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PubMed2006

Teratoma or enterogenous cyst? The histopathological and clinical dilemma in co-existing occult neural tube dysraphism.

BACKGROUND: Better understanding of embryology, histopathology and genetics of dysraphic conditions have lead to an expansion of this concept to entities with a similar microscopic appearance (e.g. enterogenous cysts, colloid cyst of the III-rd ventricle) or rated among neoplasms (e.g. mature teratoma), creating a certain conceptual confusion. Currently the diagnosis of "teratoma" is being substituted by "enterogenous cyst" or "teratomatous cyst". AIM OF PAPER: Clarification of concepts in this field and presentation of the experience of the Department of Neurosurgery of the Children's Memorial Health Institute associated therewith. MATERIAL AND METHOD: Since January 1990 through April 2005 we had treated 7 children with the final diagnosis of "an enterogenous cyst". The mean age of the children was 10.5 years and the mean follow-up time was 4.1 years. The study was performed by a retrospective analysis of medical records, imaging studies and histological preparations. RESULTS: the study group included: 3 cases of type I enterogenous cyst, 1 case of type II cyst and 3 cases of type III cyst (in 2 cases a former diagnosis of "mature teratoma" has been revised). Within this follow-up time, a good outcome was obtained in 3 cases, moderate disability - in 3 cases and severe disability - in 1 case. CONCLUSIONS: (1) Enterogenous cyst often co-exists with other dysraphic features; (2) Clinical signs of an enterogenous cyst are non-typical and depend on location of the lesion. The presence of secreting gastric mucosa may lead to chemical myelitis; (3) Severity of the postoperative neurological deficit is due to the developmental nature of the lesion and common vascular supply; (4) Late results of treatment are satisfactory, provided the lesion is excised radically; (5) Enterogenous cyst may be diagnosed in the case of a tumor composed of tissues originating from 1, 2 or 3 embryonic layers coexisting with dysraphic stigmata, congenital vertebral abnormalities or a mediastinal tumor of the same type. Types II and III of enterogenous cyst were historically diagnosed as "adult teratoma".

Adolescent↗
PubMed1975

Lymphoreticular aggregates in the newborn lung and their relation to intrauterine INFECTION and neurospinal dysraphism.

A survey has been carried out of lymphoreticular aggregates in the lungs of 200 children, stillborns and dying within 15 days of birth and in a further 21 children with anencephalus, and controls. Lymphoreticular aggregates occur in response to amniotic infection and are found in the 'intrauterine infection syndrome'. They are not found in children showing early hyaline membranes. The time interval required for the production of lymphoreticular aggregates in the human child appears to be at lease 48 h and is probably 72 h before the production of well-formed aggregates and this probably applies to both the intrauterine and the postnatal state. There is a significant increase in the number of lungs showing lymphoreticular aggregates at birth in children in whom there is neurospinal dysraphism. In such children there is necrosis of nervous tissue into the amniotic cavity and it would seem likely that this material has been antigenic to the lung in utero.

Anencephaly↗
PubMed1976

[A case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis (author's transl)].

We presented a case of hemangioblastoma associated with spina bifida occulta, persistent metopic suture, thyroid adenocarcinoma, vertebro-occipital anastomosis and erythrocytosis. We have not found a hemangioblastoma with these associations, as far as we have seen in the literature. 36-year-old male was admitted with complaints of nausea, vomiting and ataxic gait in June, 1970. On admission, the examination revealed no evidence of increased intracranial pressure except for elevated CSF pressure by lumbar puncture and incoordination. The peripheral blood count disclosed slight erythrocythemia. Vertebral angiography revealed a vascular lesion of 2.0 cm in diameter situated almost in the midline of caudal cerebellum receiving its blood supply from the right posterior inferior cerebellar artery. In addition, a right vertebro-occipital anastomosis was visualized. Plain reoentgenograms showed persistent metopic suture and spina bifida occulta of C 5 - 6. After admission, installation of Ommaya reservoir and decompressive suboccipital craniectomy were performed, and a thyroid papillary adenocarcinoma was totally removed. After discharge, he had been well for two years until a month previously to the second admission, when he commenced to have again headache, nausea, and vomiting with ataxic gait. Vertebral angiography showed the tumor enlarged in size measuring 4.0 X 5.0 cm and the tumor stain was more irregular and less homogenous than 3 years before. Brain scan revealed an increased uptake in the midline of the posterior fossa. After readmission, in April, 1973, he gradually developed dysphagia, disturbance of articulation and inactivity of mentality and died from pneumonia in October, 1974. Autopsy revealed a vascular tumor originated from the medial portion of the right cerebellum and the tumor showed multiple cyst formation in the rostral part in contrast to the caudal solid mass. Histologically the tumor tissue was composed of capillaries supported by fine argyrophilic fibers, large clear interstitial cells containing lipid granules and hemosiderin pigment. Carcinoma of the right lobe of the thyroid was found with metastasis to the bone marrow, lungs and anterior cervical lymphnodes and lymphnodes at the left supraclavicular angle. Bone marrow showed marked erythropoiesis. The case reported here provides an evidence to suggest that there is more than a random relationship between hemangioblastoma, dysraphic state and thyroid carcinoma. The other association, the vertebrooccipital anastomosis may result from the enhanced demand of blood supply by hemangioblastoma but this speculation needs further examination.

Abnormalities, Multiple↗
PubMed1992

Experimentally induced axial dysraphism and anorectal malformation in male rat fetuses by intragastric administration of ethylenethiourea.

Pregnant Sprague Dawley rats were separated into two groups. In the experimental group, single intragastric doses of ethylenethiourea (ETU) were given on the 11th day of gestation. In the control group, a single intragastric dose of distilled water was given on the same day of gestation. Fetuses were recovered on day 20 of gestation, and were prepared for dissection and light microscopy. A high incidence of axial dysraphic disorders (omphalocele and rachischisis) and imperforate anus with recto-urethral fistula were noted in experimental male rat fetuses obtained from dams subjected to varying doses of ETU.

Abnormalities, Drug-Induced↗
PubMed1992

Magnetic resonance imaging of progressive hydrosyringomyelia in two patients with meningomyelocele.

Two patients who postoperatively developed extensive multiseptated hydrosyringomyelia following surgical repair of a lumbal meningomyelocele are reported. Since MRI has been available, an increasing number of reports showed that MRI is useful in the diagnosis of hydrosyringomyelia. Hydrosyringomyelia can be considered as a dysraphic lesion. Etiology and pathogenesis of hydrosyringomyelia are still not fully understood. Probably arachnoidal adhesions and cord tethering in both patients may be potential factors in producing cystic degeneration of the underlying structure secondary to ischemia.

Child, Preschool↗
PubMed2005

Lateral sacral meningomyelocele as a gluteal swelling--an unusual presentation.

Paravertebral meningoceles are rare examples of craniospinal dysraphism. A young boy presented with a cystic swelling over left gluteal region with lumbosacral scoliosis. On exploration, he was found to have a laterally placed sacral meningomyelocele. The presence of an unusually located meningocele should be suspected when a paravertebral mass is associated with neurological deficit or deformity of the spine.

Buttocks↗
PubMed1991

Hurler's disease with diastematomyelia.

A case of Hurler's disease is reported in a child with a gibbous deformity of the lumbar vertebrae L1/L2 and a localized diastematomyelia of the spinal cord at L1/L3. The association of an enzyme disorder affecting connective tissue with spinal cord dysraphism has not been reported before, and may be fortuitous. However, as each condition is rare, the occurrence of both gibbous deformity due to Hurler's and diastematomyelia in the same child, makes it unlikely to be a chance association. We suggest that defective neurulation in this case may be related to mesodermal damage associated with mucopolysaccharidosis.

Brain↗
PubMed1980

Anomalies of heart, spleen, kidneys, gut, and limbs may result from an overdistended neural tube: a hypothesis.

A hypothesis is advanced that many congenital anomalies of non-neural organs may be produced by damage to their mesodermal or entodermal anlagen caused by overdistention of the embryonic neural tube. Evidence to support the hypothesis derives from: (1) seldom appreciated but unequivocal embryologic facts about prechoroid plexus neural tube morphogenesis; (2) an understanding of the role of neural tube overdistention in the production of various dysraphic conditions; (3) the frequency of association of non-neural anomalies with dysraphic conditions; and 4) an analysis of the anatomic features of the organ anomalies associated with dysraphism. The practical utility of the hypothesis is that (1) it helps explain a seemingly widely divergent subset of phenomenology and (2) it is testable. At present, treatment of children with congenital anomalies is largely palliative. Prevention of these distressing defects in the future will only be realizable if the mechanisms of their genesis are more clearly understood.

Abnormalities, Multiple↗
PubMed1998

A novel embryogenetic mechanism for Currarino's triad: inadequate dorsoventral separation of the caudal eminence from hindgut endoderm.

Currarino's triad is a congenital malformation involving the combination of anorectal stenosis, a presacral mass (most often a teratoma or ventral menigocele) and an anterior sacral bony defect (scimitar sacrum). Current theories regarding its embryogenesis are difficult to reconcile with our current understanding of caudal neuraxial and hindgut development. Caudal neuraxial structures develop from the caudal eminence (or tail bud), which normally separates from the hindgut endoderm concurrent with ingrowth of the posterior notochord during late gastrulation. We describe the first reported association of Currarino's triad with a caudal split cord malformation. It has previously been proposed that split cord malformations and related 'complex dysraphic malformations' involving abnormalities of one or more of the three primary germ layers arise through disordered midline axial integration during gastrulation. The presence of a split cord malformation in a patient with Currarino's triad suggests that the two disorders share a common embryogenetic pathway. We propose that the malformations of Currarino's triad arise through a failure of dorsoventral separation of the caudal eminence from the hindgut endoderm during late gastrulation.

Anus, Imperforate↗
PubMed2000

Congenital arthrogryposis associated with atlantoaxial subluxation and dysraphic abnormalities. Case report.

The authors report the case of a 27-year-old woman with an arthrogryposis multiplex congenita (AMC) associated with atlantoaxial subluxation. To the authors' knowledge, this is the first report of its kind. The authors review the literature with reference to dysraphic abnormalities associated with atlantoaxial subluxation and with AMC. The patient presented with severe tetraparesis following a minor traffic accident. She underwent a procedure in which transoral decompression and dorsal stabilization were performed and, postoperatively, made a good clinical outcome. The authors stress the need for diagnostic neuroimaging of the craniocervical junction in patients with AMC.

Accidents, Traffic↗
PubMed1975

Surgical management of bacterial meningitis.

A variety of associated lesions may require the neurosurgeon's assistance in the management of bacterial meningitis. As treatment of this infection of the central nervous system proceeds, the surgeon will have to decide about the concurrent or subsequent operative treatment of congenital dysraphic states, paraneural infections, compound fractures or penetrating wounds of thecranium or spine, or infected bypass shunts for cerebrospinal fluid (CSF). In patients with intractable meningitic infections the surgeon may have to insert a ventricular drainage-irrigation system to permit adequate perfusion of the CSF pathways with antibiotic. Hydrocephalus or subdural effusions complicating meningitis may bring the patient to the surgeon long after the infection has been cured. This paper examines these problems and outlines the current principles of management.

Age Factors↗
PubMed1995

Omphalocele-exstrophy-imperforate-anus-spina bifida (OEIS) complex in a male prenatally exposed to diazepam.

A male clinically affected by the OEIS complex was studied. His mother, aged 30 years, has an affective disorder and ingested 30 mg of Diazepam daily, from 3 months previous to the gestation and during the entire pregnancy. At birth, a closure during the entire pregnancy. At birth, a closure defect of the anterior abdominal wall, exstrophy of hemibladders, exposure of intestinal epithelium, abnormal pelvis, imperforate anus, and bifid penis were noted. Birth weight was 3600 g and other measurements were not recorded. Colostomy was performed in the postnatal period followed by partial closure of the abdominal wall defect, and iliac osteotomies. At six years, 6 months of age, physical examination showed somatometric measurements around the third percentile (height 109 cm, weight 17 kg, cephalic circumference 48.5 cm). Clinically he presented mild mental retardation, functional colostomy, incomplete closure of the vesical exstrophy, imperforate anus, bifid penis and scrotum, descended testes, diastasis of pubis, lumbosacral scoliosis and shortening of the left leg (clinical photograph of the external features is not included as we were not able to obtain authorization to do so). Radiological studies (Figure 1) revealed wide separation of the ischiopubic bones; lumbosacral region with rotoscoliosis, platyspondyly and dysraphism; left coxa valga, and right coxa vara. The abdominal ultrasonographic studies showed unilateral renal agenesis (left). Chromosomal analysis (GTG bands) in peripheral blood lymphocyte cultures demonstrated a normal 46,XY constitution. Exposure to other substances, particularly alcohol, were excluded with the study of the mother's medical history and through information obtained from relatives.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Drug-Induced↗
PubMed1975

[Osseous anomalies of centrofacial lentiginosis (39 cases) (author's transl)].

The authors present the results of an investigation on 39 cases of centrofacial lentiginosis. Eleven patients urged to be examined because of their alarming health condition, and 28 cases were found out following an inquiry within some collectivities. With centrofacial lentiginosis the cutaneous hereditary polydysplasia (characterized by typical lentigines), the dysraphic state and neuro-psychic troubles are usual and awaken the interest of the radiologist due to the frequency of various dysmorphoses, growth disturbances and a trend for arthrosis. The radiologist has to pay a due attention to these aspects representing as many manifestations of a disease of a hereditary-family type since a possible centrofacial lentiginosis also means a possible disabling potential associated with education and vocational training problems.

Adolescent↗
PubMed2006

[Clinical and electroencephalographic analysis of epilepsy in children with hydrocephalus].

Clinical and electroencephalographic features of epilepsy and the prognosis of intelligence were investigated in 156 children with hydrocephalus. Of these 53 (34.0%) had epilepsy. The incidence and outcome of epilepsy were determined by the etiology of hydrocephalus. The incidence was high in children with hydrocephalus caused by intra-cranial infection and dysgenetic hydrocephalus without dysraphism. Furthermore, it was difficult to control the epileptic seizures in these patients. In contrast, children with dysgenetic hydrocephalus caused by dysraphism and simple hydrocephalus had lower incidence of epilepsy and epileptic seizures had been well controlled in these cases. In hydrocephalic children, localization-related epilepsy was the most common. Complex partial seizures and focal motor seizures were frequently observed. Common electroencephalographic findings were focal spikes or multi-focal spikes. The intelligence quotient was significantly lower in children with epilepsy than in those without epilepsy. All these findings suggest that the incidence of epilepsy may be a crucial prognostic factor in children with hydrocephalus.

Adolescent↗
PubMed1989

Deposition of laminin and fibronectin in dysraphic mutant mice: an immunofluorescent study.

Abnormal loop-tail (Lp/Lp) mutant mouse embryos exhibiting severe exencephaly and myeloschisis were analyzed and compared with their normal (+/+; Lp/+) littermates by means of immunofluorescence histochemistry to determine regional differences in the distribution of laminin (L) and fibronectin (FN). In the neural basement membrane and adjacent mesenchymal cell matrix of the abnormal embryos, regional differences in the deposition of L and FN were similar to those in normal littermates. Moreover, most of the putative neural crest (NC) cells appeared to emigrate normally in terms of their site of detachment and migration pathways, despite the severe topographic distortions and loss of neuroepithelial integrity. However, some putative NC cells projected incorrectly from the 'luminal' surface of the neuroepithelium, suggesting that some of the NC may be abnormal or sequestered and prevented from appropriate detachment and emigration from the neural tube.

Animals↗
PubMedDate not supplied

Occult dysraphism in adulthood. A series of 24 cases.

The results of a retrospective study of 24 adult patients with occult dysraphism are described. There were 15 males and 9 females, with an average age of 31.1 years. Specific circumstances precipitated symptomatic onset in 67% of patients. Pain, often referred to the anorectal region, was the most common presenting symptom. Bladder and bowel dysfunction were also common findings. The most common tethering lesions were intradural lipoma and a short thickened filum terminale. Myelography revealed the diagnosis of tethered conus in most cases, but the addition of CT and MRI images provided valuable structural details. The surgical outcome was gratifying in relation to pain and sensory-motor deficits but disappointing in the resolution of sphincter disorders. Our conclusion is that symptoms and/or signs of TCS with onset in adult life are not invariably irreversible.

Adult↗
PubMed1975

[Two cases of spina bifida cystica with congenital dermal sinus].

Spina bifida cystica and congenital dermal sinus are congenital anomalies which are called dysraphic states. Numerous reports of congenital dermal sinus can be seen in Europe and America, but in this country we have been able to find no more than six reported cases. Also in this country, to the best of our knowledge, only two cases of combined spina bifida cystica and congenital dermal sinus have been seen. The small incidence of reported cases may be due to the fact that congenital dermal sinus tends to go unnoticed except when infection develops. Consequently when dermal sinus is asymptomatic, even in both combined cases, it probably is thought to be only spina bifida. We have seen the combination with congenital dermal sinus in two among twenty cases of spina bifida cystica which we have observed during the past seven years. We report on this and discuss it in relation to the other literature.

Dermoid Cyst↗