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PubMed1979

Extracranial CSF shunting for infantile non tumoral hydrocephalus--a retrospective analysis of 360 cases.

A series of 360 consecutive extracranial CSF shunts performed between June 1960 and June 1976 for infantile non tumoral hydrocephalus is reviewed. The surgical methods used, the reasons for re-operation, the time distribution of post-operative complications, the causes of death and the long term results are analysed and discussed for each of the four groups: tetraventricular communicating hydrocephalus, triventricular hydrocephalus, obstructive hydrocephalus with a ventriculo-cisternal block and post-meningocele or post-myelomeningocele hydrocephalus.

Bacterial Infections↗
PubMedDate not supplied

Posterior fossa cystic lesions--magnetic resonance imaging manifestations.

Cystic lesions of the posterior fossa remain a controversial subject as to clinical classification and diagnosis, especially for those in combination with other intracranial abnormalities. During the period of November 1985 to June 1991, 16 patients with cystic lesions of the posterior fossa were retrospectively reviewed on neuroradiological evaluation with MR images. The patients were 9 males and 7 females aged from 5 days to 15 years old (medium 1 year old). They were classified into three groups as Dandy-Walker cyst (group A, 5 patients), mega cisterna magna (group B, 4) and retrocerebellar pouch or cyst (group C, 7). Nine patients had associated intracranial anomalies, holoprosencephaly in 3 (group A, 3), dysgenesis of the corpus callosum in 4 (group A, 2; group C, 2), and occipital meningoceles in 2 (group C, 2). Hydrocephalus or ventricular enlargement was found in 10 patients (group A, 5; group B, 2; group C, 3). Surgical treatment (cyst-peritoneal shunt or cystectomy) was performed for 10 patients with clinical symptoms due to cyst. Postoperatively, clinical symptoms due to increased intracranial pressure or hydrocephalus improved in 7 patients. For long-term results, the cyst was reduced in 1 of 5 patients of group A and 2 of 5 of group C. Most of our patients have a poor prognosis because of the associated intracranial anomalies or atrophy of the cerebellum. Multiplanar MR images may provide sufficient evidence for the diagnosis of posterior fossa cysts, especially in the case of rotation or upward displacement of the cerebellar vermis. However, the cyst membrane, and the communication of fluid between the cyst and the cistern, cannot be demonstrated on MR images. Further investigation on these subjects and indications for surgical intervention is highly necessary.

Adolescent↗
PubMed1996

[Neonatal rhinopharyngeal obstruction due to craniopharyngioma].

BACKGROUND: Craniopharyngioma, the most common tumor of the sellar and suprasellar regions in childhood, can exceptionally develop in the infrasellar area. CASE REPORT: A premature girl (GA: 30 weeks) weighing 1240 g required intubation and ventilation because she suffered from a neonatal respiratory distress syndrome. Nasal endoscopy showed total obstruction of rhinopharynx by a tumor resembling adenoid tissue. This tumor was successfully excised and its histological examination showed a craniopharyngioma. CONCLUSION: A wide range of intranasal tumors has to be examined, endoscopy and CT scan are necessary for a precise localization and to eliminate meningocele.

Craniopharyngioma↗
PubMed2004

Imaging in spine and spinal cord malformations.

Spinal and spinal cord malformations are collectively named spinal dysraphisms. They arise from defects occurring in the early embryological stages of gastrulation (weeks 2-3), primary neurulation (weeks 3-4), and secondary neurulation (weeks 5-6). Spinal dysraphisms are categorized into open spinal dysraphisms (OSDs), in which there is exposure of abnormal nervous tissues through a skin defect, and closed spinal dysraphisms (CSD), in which there is a continuous skin coverage to the underlying malformation. Open spinal dysraphisms basically include myelomeningocele and other rare abnormalities such as myelocele and hemimyelo(meningo)cele. Closed spinal dysraphisms are further categorized based on the association with low-back subcutaneous masses. Closed spinal dysraphisms with mass are represented by lipomyelocele, lipomyelomeningocele, meningocele, and myelocystocele. Closed spinal dysraphisms without mass comprise simple dysraphic states (tight filum terminale, filar and intradural lipomas, persistent terminal ventricle, and dermal sinuses) and complex dysraphic states. The latter category further comprises defects of midline notochordal integration (basically represented by diastematomyelia) and defects of segmental notochordal formation (represented by caudal agenesis and spinal segmental dysgenesis). Magnetic resonance imaging (MRI) is the preferred modality for imaging these complex abnormalities. The use of the aforementioned classification scheme is greatly helpful to make the diagnosis.

Humans↗
PubMed2005

Pediatric paranasal sinus mucoceles: etiologic factors, management and outcome.

OBJECTIVES: To study the local and general etiological factors of paranasal sinus mucoceles in the pediatric population and to evaluate and discuss the clinical management and the results of endonasal marsupialization. DESIGN: Retrospective study. PATIENTS AND METHODS: A series of 10 cases of paranasal sinus mucocele in children were managed. For imaging, CT-scan and, sometimes MRI were performed. An ophthalmologic evaluation was performed preoperatively in all cases, and post-operatively in case of preoperative trouble. All patients were treated with endoscopic surgical procedure. Authors also looked for etiological factors. RESULTS: Cystic fibrosis was found in 6 cases out of 10. Others etiological factors were trauma and inflammatory process; one case was strictly idiopathic. Three patients out of 10 had ophthalmologic trouble related with the mucocele. With a mean follow-up of 17 months, neither recurrence nor complication were noted. All patients with ophthalmologic complain were free of trouble after surgery. CONCLUSION: Paranasal sinus mucoceles in children are still rare. In our experience, 9 out of 10 patients had predisposing factors, especially cystic fibrosis. Imaging with CT-scan and MRI allow the physician to rule out other tumors such as meningoceles or rhabdomyosarcoma. Endoscopic endonasal surgery is nowadays the gold standard for the treatment of paranasal sinus mucoceles.

Adolescent↗
PubMed2005

Intracranial complications of otitis media: 15 years of experience in 33 patients.

OBJECTIVES: Complications in the central nervous system (CNS) from acute otitis media (AOM) and chronic otitis media (COM) are becoming fewer, although they still represent a challenge for early recognition, adequate treatment, and satisfactory results. This retrospective study analyzed clinical data and therapeutic options in 33 patients with intracranial involvement resulting from otitis media. Important clinical features of intracranial complications and the sequence of the most efficient therapeutic maneuvers are discussed. METHODS: Charts of six patients with AOM and 27 patients with COM associated with CNS complications were analyzed for clinical presentation, imaging, and therapeutic approach. RESULTS: Ages ranged from 6 months to 79 years, with no gender predilection. Persistent fever, headache, and purulent otorrhea were the main symptoms. Proteus mirabilis , Enterococcus , and Pseudomonas aeruginosa were the most common microorganisms in COM, and Pneumococcus and Haemophilus were the most common microorganisms in AOM. Nineteen patients (58%) presented with more than 1 CNS complication, resulting in a total of 56 complications, including 26 cases of otogenic brain abscess, 21 cases of meningitis, 5 cases of lateral sinus thromboses, two cases of subdural empyemas, 1 case of epidural empyema, and 1 case of meningocele. Surgical interventions included craniotomy and drainage of the abscess (n = 17), open mastoidectomy with abscess drained through the mastoid (n = 10), open mastoidectomy alone (n = 8), and closed mastoidectomy (n = 2). Twelve patients who underwent craniotomy had subsequent mastoidectomy for recurrent abscess. At the 6-month, 66% of patients presented without sequelae, 24% presented with sequelae, and 9% died. CONCLUSION: Early identification and prompt clinical and surgical intervention with mandatory drainage of the middle ear (primary disease), was essential for better outcome.

Adolescent↗
PubMed2007

Brain malformation and infantile spasms in a SCAD deficiency patient.

This report presents a case of short-chain acyl-coenzyme A (CoA) dehydrogenase deficiency with a previously unreported presentation with brain malformations and infantile spasms. This female infant developed repeated tonic clonic seizures at the age of 3(1/2) months. She subsequently developed West syndrome at the age of 4 months. Her electroencephalogram disclosed hypsarrhythmia, and video-electroencephalographic monitoring confirmed the presence of infantile spasms. Magnetic resonance imaging revealed a small midline frontal meningocele, abnormal cortical gyration, and partial agenesis of the corpus callosum consistent with neuronal migrational disorder. Metabolic evaluation indicated ethylmalonic acidemia. Muscle biopsy with enzymatic assay of short-chain acyl-coenzyme A revealed low enzymatic activity confirming the diagnosis of short-chain acyl-coenzyme A dehydrogenase deficiency. To our knowledge, this is the first report of the coexistence of short-chain acyl-coenzyme A dehydrogenase deficiency, infantile spasms, and brain malformation. We conclude that short-chain acyl-coenzyme A dehydrogenase deficiency should be considered in the differential diagnosis of gyral abnormality, corpus callosal hypoplasia, and infantile spasms.

Agenesis of Corpus Callosum↗
PubMedDate not supplied

Orbital cysts of childhood--classification, clinical features, and management.

There is little in the literature about the clinical spectrum of orbital cysts of childhood and no comprehensive classification has been proposed. The authors propose a classification of orbital cysts of childhood and review their clinical features, pathology, and management. The major categories in the classification include cysts of surface epithelium, teratomatous cysts, neural cysts, secondary cysts, inflammatory cysts, and noncystic lesions with cystic component. Cysts of the surface epithelium are further divided into simple epithelial cyst (epidermal, conjunctival, respiratory, and apocrine gland), and dermoid cyst (epidermal and conjunctival). Epidermal dermoid cyst (dermoid) is by far the most common orbital cystic lesion in children, accounting for over 40% of all orbital lesions of childhood and for 89% of all orbital cystic lesions of childhood that come to biopsy or surgical removal. Neural cysts include those associated with ocular maldevelopment (congenital cystic eye and colobomatous cyst) and those associated with brain and meningeal tissue (cephalocele and optic nerve meningocele). The most important secondary cyst is mucocele that can occur in children with cystic fibrosis. Inflammatory cysts are generally due to parasitic infestations and are more common in tropical areas of the world. Noncystic lesions that can have a cystic component include adenoid cystic carcinoma, rhabdomyosarcoma, lymphangioma, and others. Each type of cyst has rather characteristic, but not pathognomonic, clinical features. Computed tomography and magnetic resonance imaging can help differentiate a cystic lesion from a solid tumor, suggest the type of cyst, and help in planning management. The pathology varies with the cells that line the cyst and with the inflammatory agent. Management varies from local excision to observation, depending on the location and type of cyst. Orbital cysts of childhood can be classified into categories, based mainly on their histopathology. The clinical, radiologic, and histopathologic features can be correlated with the classification in order to better evaluate a child with a cystic lesion in the orbit.

Adolescent↗
PubMed2006

Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: report of a case.

A case of epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma is reported. A newborn female presented with a large skin-covered mass arising from the palate, cleft palate, and lobulated tongue with a soybean-sized lingual lesion. The palatal and lingual masses were removed on the 9th day after birth. A cystic lesion was also found on the nasal septum after removal of the palatal mass; this lesion was resected at its base. Glossoplasty was performed after excision of the lingual lesion. Histologically, the palatal mass was diagnosed as epignathus. The cystic lesion was suspected to be meningothelial tissues on the basis of hematoxylin-eosin (HE) and immunohistochemical staining: Vimentin and epithelial membrane antigen were positive, and factor VIII was negative. The mass of the tongue dorsum was diagnosed as hamartoma. No recurrence of epignathus or evidence of meningocele have been observed after 5 years of follow up.

Abnormalities, Multiple↗
PubMed1993

CT cisternography in the investigation of cerebrospinal fluid rhinorrhoea.

Eighteen consecutive patients with cerebrospinal fluid (CSF) rhinorrhoea investigated by computed tomographic cisternography (CTC) are reported and the literature is reviewed. Twenty-one CTC studies were performed without complications on nine patients presenting with spontaneous CSF rhinorrhoea, six with head injury and three who had undergone craniotomy. Two meningoceles and two encephaloceles were found in patients with spontaneous rhinorrhoea. The site of the CSF leak was identified in 17 examinations (81%) and verified at operation in 14 patients--no surgery was performed following two examinations--and in one the site of leakage was not identified at operation. No defect was identified in three (14.3%) examinations and in one the result was equivocal (4.7%). CTC is an accurate, well-tolerated procedure and should be regarded as the method of choice for investigation of this condition.

Adolescent↗
PubMed1997

Outcome of 1040 consecutive breech deliveries: clinical experience of a maternity hospital in Turkey.

OBJECTIVE: To determine the optimal route of delivery in breech presentation. METHOD: One-thousand and forty singleton breech deliveries among a total of 41785 deliveries that occurred at Zubeyde Hanim Maternity Hospital between 1990 and 1994 were analyzed. RESULT: The breech deliveries accounted for 2.4% of all deliveries. Of the patients, 572 (56.3%) were delivered vaginally and 468 (43.7%) were delivered by cesarean section. The mean ages of vaginal and cesarean delivery groups were 25.2 and 24.6 years, respectively. Forty-one (3.9%) of the fetuses had congenital anomaly. The leading congenital anomalies were meningocele in seven and hydrocephalus in six patients. Mullerian anomalies were encountered in 29 (6.1%) patients at cesarean section. Fetal morbidity observed in cesarean deliveries were fracture of the humerus in one and fracture of the femur in one and soft tissue injury in four cases. Fracture of the humerus was observed in two, Erb's paralysis in four, facial paralysis in one and soft tissue injury in 43 fetuses delivered vaginally. The leading causes of puerperal maternal morbidity were genital tract lacerations in vaginal delivery group and wound infection in cesarean delivery group. The overall perinatal mortality of this series was 54.8/1000 (57/1040). The corrected figures for cesarean delivery and vaginal delivery groups were 8.5/1000 (4/467) and 57.9/1000 (32/552), respectively. CONCLUSION: We favor a selective approach for mode of delivery in patients with breech presentation in order to balance the fetal morbidity associated with vaginal delivery and maternal morbidity and cost associated with cesarean delivery.

Birth Injuries↗
PubMed1984

Imperforate anus: the neurologic implication of sacral abnormalities.

The association of imperforate anus with bony sacral abnormalities and neurologic deficits is well recognized. These neurologic deficits have been considered static rather than progressive. However, recent experience indicates that some patients may develop progressive neurologic problems due to spinal cord lesions that are amenable to neurosurgical correction. To investigate the frequency of such lesions, routine myelography of imperforate anus patients with sacral anomalies was undertaken. The extraordinarily high incidence of unsuspected lesions known to cause progressive bowel, bladder, and musculoskeletal dysfunction is the basis of this interim report. Thirty percent of patients with anorectal abnormalities had sacral dysplasia. Of the nine patients undergoing myelography to date, eight have been abnormal. Six children had a tethered spinal cord, one had narrowing of the bony spinal canal and dural sac stenosis, and one an anterior meningocele. Spinal cord conditions that may cause deterioration of bowel, bladder, and extremity function should be defined and corrected before irreversible damage occurs. Because of the high incidence of spinal cord lesions detected in these patients with coexisting anorectal and sacral anomalies, routine screening for spinal cord lesions is recommended.

Anus, Imperforate↗
PubMed1996

Prenatal diagnosis of abdominal wall defects and their prognosis.

To determine whether associated anatomic features may be useful in predicting fetal prognosis, 43 fetuses who had ultrasonographic evaluation for abdominal wall defects during a 13-year period were reviewed. Thirty-one fetuses had omphalocele and were classified into three groups according to fetal ultrasonography results: ruptured omphalocele with exposed liver, giant omphalocele with exposed liver, and small omphalocele without liver herniation. Twelve fetuses had gastroschisis. Ten of the 12 fetuses with gastroschisis survived; one died in utero. Nine of the 12 with a small omphalocele survived. Ten of 12 fetuses with giant omphalocele survived, but six of the seven with ruptured omphalocele died of pulmonary hypoplasia and respiratory insufficiency. Ruptured omphalocele recognized in utero was accompanied by intrauterine growth retardation and liver herniation and frequently was associated with deformity of the spine, diaphragmatic defects, vesicointestinal fissure, and meningocele. These results indicated that, together with the deformity of the spine, rupture and absence of the covering membrane with an exposed liver (noted through fetal ultrasonography) may suggest a poor prognosis because of pulmonary hypoplasia.

Abdominal Muscles↗
PubMed1998

Split notochord syndrome with prolapsed congenital colostomy.

A case of split notochord syndrome associated with a prolapsed colostomylike dorsal enteric opening, a foreshortened colon, imperforate anus, and meningocele is presented. The surgical management of this disorder is discussed and available literature is reviewed. The patient was successfully treated with a combined, single-stage surgical correction.

Abnormalities, Multiple↗
PubMed1998

Hydatid cyst: an unusual etiology for a cystic lesion of the posterior mediastinum.

The cystic lesions of the posterior mediastinum include bronchogenic, enteric and intramural esophageal cysts, lymphangioma, and anterior meningocele. Hydatid cysts are usually located in the liver, lung, and brain. Although many uncommon locations have been reported, hydatid cyst has not previously been encountered as an isolated posterior mediastinal lesion. A 4-year-old boy with a posterior mediastinal cystic lesion, which has been proven to be a hydatid cyst, is reported.

Child, Preschool↗
PubMed1977

Familial osteosclerosis with abnormalities of the nervous system and meninges.

A mother and daughter with osteosclerotic dysplasia are described. The daughter had generalized osteosclerosis, flattening of the angles of the mandibles, high-arched palate, mandibular and facial bone hypoplasia, a large sella turcica, and spacious foramen magnum, platybasia, basilar impression, widened spinal cord with enlarged intervertebral foramina, and scalloping of the posterior surfaces of the vertebral bodies. Radiographic contrast studies and operative intervention revealed multiple thoracic and lumbar meningoceles and an "empty" sella, as well as evidence of maldevelopment of the spinal cord, cerebellum, and cerebral cortex. Many of these skeletal features were noted to a lesser degree in the asymptomatic mother.

Abnormalities, Multiple↗
PubMed2006

[Focus on Dandy-Walker malformation].

The aim of this review is to summarize our knowledge about Dandy-Walker malformation (DWM) and introduce recent notions about its prognosis. DWM is a malformation associating hypoplasia of the vermis, pseudocystic fourth ventricle, upward displacement of the tentorium, torcular and lateral sinuses and anterio-posterior enlargement of the posterior fossa. It is frequently associated with genetic anomalies, brain malformations (anomalies of gyration, grey matter heterotopias, meningoceles, corpus callosum agenesis...) or systemic malformations (heart, orthopedic, intestinal, urogenital and facial anomalies). It is also part of many syndromes. Its rarity, the difficulty of its diagnosis, the fact that the malformation is mainly known through hospitalized patients, mainly from neurosurgical departments, have made its definition variable and prevents us from having an accurate knowledge of its natural history and prognosis. Hydrocephalus, so frequent in neurosurgical series that some have included it in the definition, is actually rare as has been seen in prenatal series. Treatment, when necessary, is still controversial, the main options remaining cyst fenestration, ventriculo- and/or cystoperitoneal shunts and more recently endoscopic third ventriculostomy. The prognosis, commonly said to be unpredictable, is actually foreseeable after scrutinous observation of the brain anatomy, and mainly of the vermis. A cerebellar vermis with three groups of lobes and two main fissures, as identified on MRI T2 sagittal views, not only has the greatest chance to not be associated with other malformation but also to have a favorable neurocognitive outcome. On the contrary, a deeply dysgenetic vermis with only two or one recognizable lobes is not only constantly associated with other brain malformation but also with poor prognosis. This is a recent advance that may be important for those involved in prenatal counselling.

Dandy-Walker Syndrome↗
PubMed2006

[Pedicle closing-wedge osteotomy for the treatment of fixed sagittal imbalance].

PURPOSE OF THE STUDY: The aim of this study was to analyze x-ray results in patients who underwent pedicle subtraction osteotomy for complex deformations (flat back, kyphosis) leading to fixed sagittal imbalance. MATERIAL AND METHODS: Thirty-four patients underwent surgery between 1996 and March 2003 in two centers, the orthopedic surgery unit devoted to spinal surgery and sports traumatology at the Strasbourg University Hospitals and the spinal surgery unit at the Chenove Clinic (Dijon). The series included 20 women and 14 men, mean age 46.5 years (range 14-74 years) treated for: postoperative flat back (n = 19), kyphoscoliosis (n = 6), ankylosing spondylarthritis (n = 5), and malunion (n = 4). After careful planning to achieve the necessary degree of correction, the patient was installed on a Cotrel table with a stable support enabling limited blood loss and facilitated reduction maneuvers via elevation of the lower limbs. The technique started by resection of the posterior arch, the transverse processes, and the pedicles, followed by partial resection of the body with a chisel under fluoroscopic control. The closure was achieved by in situ reduction with compressive rotation. Adjacent discs were then evaluated to search for any opening which could be filled by an anterior graft to complete the stabilization. The osteosynthesis used 2-, 4-, and 6-rod assemblies. The 6-rod assemblies enabled anchoring the fixation above and below before the osteotomy. Two prebent rods completed the assembly at reduction after osteotomy. Weight-bearing started on day 4. A protective corset was worn for three months by 19 patients. The patients resumed their occupational activities on average 6.73 months postoperatively without change in activity for 63%. Overall satisfaction was close to 90% excellent outcome. RESULTS: All clinical results were analyzed with the Profil software of Surgiview. X-ray data were analyzed with Spineview. A self-administered clinical questionnaire was used to evaluate pain, result of the operation, and the impact on the patient's body image. On average, the wedge osteotomy measured 28.9 degrees for correction of lumbar lordosis by 31.5 degrees with a maximum of 65 degrees. The misalignment at C7 was reduced from 51 mm to 2 mm. At the pelvic level, version improved 16 degrees, sacral slope 8 degrees. There were four dura mater breaches which had to be sutured, but with no clinical consequence (one progression to meningocele). Mean intraoperative blood loss was 1400 cc (300-4500cc) for a mean operative time of 260 minutes (165-450 min). There were two early infections at day 15 postop which resolved with antibiotics. Four patients developed resolutive sciatica (n = 2) or cruralgia (n = 2) during the following months. The short-term follow-up revealed eight non-unions (25%) with rupture of osteosynthesis material and loss of correction. The opening in the adjacent discs and the insufficient posterior closure explained this mechanical complication. CONCLUSION: Pedicle subtraction osteotomy is an original operative technique reserved for specialized centers. A well-experienced team is needed to control the risks involved. Sagittal balance should be one of the important goals for modern spinal surgery.

Adolescent↗