retrobulbar hemorrhage caused by retraction of an ethmoid artery occurs, immediate intervention is necessary. Usually a transfacial approach, resection of the medial orbital wall and retrobulbar decompression are performed. In some cases lateral canthotomy may be the best way to drain haematoma and decompress the optic nerve. Subsequently, orbital revision and ligation of the retracted artery must be performed. Any delay can result in persistent visual loss. We conclude that the extranasal frontoorbital approach should be part of the residency training program in ENT departments. Any surgeon performing endonasal sinus surgery must be trained in transfacial emergency procedures, which should be part of anatomic preparations in teaching courses, thus avoiding severe damage in case of intraoperative complication.

Aged↗

Gender and spina bifida--some misconceptions.

1367 survivors (997 myelomeningoceles, 207 meningoceles, 81 lipomas of the cauda equina and 82 encephaloceles) were included in this study of sex differences. Contrary to widespread opinion, very few differences were observed. Myelomeningocele patients had significantly lower IQs, smaller heads and thinner brains. However, these differences were small and clinically inconsequential. There were no significant differences in degree of physical handicap. Any differences which were found tended to reflect research findings from the nonhandicapped population. Likewise, the males and females with meningoceles, lipomas and encephaloceles were remarkably similar in their intellectual and physical handicap characteristics. Sex differences in patients with neural tube malformations are of minimal significance and only likely to affect clinical decisions in the area of genito-urinary problems.

Adolescent↗

Pre-natal diagnosis of occult spinal dysraphism by ultrasonography and post-natal evaluation by MR scanning.

Eight children born with occult spinal dysraphism were diagnosed in utero by ultrasonography. Post-natally, they were evaluated by MR scans. The ultrasound scans in all 8 fetuses revealed a spina bifida: the spinal cord was long in 5 and in 3 a meningocele was thought to be present, however, in 1, a post-natal MR scan revealed a lipomeningocele instead of a simple meningocele. In 3 fetuses an echogenic area was seen on the ultrasound scan which raised the possibility that an intradural lipoma was present, and was confirmed by post-natal MR scans. In 3 fetuses vertebral body anomalies and an additional ossification centre in a midline bony spur together with widening of the spinal canal were seen in the lower lumbar region. The lesions in all 8 fetuses were skin-covered. None had ventriculomegaly or an Arnold-Chiari malformation. The prognosis for fetuses with spina bifida aperta is well documented in contrast to that for those with spina bifida occulta. The ability to identify a spinal dysraphic lesion pre-natally allows a more accurate assessment to be made of the likely outcome for an individual fetus.

Female↗

Growth of trunk and legs of children with myelomeningocele.

Total body length and body segments (crown-rump length, sub-ischeal length) of 78 children with myelomeningocele were measured at regular intervals during growth for a mean duration of 4.4 years. These children were shown to have defective growth, with an increased upper segment/lower segment ratio. It was found that the higher the level of the meningocele the greater the growth defects. However, the relationship was statistically significant only for the first seven years of life. It is concluded that neurological damage was mainly responsible for defective growth during the first years of life, but that other factors independent of the level of the meningocele also come into play in later years.

Anthropometry↗

Survival and handicap of infants with spina bifida.

A follow-up study was carried out on 213 infants born with spina bifida cystica (including encephalocele and occipital meningocele) from 1965 to 1972 to women resident in Oxfordshire and the western part of Berkshire. The 5-year survival rate was 36% (39/107) for those with open lesions, 60% (30/50) for those with closed ones, and 18% (10/56) for those with lesions which could not be classified (not known) but which were probably nearly all open. The extent of handicap among these survivors was assessed by means of criteria described by Lorber; among those with open lesions (including 'not known') 84% (41/49) were severely handicapped, 10% (4/49) were moderately handicapped, and only 6% (3/49) had no handicap; among those with closed lesions, 37% (11/30) were severely handicapped, 33% (10/30) were moderately handicapped, and the remaining 30% (9/30) were not handicapped. Closed head lesions (encephalocele or occipital meningocele) were more often associated with severe handicap (6/8; 75%) than were closed spinal lesions (5/22; 23%). The children with open lesions who survived for at least 5 years spent, on average, at least 6 months in hospital during the first 5 years of their life and had, on average, at least 6 major surgical operations. In comparison, those with closed lesions spent one-third less time in hospital, and had fewer than half as many operations. During the period of the study a selective treatment policy was adopted typical of that commonly practised now, and all the infants were born before antenatal screening had been introduced. Our results therefore may be helpful in assessing the benefits to be expected from antenatal screening for open spina bifida.

Encephalocele↗

Primary cutaneous extravertebral meningioma. Case report.

A case of cutaneous extravertebral meningioma is presented. It was diagnosed in infancy as a lumbar meningocele. Operation was initially refused but was subsequently demanded for cosmetic reasons. The findings were a very thick corrugated skin and a cutaneous meningioma connected by a fibrous tract to the dura mater. The presence of a fibrous stalk linking the tumor to the dura mater might have been the pathogenetic connection between the meningocele and cutaneous meningioma.

Adolescent↗

Terminal myelocystoceles: a series of 17 cases.

OBJECT: A terminal myelocystocele is a rare form of spinal dysraphism in which the hydromyelic caudal spinal cord and the subarachnoid space are herniated through a posterior spina bifida. In their study of 17 cases, the authors attempt to develop treatment guidelines for patients suffering from this condition. METHODS: Seventeen patients (age range 2 months-5 years) underwent surgery during a 7-year period. Surprisingly, nine of 17 patients had no neurological deficits. Among these nine asymptomatic cases, three patients had a dermal sinus and two had a skin dimple, whereas one had congenital talipes equinovarus deformity (CTEV). Among the eight patients with neurological deficits, seven had lower-limb weakness (two had complete paraplegia), and one patient had urinary incontinence and constipation. Four patients each had a dermal sinus and CTEV; interestingly, one infant had an accessory phallus. Radiologically, eight patients had pure myelocystoceles, and a diagnosis of lipomyelocystocele was made in nine instances on the basis of magnetic resonance images. All patients underwent surgery. The meningocele sacs were excised, and the bands tethering the myelocystocele sac to the meningocele sacs were incised, with as little damage to the roots as possible. Two patients experienced postoperative CSF leakage (one required reexploration), and two others had subcutaneous CSF collection. No patient had deterioration of the neurological condition after surgery. CONCLUSIONS: The authors present the largest study of patients with terminal myelocystocele to date and emphasize that the possibility of this condition must be kept in mind when addressing all congenital lumbosacral masses. The authors assert that myelocystoceles and lipomyelocystoceles should be kept in the same category and recommend early surgery in all cases.

Child, Preschool↗

[An infantile skull fracture followed by the enlarging of the fracture line ("enlarging skull fracture") (author's transl)].

We experience sometimes an infantile skull fracture which is followed by the skull fracture line and bulging of the fractured area day by day after the head injury. Since John Howship reported the case of the partial absorption of the right parietal bone, arising from a blow on the head in a child aged 9 month in 1816, this phenomenon was variously described meningocele spuria, traumatic cephalohydrocele, leptomeningeal cyst, fibrosing osteitis, cerebrocranial erosion, traumatic meningocele, die wachsende Schädelfrakture, growing skull fracture, etc. So called "growing skull fracture" has generally the triad of the symptoms which are the parietal skull fracture in infancy or childhood, traumatic dural tears, and subsequent enlargement of the fractures. And it is said that the dural tear is an indispensable condition for the developing of the "growing skull fracture". But we recently had the case of a 14 day old male infant who had neither traumatic dural tear nor subdural hematoma, but the progressive enlarging of the fracture line in the left parietal bone. The authors suggest that there should be the difference between the growing skull fracture (with the dural teat) and the enlarging skull fracture (without the dural tear).

Fractures, Ununited↗

[Analysis on 2158 neural tube defects diagnosed prenatally by ultrasound examination].

A retrospective survey was made to study the prenatal diagnosis on neural tube defects(NTD) by ultrasound examination from 1990 to 1993 in China. A total of 2158 livebirths and stillbirths affected by NTD with 28 weeks or more of gestation were investigated in 233 hospitals of 28 provinces, municipalities and autonomous regions all over the country. The results showed that 61.8% of the NTD were diagnosed prenatally, and 56.5% of the NTD were diagnosed by ultrasound examination. The ratios of anencephaly, myelocele and meningocele diagnosed prenatally by ultrasound examination were 66.7%, 62.5% and 52.5% respectively. Thoracic(46.4%) and lumbar (43.4%) spina bifida were diagnosed more frequently than cervical(35.5%) and sacral(31.7%) spina bifida. The ratios of prenatal diagnosis on NTD by ultrasound examination in provincial, municipal, county, and factory hospital units were 55.7%, 59.4%, 49.0% and 57.2% respectively. The ratios of prenatal diagnosis of NTD by ultrasound examination from 1990 to 1993 were 53.5%, 55.8%, 60.7% and 54.7% respectively. There was an increase in the prenatal diagnosis of NTD by ultrasound examination from 1990 to 1993. The ratios of prenatal diagnosis of NTD in county hospitals by ultrasound examination were relatively low. Anencephaly, myelocele and meningocele were more frequently diagnosed by prenatal ultrasound examination.

Adult↗