Sharon Duguay

Long-Term Survival of Individuals with Myelomeningocele

The objectives of this study were to extend survival analysis into adulthood for patients with myelomeningocele (MM) and to compare survival curves for patients born with varying defect severity before and after 1975. We have reviewed existing data for 904 patients with MM seen in a large multidisciplinary children’s clinic over 43 years. Before 1975, a major contributor to decreased survival is death during infancy. The presence of cerebral spinal fluid shunting is a major contributor to increased survival. After 1975, survival to adolescence is similar regardless of shunt status (p = 0.17). For all patients alive at age 16, a significant decrease in survival probability after age 34 years was found for individuals with shunted hydrocephalus compared to those without a shunt (p = 0.03). Although childhood survival for individuals born after 1975 is not related to shunt status, adults with MM and shunted hydrocephalus may be at risk for decreased longevity.

Acquisition of autonomy skills in adolescents with myelomeningocele

This study describes ages of acquisition (AOA) of typical adolescent autonomy skills in a regional cohort of individuals with myelomeningocele (MM), aged 12 to 18 years, with a mean age of 14 years 11 months (SD 2y 5mo). Prospectively collected data over 10 years were analyzed. Regression analysis, using Generalized Estimation Equation, provided 50th centile and 75th centile AOA for each skill. One hundred and fifty‐eight participants (90 males, 68 females) attended 378 annual patient visits. Patient contacts were equally distributed across age and physical severity groupings. Twenty‐four percent of participants had functional lesion levels at or above L2, and 38% between L3‐L5, 38% at S1 or below. Eighty‐four percent had shunted hydrocephalus, 24% were independent in ambulation, and 69% achieved toileting independence before adolescence. AOA for autonomy skills were delayed by 25 to 30% when compared with typically developing adolescents. Differences in cognitive ability explained the variance in median ages for skill acquisition (p=0.01) more than physical lesion level. Participants acquired community skills at a median age of 16 years 6 months. Twelve percent of 18‐year‐olds drove cars (9% with supervision, 3% independently), regardless of physical lesion severity. We conclude that adolescents with MM acquire the majority of autonomy skills 2 to 5 years later than their typically developing peers. This study establishes AOA timelines for autonomy skills in adolescents with MM.

Contributory factors to postoperative spinal fusion complications for children with myelomeningocele.

Study Design. This is a retrospective review of all children with myelomeningocele (MMC) who were undergoing surgery for scoliosis at our institution. Objective. Our aim was to investigate possible correlations between poor nutritional indexes and/or positive urinary cultures before surgery to perioperative infection risk. Summary of Background Data. Patients who have scoliosis secondary to MMC have been shown to have a high rate of infectious complications. Many children with MMC have been found to have asymptomatic urinary tact infections due to their neurogenic bladder. There is little literature on risk factors for and ways to prevent postoperative infections for these patients. Methods. We reviewed a comprehensive database for the outcome for spinal fusion surgeries of 59 patients with MMC treated at our institution over the past 45 years. Perioperative infections were correlated to preoperative nutritional status (measured by patient hematocrit [Hct]) and presence of active infection using Fisher Exact Probability Test. Results. A total of 84 different spinal fusion operations for 59 patients were recorded. Patients presenting with poor preoperative nutrition (Hct ≤33) and positive preoperative urinary cultures demonstrated the highest risk for perioperative wound infection (P = 0.001). Patients with preoperative poor nutrition without a positive urinary culture as well as those with adequate nutritional status but a preoperative positive urinary culture showed an increased risk of wound infection respectively (P = 0.03) and (P = 0.002). Conclusion. Positive urine cultures obtained before spinal surgery for children with MMC significantly increased the risk for perioperative wound (P = 0.002). Urinary tract infections can be easily detected and treated. We believe urinary culture, protein deficiencies, or Hct ≤33, should be treated and corrected before surgery.

Haemophilus influenzae and Streptococcus pneumoniae infections in children with cerebrospinal fluid shunts.

Objective: This paper reviews the frequency of central nervous system infections due to Haemophilus influenzae and Streptococcus pneumoniae associated with cerebrospinal fluid (CSF) shunts in pediatric patients. The need for immunizations in this patient population is also evaluated. Patients: All patients with cerebrospinal fluid shunts except those with brain tumors seen in our clinics. Methods: We reviewed data in three computer databases, kept prospectively recording details of CSF shunt procedures and CSF shunt-related infections. Results: 1,226 patients underwent 3,889 shunt placements between 1957 and 2007. Twelve patients had 14 episodes of Haemophilus or pneumococcal infections. Conclusions: Children with CSF shunts are at high risk for infection with H. influenzae and S. pneumoniae. Routine immunizations during infancy in addition to the 23-valent polysaccharide pneumococcal vaccine should be highly and actively encouraged by health care providers caring for children with CSF shunts. Additional expanded-coverage vaccines should be utilized if and when they become available.

Outcome for fetuses with a diagnosis of myelomeningocele, Hydrocephalus and Brain Anomalies

Materials and methods We will report the outcome for all 241 patients referred over the past 25 years by tertiary level Obstetricians to our Neurodevelopmental team to either explain the options for treatment of their affected foetus or to provide prognosis for their determination to terminate the pregnancy or carry to term. They include all patients in the cohort and include the following diagnoses: Major brain malformations – 22, Hydrocephalus – 45, Syndromes – 3 and Myelomeningocele (MM) – 171. All but one case with MM were delivered by caesarean section birth before the rupture of amniotic membranes. Analysis was by X2 or Fishers Exact.

Comprehensive, economical management of cerebrospinal fluid shunt infections – a twenty-one year experience

Materials and methods We describe analysis of data stored in two computer databases for 2,193 shunt related operations involving 786 patients. Infections were defined as positive cultures of the CSF or the shunt. Antibiotic levels in the CSF and Minimam Bacteriocidal (MBC) levels are expressed in ug/ml. Operative procedures were complete shunt removal, placement of an extra ventricular drain, antibiotics until the CSF was sterile, then replacement of the shunt ((CSR+EVD+abx+R), externalization of the distal limb, abx, then R (Ex+abx+R) and antibiotics alone. Analysis was by transfer to Excel files, Fisher Exact and Student ttests. A cure was defined as 16 months without recurrent infection or a new infection with another organism.

Efficacy of conventional valves compared to programmable valves in managing children & adolescents with hydrocephalus: a 450 valve retrospective study

Materials and methods The authors collected computerized data for all patients subjected to a CSF shunt insertion or revision except those with brain tumours from the Patient Data Management System and the Computer Information System of the Seattle Childrens Hospital from 1 January 2000 through 31 December 2008. Data collected included the patients diagnosis, birth date, details of the operation insertion and components of revisions, valve type and location, type and timing of prophylactic antibiotic administration, time of day of the operation, duration of operation, presence of a trainee, incidence of shunt complications and white blood cell (WBC) and red blood cell counts (RBC) in the CSF obtained at the time of operation. Patients were followed for a minimum of one year post-operatively and statistical analysis was done using Vassar STATS® and MedCalc® which included Chi Square, Kaplan Meier Survival Curves and multi-variant analysis. Results A total of 1,278 operations were analyzed that incorporated 450 valve (233 PVS & 217 NPVS) implantations (166) or revisions (284) for 306 patients. Patients with a PVS that were adjusted (≥ 6 mo after implantation) demonstrated no significant difference to rate of non-valve revision (X2 p = 0.63), infection(X2 p = 0.99), or overall valve survival (KM p = 0.24) compared to patients with NPVS. Complications leading to valve replacement of the initial implantation occurred in 42% of NPVS and 45% of PVS(X2 p = 0.89). By differentiating patients by their preoperative WBC (poly ≥ 3 or mono ≥ 50) and/or RBC ≥ 30 in the CSF (as indications for inflammation), revisions of NPVS were significantly greater in valve survival rate than the PVS (p = 0.002). No significant differences existed between the two valve systems for reason of revision, location, age of patient, time of day, duration of the operation type of antibiotic used or time of administration or presence of trainee.

Outcome of surgery for scoliosis in patients with myelomeningocele

Background Since the 1950s, patients with severe, progressive scoliosis have undergone spinal instrumentation to reduce the curvature and to provide more stability to the spinal fusion. Patients that have scoliosis secondary to myelomeningocele (MMC), have been shown to have an increased number of complications compared to patients with scoliosis due to other conditions. There is little literature on risk factors for and ways to prevent post operative complications for these patients. Many children have been found to have asymptomatic urinary tact infections due to their neurogenic bladder.

Prenatal counseling for myelomeningocele: prognoses, decisions and outcomes

Background In 2007, we reported the outcomes of 238 patients counseled by pediatricians following ultrasound (US) diagnosis of a nervous system defect (Avellino et al.). Here, we compare a subset of these patients diagnosed with myelomeningocele (MM) to a group of patients not counselled by a pediatrician. We have gathered data regarding prenatal prognoses and outcomes for these two groups.

Conservative management of cerebrospinal fluid shunt infections

Background Brown et al. [1] published a favourable experience with treatment of Staphylococcus coagulase negative (SCoN) caused cerebrospinal fluid shunt (CSF) infections without surgery using Rifampin (Rif). We have presented our management of CSF shunt infections, in part indicating surgery is not needed for some cases. This paper is to further describe our experience over the last 45 years.