Spinal lipomas in children

Spinal cord lipomas are a common cause of cord tethering that can lead to progressive neurological defects. The role of prophylactic surgery for spinal lipomas has recently been questioned. Between 1985 and 1999, 59 children underwent a total of 69 surgical procedures at the Birmingham Children's Hospital in Birmingham, UK. The spinal lipomas were classified into: 18 terminal, 17 transitional, 6 dorsal and 18 filum lipomas - including 12 who had a typical thickened filum terminale. At the first operation, 19 patients (32%) were asymptomatic, and 40 patients (68%) presented with symptoms. Surgical indications in the asymptomatic group included the presence of a dermal sinus tract or syrinx. Prophylactic surgery was undertaken in selected cases. The mean total follow-up for the group since the first surgical procedure was 61.8 months (range: 7.0-203.0 months). In the asymptomatic group, 26% of the patients had late neurological deterioration. Of the 14 patients with asymptomatic conus lipomas, 3 (21%) developed sphincter dysfunction and motor problems at long-term follow-up. In the symptomatic group, 68% improved, 20% remained unchanged, and 12% had late neurological deterioration. None of the 18 patients with symptomatic filum lipoma deteriorated postoperatively. However, 39% had bladder dysfunction, 54% had neuro-orthopaedic deformity, and only 15% returned to overall normal function at latest follow-up. Of the 27 patients with symptomatic conus lipomas, 67% improved, 15% remained stable, and 18% had late neurological deterioration. However, 74% had bladder dysfunction, 67% had neuro-orthopaedic deformity, and 45% had motor problems at long-term follow-up. Spinal lipomas can cause progressive neurological deficits irrespective of spinal untethering surgery. This study demonstrates that filum and conus lipomas have similar clinical presentation, but differ in their outcome following surgery. Filum lipomas are 'benign', for which surgery is safe and effective. Conus lipomas are more difficult to manage. When asymptomatic, our results suggest that prophylactic surgery does provide some protection from future neurological deterioration. When symptomatic, conus lipoma surgery is effective in stopping further deterioration. Improvement in neurological function can occur, but few patients return to normal overall function, and pre-existing sphincter dysfunction is not significantly altered by surgery.     

Spinal lipomas in children--surgical management and long-term follow-up

Since its introduction, MR imaging has been easy to perform on all children with lumbosacral cutaneous stigmata, and has enabled the phenomenal refinement of spinal pathology. We investigated the overall outcomes of children with spinal lipomas at the Osaka Medical Center and Research Institute for Maternal and Child Health in Osaka, Japan. Between 1991 and 2003, 76 children with a tethered cord underwent a total of 90 surgical procedures at our institutes. Of this cohort, 67 cases had spinal lipomas. The mean age of patients at first operation for asymptomatic lipoma was 22.4 months (range: 1 month to 16 years, trim mean: 10.1 months, mode: 5 months), except 4 cases. The mean total follow-up for the cohort since the first surgical procedure was 7.2 years (trim mean: 7.9 years). Since the introduction of MR imaging, the reoperation rate for symptoms or signs in our series was 16.4% (11 cases). Regarding the 13 subsequent reoperations (2 patients had 2 operations), 3 patients were reoperated on due to multiplication of the lipomas after untethering, 3 due to urologic symptoms and 5 due to orthopedic signs. There were 2 cases who also had to undergo reoperation early due to CSF leakage. Filum and conus lipomas have similar tethering pathologies, but differ in the outcome following surgery. Filum lipomas are benign, and therefore surgery is safe and effective. Conus lipomas are more difficult to manage.     

Psoriasis in childhood]

Psoriasis is a common chronic disease in childhood of yet unclear etiology. Thirty per cent of psoriatic patients experience onset of their disease before 15 years of age. Psoriasis is exceptionally congenital but may present in infancy as a napkin dermatitis. There are specific pediatric clinical forms, but at the beginning the eruption is usually discrete, and the diagnosis can be difficult. Pustular, erythrodermic and arthropathic forms are rare. Treatment must weigh the advantages and disadvantages of the possible therapies and be kept to the minimum compatible with asymptomatic control.     

Spinal bone tumors in children]

The occurrence of back pain in children and adolescents varies from 30 to 51% in the literature. Bone tumors can be responsible for back pain. This paper presents the more common spinal bone tumors in children and adolescents, and specifies their etiology, their natural history, and their treatment as well.     

Berger's disease in childhood]

Berger's disease or IgA nephropathy (NIgA) is the most common form of glomerulonephritis in the world. In children macroscopic haematuria is the first sign in about 80% of the patients. Renal failure appears in 20% of cases after twenty years of follow-up. The most important prognosis indicators are a nephrotic syndrome at the onset, a proteinuria > 1 g/24 hours, diffuse tubulo-interstitial lesions and extracapillary proliferation with crescents in more than 50% of the glomeruli. The pathogenic mechanisms are just emerging and involve a disrupted process of the systemic tolerance to mucosal antigen with abnormal mucosal gamma delta T cell repertoire, abnormally glycosylated IgA1 molecules and a down-regulation of Fc alpha receptors on blood cells. After IgA deposition, the mechanisms of mesangial cell damage and activation involve vascular factors as endothelin/nitric oxide system, cytokines and growth factors such as interleukine-6, platelet derived growth factor and transforming growth factor beta. There is no curative treatment but steroids are useful in diffuse proliferative extracapillary forms, when histological activity score is high with a short delay between diagnosis and treatment, or for moderately severe NIgA with normal renal function.     

Plasma aldosterone in childhood]

According to a radioimmunological method modified in our laboratory we determined plasma aldosterone levels under standardized conditions in children of different age from 2 weeks to 14 years, in women undergoing uncomplicated vaginal delivery, and in the cord blood of the corresponding newborn babies. Furthermore, the effect of different stimulating factors (ACTH, salt restriction, severe vomiting, fever) in children was studied. In mothers and cord blood we found 68.6 +/- 39.3 ng/100 ml and 75.5 +/- 37.4 ng/100 ml, respectively. In the first two years of life there is a decrease in plasma aldosterone levels to the relatively constant concentration at the age of 2 to 14 years (7.3 +/- 4.7 ng/100 ml). The age groups 2 weeks to 1 year, 1 to 2 years and 2 to 14 years all show significant differences. A correlation of age (first two years of life) and plasma aldosterone level could be shown (correlation coefficient -0.50 according to Pearson). The stimulation results are comparable with literature data concerning adults. The mechanism of stimulation by vomiting and fever is discussed.     

Hodgkin's disease in childhood]

Only in these latest years has been possible to consider Hodgkin disease (HD) as a neoplastic syndrome, thank of immunohistochemistry and cytogenetic techniques which have confirmed the monoclonal origin of typical cellular marker of disease: the Reed-Sternberg cell (R-S cell). Interesting associations have been observed between children suffering from HD and the positivity of EBV antigen above all in the socio-economically developed countries. The histopathologic classification of HD is divided in four sub-types, with different incidence in the pediatric age: the nodular sclerosis and the mixed cellularity are more rap-presented than the lymphocyte predominance and mostly the lymphocyte depletion. Histopathologic classification is essential for the prognosis and a correct therapeutic approach to disease. The management of HD is based on chemotherapy and radiotherapy associated; the results of treatment are more and more encouraging with a global survival over 95%. Sequelae of treatment are reduced in modern therapeutic trials: in particularly injury to somatic growth, cardiopulmonary system, gonadal and thyroid functionality is reduced by using low dose and involved fields for the early stage patients. The most important sequela in children treated for HD is the risk to developed a second malignant neoplasm; in particularly acute nonlymphocytic leukemia and non Hodgkin's lymphoma. In patients treated with radiotherapy alone increase the risk to develop solid tumors like sarcomas and carcinomas, which can appears several years after diagnosis.     

Allergic rhinitis in childhood]

Prevalence of allergic rhinitis has increased in the past years. The determination of the aeroallergens involved is important. Children with persistent rhinitis must be evaluated for asthma. Early treatment of allergic rhinitis may avoid asthma onset. The management of allergic rhinitis must include allergen avoidance, pharmacological treatment and immunotherapy. Sublingual swallow immunotherapy is a safe and effective method of treatment.     

Adnexal torsion in childhood]

Five cases of torsion of normal uterine adnexae are reported in children aged 9 to 13 years. From this series, the authors review the clinical and ultrasonographic features of the torsion of normal uterine adnexa. Ultrasonography should be performed in emergency as the conservation of the ovary depends on the precocity of diagnosis and treatment.     

Rotavirus diarrhea in childhood]

Electron microscopy of 350 diarrhoeal faeces revealed Rotavirus-particles in 145 cases. All patients with Rotavirus-infections showed symptoms of acute gastroenteritis lasting 1 to 8 days. Additionally to diarrhoea most cases presented fever and vomiting. None of the patients showed toxicosis.     

Mucociliary clearance in childhood]

Aim of the study: A new, nearly monodisperse human serum albumin particle produced by air-pressure-nebulization was inhaled by children and lung-transplant patients. Method: After inhalation of the particles obtained with an air-pressure nebulizer, the initial deposition pattern showed a marked tracheobronchial deposition which could be reproducibly obtained without a special breathing technique, the alveolar deposition being not higher than 10%. With the use of 99mTc, the radiation exposure is limited to a level which is low enough for children, but images can be taken up to 24 hrs later. Further parameters for in vivo characterization of the mucociliary function are the 24 h retention pattern and the velocity of particle motion in the trachea. Results: Within the first 45 minutes, the global clearance rate was 51% in healthy children, which is rather high in comparison with the literature, most likely due to size of particles and the selection of patients with a mean age of 10.7 years. In ciliary dysfunction, the initial clearance rate was 16% and 46% within 24 h. Conclusions: With simplification of the preparation, application, and examination technique, this method is to be used in children, so that a wider use can be anticipated. The normally fast initial elimination of particles allows quick differentiation of normal and impaired ciliary function.     

Hydrocarbon poisoning in childhood]

The authors report the experience of a pediatric clinic on hydrocarbon poisoning. Among the reported cases was that of a girl showing a serious pneumonitis.