Saturday, November 24, 2007

Tethered cord syndrome in children: a review

Bui CJ, Tubbs RS, Oakes WJ. Tethered cord syndrome in children: a review. Neurosurgical Focus. 2007;23(2):1-9.

The treatment of a patient with symptoms of a tethered spinal cord and in whom a fatty infiltrated terminal filum is found is controversial. The authors review their experience and the literature regarding this aspect of occult spinal dysraphism. From experience, transection of a fatty terminal filum in patients with symptoms related to excessive caudal cord tension is a minor procedure that generally yields good results. The most problematic issue in the literature is what patients and symptoms are best suited to surgical treatment.

PMID: 17961017

Interpreting changes in the epidemiology of anencephaly and spina bifida following folic acid fortification of the U.S. grain supply

Besser LM, Williams LJ, Cragan JD. Interpreting changes in the epidemiology of anencephaly and spina bifida following folic acid fortification of the U.S. grain supply in the setting of long-term trends, Atlanta, Georgia, 1968-2003. Birth Defects Research. Part A: Clinical Molecular Teratology . 2007 Nov;79(11):730-6.

BACKGROUND:: The prevalence of anencephaly (AN) and spina bifida (SB) was declining long before fortification of enriched grains in the U.S. with folic acid. We examined whether changes in these defects surrounding fortification could be distinguished from preexisting trends.

METHODS:: We used data from the Metropolitan Atlanta Congenital Defects Program to identify three ascertainment periods: Period 1 (1968-1981), prenatal diagnoses rarely made; Period 2 (1981-1993), prenatal diagnoses made but not ascertained; Period 3 (1994-2003), prenatal diagnoses ascertained. We compared the annual percent change (APC) in AN and SB for each period using Poisson regression, then compared prevalences during each period for categories of pregnancy outcome, sex, race, gravidity, and maternal age.

RESULTS:: The prevalence of AN (N = 434) and SB (N = 663) declined during 1968-2003. The APCs in Periods 1, 2, and 3, respectively, were -6.9%, -2.9%, and -6.8% for AN, and -7.1%, -7.0%, and -6.2% for SB; 95% confidence intervals around the APCs for Periods 2 and 3 overlapped for both defects. Prevalence ratios (PRs) for females relative to males decreased for AN (2.3 in Period 1; 1.2 in Period 3); PRs for whites relative to blacks or African Americans decreased for both AN (2.7 in Period 1; 1.2 in Period 3) and SB (2.5 in Period 1; 1.1 in Period 3).

CONCLUSIONS:: Our analysis suggests that changes in AN and SB surrounding folic acid fortification (Period 3) could be part of preexisting trends. This must be considered when evaluating prevention efforts. Birth Defects Research (Part A) 79:730-736, 2007. (c) 2007 Wiley-Liss, Inc.

PMID: 17990332

Management of Infection After Instrumented Posterior Spine Fusion in Pediatric Scoliosis

Ho C, Skaggs DL, Weiss JM, Tolo VT. Management of Infection After Instrumented Posterior Spine Fusion in Pediatric Scoliosis. Spine. 2007 Nov 15;32(24):2739-2744.

STUDY DESIGN.: Case series retrospective review.

OBJECTIVE.: To identify what factors predict successful eradication of infection after I&D of an infected posterior spinal fusion with instrumentation.

SUMMARY OF BACKGROUND DATA.: The treatment of infection of instrumented spine fusions in children has few clear guidelines in the literature. METHODS.: The medical records of patients who required a surgical irrigation and debridement (I&D) for infection after posterior spinal fusion and instrumentation for scoliosis from 1995 to 2002 were retrospectively reviewed.

RESULTS.: Fifty-three patients were identified with the following underlying diagnoses: 21 patients (40%) idiopathic scoliosis, 10 patients (23%) cerebral palsy, 3 patients (6%) spina bifida, 1 patient (2%) congenital scoliosis, and 17 patients (32%) other. There were 31 patients (58%) with surgery <6 months from initial fusion, and 22 (42%) patients >6 months. Of the 43 patients with implant retained at the time of the first I&D, 20 patients required a second I&D (47%). Of the 10 patients with complete implant removal, 2 patients required a second I&D (20%). Coagulase-negative Staphylococcus was the most prevalent organism, growing in 25 (47%) of the cultures. Of patients with idiopathic scoliosis, 8 of 21 (38%) required a second I&D; of the patients with other diagnoses, 14 of 32 (44%) required a second I&D, which was not a significant difference (P > 0.05).

CONCLUSION.: To the best of our knowledge, this is the largest reported series of spinal implant infections. When children with an infection after posterior spinal fusion with instrumentation undergo irrigation and debridement, there is a nearly 50% chance that the infection will remain if all spinal implants are not removed. As nearly 50% of the infections were caused by coagulase-negative Staphylococcus, we recommend that prophylactic antibiotic coverage for this organism is used at the time of the initial spinal fusion.

PMID: 18007254

Urinary bladder adenocarcinoma arising in a spina bifida patient

Bitar M, Mandel E, Kirschenbaum AM, Unger PD. Urinary bladder adenocarcinoma arising in a spina bifida patient. Annals of Diagnostic Pathology. 2007 Dec;11(6):453-6. Epub 2007 Jul 24.

Urinary bladder adenocarcinomas are rare malignancies accounting for approximately 2.5% of all urothelial neoplasms. Intestinal metaplasia of the urothelium indicates the presence of intestinal-type goblet cells and was generally observed to coexist with or to precede the diagnosis of bladder adenocarcinomas. Controversy continues of whether intestinal metaplasia is an acquired precancerous lesion, secondary to different insults to the urothelium, or a concomitant lesion in glandular carcinogenesis. Patients with neurogenic bladders are particularly at risk for developing bladder cancer, mostly squamous cell carcinoma and rarely adenocarcinoma. In these patients, chronic irritation of the urothelium as well as long-term indwelling urinary catheters were the most significant risk factors. Spina bifida is a congenital developmental abnormality that may result in neurogenic bladder. There is only one previously reported case of urothelial carcinoma with associated squamous metaplasia of the bladder occurring in a spina bifida patient. We report the first case of bladder adenocarcinoma associated with intestinal metaplasia occurring in a spina bifida occulta patient. The patient had a complicated clinical course and suffered recurrent urinary tract infections, renal calculi, and urinary incontinence and was managed with intermittent as well as indwelling catheterization.

PMID: 18022132

Wednesday, November 7, 2007

Allergic reactions to latex in myelodysplasia: a review of the literature

Gunther KP, Nelitz M, Parsch K, Puhl W. Allergic reactions to latex in myelodysplasia: a review of the literature. Journal of Pediatric Orthopedics B. 2000 Jun;9(3):180-4. Review.

Current research has identified clinically relevant allergens in natural latex. Children with myelodysplasia are especially considered to be at risk for the development of immunoglobulin E-mediated hypersensitivity, which can lead to life-threatening intraoperative anaphylaxis. A careful medical history is mandatory to identify patients who might be predisposed to anaphylactic reactions. Preventive measures involve primarily the avoidance of latex contact at home and in hospitals for all patients with myelodysplasia. The effectiveness of additional pharmacologic prophylaxis has to be determined further.

PMID: 10904904

Latex sensitisation and allergy in children with myelomeningocele

Rendeli C, Nucera E, Ausili E, Tabacco F, Roncallo C, Pollastrini E, Scorzoni M, Schiavino D, Caldarelli M, Pietrini D, Patriarca G. Latex sensitisation and allergy in children with myelomeningocele.
Child's Nervous System. 2006 Jan;22(1):28-32. Epub 2005 Feb 10.

INTRODUCTION: Children with spina bifida (SB) have a high degree of exposure to latex products as a consequence of repeated surgical procedures, implantation of latex-containing materials and catheterisation. The consequence is a higher incidence of latex allergic reactions.

OBJECTIVE: The aim of this study is to evaluate the prevalence of latex sensitisation and allergy in a population of children with myelomeningocele (MMC) and to assess the role of associated risk factors.

RESULTS: Forty-eight percent of the patients (29 out of 60) showed a latex sensitisation with specific IgE >0.7 kU/l while 15% (9 out of 60) were allergic to latex (specific IgE >0.7 kU/l and clinical manifestations). The principal factor correlated with allergy to latex was specific serum IgE to latex (radioallergosorbent test [RAST]) values (p<0.01). Other factors were total serum IgE (paper radioimmunosorbent test [PRIST]) values, number of surgical procedures and familiarity with allergy.

CONCLUSION: These results underline the importance of prophylactic measures to avoid the exposure, not only in the sanitary environment, through the institution of latex-safe routes, but also in daily life, to prevent potentially serious allergic reactions.

PMID: 15703967

Prevalence of latex allergy in spina bifida: genetic and environmental risk factors

Ausili E, Tabacco F, Focarelli B, Nucera E, Patriarca G, Rendeli C. Prevalence of latex allergy in spina bifida: genetic and environmental risk factors.
European Review for Medical Pharmacological Sciences. 2007 May-Jun;11(3):149-53.

AIM OF STUDY: To evaluate the prevalence of latex allergy in a population of children with spina bifida (SB) and to assess the role of early exposure to latex products and others risk factors.

INTRODUCTION: SB is related with an higher incidence of latex allergic reactions. These patients received repeated surgical procedures, implant of latex-containing materials and catheterization.

MATERIALS AND METHODS: Eighty consecutive subjects affected with SB besides answering a questionnaire, underwent a skin-prick test (SPT) to latex and the determination of the specific serum IgE (RAST CAP) to latex. 40% (32/80) of the patients showed a latex sensitization with specific IgE > 0.7 kU/I but only twelve of the 32 sensitized patients (40%) suffered from clinical reactions to latex (urticaria, conjunctivitis, angioedema, rhinitis, bronchial asthma). Number of surgical procedures, but particularly early exposure to latex and familiarity for allergy are correlated with latex allergy (p < 0.01).

CONCLUSION: Latex allergy in SB children is multifactorial situation related with a disease-associated propensity for latex sensitization, early exposure and number of surgical procedures. Prophylactic measures to avoid the exposure, not only in the sanitary environment, through the institution of latex-safe routes and every day, prevent potentially serious allergic reactions.

PMID: 17970230