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[A case report of a scalp dermoid cyst containing watery-clear fluid].

We report a case of a 7-month-old female with a dermoid cyst on the anterior fontanelle. She was born with a vacuum extractor. Two weeks later, her scalp on the anterior fontanelle bulged. A doctor was consulted when she was 3 months old, because the tumor was enlarging. Magnetic resonance image (MRI) showed a cystic mass on the anterior fontanelle. She was brought to our institute. At the first medical examination, she was alert and had no neurological deficit. The tumor was suspected to be a sinus pericranii or a pseudo meningocele. She was observed as an out patient, but the tumoral size become more enlarged. When she was seven months old, we punctured the cystic tumor and the tumor collapsed. But, two weeks later, it enlarged again. The cystic fluid was watery clear and the composition differed from cerebrospinal fluid (CSF). The tumor was resected totally. The histological examination revealed a dermoid cyst which involved ducts of the eccrine glands and folliculi pili. The cystic fluid was thought to be secreted from the eccrine glands.

Dermoid Cyst↗

[Endoscopic endonasal diagnosis and treatment of basal liquorrhea and encephalocele of the anterior regions of the skull base].

Endoscopic endonasal plastic repair of cerebrospinal-fluid fistulas and meningocele of the anterior regions of the skull base is an effective and miniinvasive procedure as compared with transcranial interventions. Endoscopic techniques may be used to close skull base defects in the nasofrontal pocket, ethmoidal infundibulum, cribriform lamina, sphenoid sinus. Limitations on the use of this procedure are cases of a fistula located within the posterior wall of the frontal sinus, within the lateral wall of the large sphenoid sinus, huge or multiple posttraumatic defects of the skull bases, as well as a combination of liquorrhea basalis and acute brain injury. According to latest papers, the successful outcome of endoscopic plastic repair ranges from 86 to 97%.

Cerebrospinal Fluid↗

An unusual presentation of occult spinal dysraphism.

Occult spinal dysraphism can lead to irreversible neurological complications, early diagnosis and treatment are necessary. It can be suspected from the presence of any cutaneous abnormality. We report a case with bony spur formation on the top of the 5th lumbar vertebra spinose process covered with skin mimicking a meningocel sac. By the help of this bony spur tethered cord could be diagnosed before any neurological deficit.

Bone Diseases↗

[Our experience in the prenatal echographic diagnosis of spinal dysraphism in the fetus].

The investigation consists of 141 cases of prenatal diagnosing structural and chromosomal anomalies of the fetus. Seventeen of the cases, quoted above, concern spinal dysraphism (spina bifida, occipital meningocele, meningoencephalocele). It has been made an analysis of ultrasound signs at this group of defects which turned out the most useful for the definite diagnosis. According to the literary survey and author's experience are formed two main groups of indices at echo-sounding study--direct and indirect. There have been considered the technics of echoscopy in searching the signs of different terms of pregnancy. It has been made a partial comparison of the reliability in the two groups ultrasound signs with this in biochemical diagnosis of spinal dysraphism in the fetus.

Acetylcholinesterase↗

[Cervical root avulsion presenting proximal segmental muscular atrophy of unilateral upper extremity].

A 56-year-old woman noticed non-progressive weakness in the proximal part of the right upper extremity from her childhood. At the age of 37 years, she was diagnosed as "spinal muscular atrophy" by an orthopedic surgeon. At the age of 56, neurological examinations revealed muscular atrophy and weakness confined to the right deltoid, biceps brachii and brachioradialis together with minor sensory disturbance in the lateral side of the right shoulder and forearm without pyramidal sign in the lower extremities. The neurological features of this case differed from those of juvenile type of distal and segmental muscular atrophy of upper extremities in distribution of muscular atrophy, and simulated those of cervical spondylotic amyotrophy. Myelography demonstrated root avulsion of the right C5 and C6 roots. CT myelography revealed traumatic meningocele. Therefore a clinical diagnosis of cervical root avulsion resulting from unrecognized birth injury was made. The reason of motor dominant pictures of this case may be vulnerability of the anterior nerve roots to traction injury.

Arm↗

Head injuries in infants and young children.

Head injury in infants and young children may produce lesions that are relatively unique for this age group. The uniqueness is generally due to the structural immaturity of the skull, meninges and brain."Derby-hat" and diastatic fractures are common in this age group. Spurious meningoceles result from tearing of the dura which is closely adherent to the skull. The syndrome of "delayed" concussion is more commonly manifested in children. Extradural and subdural hemorrhage may develop from lacerations of the major venous sinuses. A classical extradural hematoma may occur in the absence of fracture across meningeal arterial channels. The management of patients with head injury has been improved by the more frequent use of tracheotomy, hypothermic techniques and drugs of the "lytic cocktail." Solutions of urea in 10 per cent invert sugar are administered intravenously to control cerebral edema in selected patients.

Brain↗

[Evaluation of rhinorrhea by computed tomography cisternography].

PURPOSE: We aimed to emphasize the indication for and importance of CT cisternography in the diagnosis of rhinorrhea patients by reviewing the literature with the support of unenhanced cranial CT findings. MATERIALS AND METHODS: Unenhanced cranial CT with axial sections and CT cisternography with coronal sections were performed in 14 patients who had spontaneous (n = 3) and posttraumatic (n = 11), intermittent or continuous CSF rhinorrhea. The slice thickness of infratentorial planes was 5 mm and supratentorial planes 10 mm. CT cisternography was performed from nasion to dorsum sella with a 3 mm slice thickness and 3 mm intervals. Bony and/or dural defects with entry of contrast into the ipsilateral paranasal sinuses were accepted as positive results. RESULTS: A CSF fistula was determined in nine of eleven posttraumatic patients. Three of them had accompanying cerebral herniation while on had a pseudomeningocele. The etiology of two spontaneous rhinorrhea patients was shown to be empty sella and meningocele pouch respectively. Eleven patients (nine posttraumatic, two spontaneous) whose CSF fistula had been shown on CT cisternography were operated on and our findings were confirmed. CSF fistula or bony defect was not observed in the CT cisternography of 3 biochemically proven rhinorrhea patients (2 posttraumatic, 1 spontaneous). These 3 patients were clinically observed and their rhinorrhea ended spontaneously. One posttraumatic patient who had had 2 operations had recurrence of rhinorrhea with an infected extradural collection six months after the operation. Another posttraumatic patient developed meningitis before CT cisternography. CONCLUSION: CT cisternography should be the preferred examination method in rhinorrhea patients, since it is cheaper than MR cisternography, is a widely used scanning technique and reduces the risks of surgical recurrences by showing the exact location of the fistula.

Adolescent↗

The pathology of extracranial scalp and skull masses in young children.

OBJECTIVE: Extracranial subcutaneous masses involving the scalp and/or skull in young children are uncommon lesions that get excised by the neurosurgeon. Although the most common reported lesion is the dermoid cyst, our experience suggests that the spectrum of pathology in these lesions can present diagnostic challenges to the pathologist. MATERIAL: We reviewed 30 consecutive extracranial masses from 29 patients between July 1998 and June 2003. METHOD: Hematoxylin and eosin-stained sections were reviewed in all cases, and immunohistochemistry was performed in select cases. RESULTS: Twenty-three were within the scalp, 5 involved the scalp and skull and 2 were within the limits of the inner and outer tables of the skull. There were 8 dermoid cysts, 2 epidermoid cysts, 6 post-traumatic lesions including 3 calcified cephalhematomas and 3 pseudocysts, 5 vascular lesions including 3 capillary hemangiomas, 1 venous angioma and 1 lymphangioma, 2 cases of cranial fasciitis and 1 case each of benign teratoma, deep granuloma annulare, benign fibrous histiocytoma, congenital melanocytic nevus, hamartoma with ectopic meningothelial elements, cutaneous hyalinised ectopic meningioma and a meningocele with a fibrohistiocytic reaction. No lesions have recurred or exhibited malignant features. CONCLUSIONS: Surgical pathologists and neuropathologists should be aware that the differential diagnosis of "lumps and bumps on babie's heads" is quite varied and can be histologically challenging.

Child, Preschool↗

Postoperative spinal ultrasonography findings in spinal dysraphia.

Diastematomyelia is a form of spinal dysraphism involving sagittal clefting of the spinal cord, conus medullaris, and/or filum terminale into two hemicords. It can be an isolated finding or can be associated with meningomyelocele or meningocele. In this report, we present postoperative spinal ultrasonography findings in a patient with diastematomyelia and a tethered cord.

Adolescent↗

Chiari type II malformation: a case report and review of literature.

INTRODUCTION: Herniation of cerebellar vermis through the foramen magnum, internal hydrocephaly and spina bifida cystica are the major signs of Chiari type II malformation. Spina bifida cystica (1 in 2000 neonates) is very often the first clinical manifestation of the disease. AIM: To discuss the pathomorphology, clinical picture and possible treatment of this underestimated malformation. PATIENTS AND METHODS: Lumbosacral spina bifida aperta and flaccid paraplegia of the lower limbs were found in a female newborn. Later on, pneumonia and evidence of markedly expressed internal hydrocephaly were found. At 48 days of age, surgical correction of the meningocele was undertaken. There was a sudden heart and respiratory arrest at the end of surgery but in spite of the cardiopulmonary resuscitation the infant died 24 hours later. RESULTS: Postmortem pathological examination revealed expressed internal hydrocephaly, small posterior fossa, herniation of vermis and atrophic medulla oblongata; presence of these signs verified the Chiari type II malformation. It is very difficult to diagnose this malformation antemortem without magnetic resonance imaging. Brainstem dysfunction is the most common cause of death in children under 2 years of age with Chiari type II malformation. Its clinical manifestation can be episodic and poorly expressed. CONCLUSIONS: A thorough understanding of this entity (clinical and pathomorphological manifestations) and magnetic resonance imaging are mandatory for the malformation to be diagnosed. Early recognition of symptoms of brainstem compression and a subsequent surgical decompression can decrease the high mortality rate among children with Chiari type II malformation.

Arnold-Chiari Malformation↗

[A case of atretic cephalocele].

We report a case of atreric cephalocele. A 13-year-old boy who was developmentally normal was admitted to our hospital, complaining of scalp mass located at the midparietal region. He was noticed to have had it since his birth. Physical examination revealed 2.5 x 2.5cm, round, skin-covered midline subscalp mass with a hairless top. Bone 3D-CT showed bone defect at the base of the mass. MRI and MR venography showed the embryonic positioning of the straight sinus. He was treated with surgical excision of the mass, and fibrous tract extending intracranially at the base of the lesion was identified. Pathologic examination showed it to be meningocele.

Adolescent↗

Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part XII: appendix.

In an eleven part series published in Pathologica, we have presented various tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue (ST), which emerged as new entities or as variants of established entities during the last quarter of a century. Detailed clinicomorphological and differential diagnostic features of approximately sixty entities were chosen on the basis of their clinical significance and morphologic distinctiveness. The series included fibrous and myofibroblastic tumors (e.g. solitary fibrous tumor, high grade classic and pigmented dermatofibrosarcoma protuberans, inflammatory myofibroblastic tumor and myofibrosarcomas), fibromyxoid and fibrohistiocytic neoplasms (e.g., Evans' tumor, phosphaturic mesenchymal tumor, inflammatory myxohyaline tumor), special adipocytic/vascular/and smooth muscle lesions (e.g., chondroid lipoma, Dabska's tumor, ST hemangioblastoma, lipoleiomyosarcoma), epithelioid mesenchymal malignancies of diverse lineages (e.g., epithelioid liposarcoma, proximal-type epithelioid sarcoma, neuroendocrine extraskeletal chondromyxoid sarcoma), ST Ewing's tumor and peripheral nerve sheath tumors (perineuriomas and pigmented and rosetting tumors of the schwannoma/neurofibroma group), extranodal dendritic or histiocytic proliferative processes (follicular dendritic cell sarcoma, Rosai-Dorfman disease, Castleman's disease, and plexiform xanthomatous tumor), and tumors with myoepithelial differentiation. The section devoted to selected pseudotumoral entities considered representatives of the hamartoma group (neural fibrolipomatous hamartoma, ectopic hamartomatous thymoma, rudimentary meningocele), metabolic diseases (amyloid tumor, nephrogenic fibrosing dermopathy, tophaceous pseudogout, pseudoinfiltrative parathyromatosis), stromal tissue reactions to trauma (fibroosseous pseudotumors of digits) and infections (bacillary angiomatosis), and normal organs (glomus coccygeum). To conclude the descriptive phase, supplementary material has now been collected and appended in an attempt to provide a quick digest of essential knowledge both for comparison and differential diagnosis. The data have been tailored to synthesize diverse sources, integrating clinical elements and references to articles that previously appeared in Part I ("Introduction"), Part II ("The List and Review of New Entities") and Parts III to XI ("Excerpta"). At the very least we hope this final part ("Appendix") will provide the reader with a useful tabular organization of ST lesions and a reference resource.

Humans↗

["Clam" enteroplasty in the treatment of unstable or low compliance bladders].

The treatment of urinary incontinence due to unstable bladder remains difficult. In the case of failure of medical or physiotherapeutic treatments, clam enterocystoplasty represents a major and important therapeutic possibility. The operation consists of frontal section of the bladder to form a bivalve followed by interposition, between the two valves, of a detubed ileal graft in order to increase the functional vesical capacity and to interrupt the transmission of detrusor contraction waves from one valve to the other. 10 patients (7 men and 3 women) suffering from urinary incontinence due to neurogenic unstable bladder (4 cases: 3 meningoceles, 1 operated cauda equina neuroma) or to another cause (6 cases, including one sequela of radiotherapy for prostatic cancer and 5 cases of apparently primary urinary incontinence) underwent enterocystoplasty combined, in the case of neurogenic incontinence (2 cases simultaneously and 2 cases previously), with the insertion of an AMS 800 sphincter. No major complication was observed. 9 patients are continent and 1 had to be reoperated to undergo an augmentation enterocystoplasty (failure of the initial operation due to radiation changes of the bladder), 3 retain a post-voiding residual of about 200 ml but not requiring self catheterisation due to the absence of any repercussions on the upper urinary tract. Lastly, urodynamic studies demonstrated a spectacular increase in compliance and functional capacity of the bladder together with a reduction of the amplitude of intravesical pressure peaks.

Adolescent↗

MR imaging of spinal neurofibromatosis.

The MR findings in 7 patients with neurofibromatosis involving the spine were evaluated. Six patients had paraspinal tumors at multiple levels. In 4 they were bilateral. Five patients had multiple intraspinal lesions, frequently with growth through the neural foramen. In one patient paraspinal tumors were found in the lumbar sacral plexus and in another bony dysplasia and meningoceles but no tumors were disclosed. In 2 patients the lesions were associated with bilateral acoustic neuromas and multiple intracranial meningeomas. In one of these a spinal meningeoma with signal characteristics close to spinal cord was found. The other tumors had a signal that was equal to or slightly lower than the spinal cord and slightly higher than muscle on T1-weighted images. On T2-weighted images the tumors had a markedly increased signal compared to surrounding tissue. In 3 patients with tumors larger than 4 cm the signal intensity was inhomogeneous with decreased signal in the center on T2-weighted images, indicating the presence of increased fibrous tissue. Gadolinium-DTPA was given to one patient with marked increase in intensity of small tumors on T1-weighted images. The study shows that MR imaging is the modality of choice for evaluating most aspects of spinal and paraspinal neurofibromatosis.

Adolescent↗

[Congenital serous cyst of the orbit with anophthalmos].

The authors reported a case of orbital congenital serous cyst with anophtalmos. It was a third type of Duke Elder classification. Although it looked sporadic, this malformation is hereditary and due to a disharmony between optic cup growth and differenciation. Ultrasonography and scanner could confirm the diagnosis and helped to discard orbital and eyelid angioma and meningocele. After excision, a prosthesis must be adapted in the orbit.

Anophthalmos↗

A clinical study of spontaneous CSF rhinorrhoea.

A retrospective study of 10 patients presenting with spontaneous (normal pressure) CSF rhinorrhoea was performed. The epidemiology, role of radiology and surgical treatment are discussed. Nose blowing was found to cause onset of rhinorrhoea in 30% of patients studied. The presence of congenital dehiscences and the formation of small meningoceles are the most likely aetiological basis for this condition. The Valsalva like effect of nose blowing may cause dural rupture at sites of dehiscence thereby leading to rhinorrhoea. Contrast CT imaging identified the site of leakage in 70% of cases and further identified the side of leak in 20%. Contrast CT imaging is, therefore, the best modality for investigating these patients. Extracranial methods successfully repaired 88% of leaks compared with 50% for craniotomy. Extracranial techniques should be the primary surgical option since they have higher rates of successful repair and less morbidity than craniotomy.

Adult↗

[Concept, monitoring program and results of ambulatory care of children with meningomyelocele (spina bifida)].

The ambulant long term care for children with meningomyeloceles requires the interdisciplinary cooperation of pediatricians, pediatric neurologists, surgeons, orthopedists, radiologists and psychologists, social workers, physiotherapists and orthopedic mechanics. We present the concept for our out-patient consulting hour for 154 children and adolescents with meningomyeloceles, meningoceles and other forms of paraplegia, and the program of medical examinations for the detection of urologic malformations and the consequences of neurogenic disturbance of bladder function, neurologic complications (seizures, tethered cord etc). and dysfunction of liquor shunts. Additional items are the control of physiotherapy and the regular and adequate prescription and inspection of orthoses. The rate of complications is presented: revision of shunts 60%, seizures 17%, cerebral abnormalities 16%.

Ambulatory Care↗

Congenital intradural pathology.

Congenital spinal malformations discussed include myelomeningocele, tethered cord, diastematomyelia, diplomyelia, neuroenteric cysts, anterior meningocele, and Chiari malformations. The authors discuss the clinical presentation, radiologic evaluation, and treatment of these congenital anomalies.

Diagnostic Imaging↗