Oakeshott P, Hunt GM, Poulton A, Reid F. (2010) Expectation of life and unexpected death in open spina bifida: a 40-year complete, non-selective, longitudinal cohort study Dev Med Child Neurol. 2010 Aug;52(8):749-53.
AIM:
The aim of our study was to investigate survival and causes of death in a complete cohort of open spina bifida at the mean age of 40 years.
METHOD:
We conducted a community-based, prospective study of 117 consecutive infants (50 males, 67 females) with open spina bifida whose backs were closed non-selectively within 48 hours of birth between 1963 and 1971 at Addenbrooke's Hospital, Cambridge, UK. Of those who survived to age 1 year, 89% (82 out of 92) had a cerebrospinal fluid shunt. In 2007, all survivors were surveyed by postal questionnaire backed up by telephone interview. Details of deaths were obtained from the Office for National Statistics, medical records, and autopsy reports, and Kaplan-Meier survival curves were constructed.
RESULTS:
One in three of the cohort (40/117) died before the age of 5 years. A further 26% (31/117) died during the next 35 years, over 10 times the national average. Half the deaths (16/31) after the age of 5 were sudden and unexpected. All occurred in the community and were followed by a coroner's autopsy. The most frequent causes of these unexpected deaths were epilepsy, pulmonary embolus, acute hydrocephalus, and acute renal sepsis. The prognosis for survival was strikingly poor in those with the most extensive neurological deficit. Only 17% (7/42) of those born with a high sensory level (above T11) survived to the mean age of 40 years, compared with 61% (23/38) of those with a low sensory level (below L3; p=0.001).
INTERPRETATION:
Doctors and care planners need to be aware that, contrary to previous suggestions, there is continuing high mortality throughout adult life in individuals with open spina bifida, and many deaths are unexpected.
Showing posts with label Morbidity. Show all posts
Showing posts with label Morbidity. Show all posts
Monday, January 9, 2012
Monday, October 29, 2007
Do grip and pinch strength predict neurologic complications in children with spina bifida and hydrocephalus
Liptak GS, Fried R, Baltus-Hebert E, Eyer Tierney S, Fucile S, Doremus TL. Do grip and pinch strength predict neurologic complications in children with spina bifida and hydrocephalus? Pediatric Neurosurgery. 2006;42(4):208-13.
BACKGROUND: Neurological complications occur commonly in children with meningomyelocele and can cause significant morbidity and mortality. An earlier study suggested that acute changes in grip and pinch strength could be used to identify individuals at increased risk for developing a neurological complication.
OBJECTIVE: To evaluate the use of grip and pinch measurements to screen for neurological problems in children with spina bifida and hydrocephalus.
DESIGN: A prospective evaluation of screening tests.
PATIENTS: 92 children, born since 1976, who had meningomyelocele and hydrocephalus and were treated at the University of Rochester Medical Center.
METHODS: Grip and pinch strength were measured between July, 1991, and June, 2003. RESULTS: Mean grip and pinch strengths were similar to those found in previous studies of children with meningomyelocele; 58 neurological events occurred in 39 (40%) individuals. These included 31 episodes of ventricular shunt failure and 22 symptomatic tethered cord occurrences. Specificity, sensitivity and likelihood ratios were calculated in multiple ways using different criteria for loss of grip or pinch strength and for interval to neurological event. Sensitivities were low (<0.35) and the highest positive likelihood ratio found, using fall in either lateral pinch with 6 months to neurological event, was 2.3.
CONCLUSIONS: Despite previous recommendations, grip and pinch measurements were not helpful when used as a routine screening test for neurological dysfunction for children with meningomyelocele and hydrocephalus.
PMID: 16714860
BACKGROUND: Neurological complications occur commonly in children with meningomyelocele and can cause significant morbidity and mortality. An earlier study suggested that acute changes in grip and pinch strength could be used to identify individuals at increased risk for developing a neurological complication.
OBJECTIVE: To evaluate the use of grip and pinch measurements to screen for neurological problems in children with spina bifida and hydrocephalus.
DESIGN: A prospective evaluation of screening tests.
PATIENTS: 92 children, born since 1976, who had meningomyelocele and hydrocephalus and were treated at the University of Rochester Medical Center.
METHODS: Grip and pinch strength were measured between July, 1991, and June, 2003. RESULTS: Mean grip and pinch strengths were similar to those found in previous studies of children with meningomyelocele; 58 neurological events occurred in 39 (40%) individuals. These included 31 episodes of ventricular shunt failure and 22 symptomatic tethered cord occurrences. Specificity, sensitivity and likelihood ratios were calculated in multiple ways using different criteria for loss of grip or pinch strength and for interval to neurological event. Sensitivities were low (<0.35) and the highest positive likelihood ratio found, using fall in either lateral pinch with 6 months to neurological event, was 2.3.
CONCLUSIONS: Despite previous recommendations, grip and pinch measurements were not helpful when used as a routine screening test for neurological dysfunction for children with meningomyelocele and hydrocephalus.
PMID: 16714860
Spina bifida outcome: a 25-year prospective
Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year prospective. Pediatric Neurosurgery. 2001 Mar;34(3):114-20.
BACKGROUND: Open spina bifida is the most complex congenital abnormality compatible with long-term survival. This report outlines the 20- to 25-year outcome for our original cohort of patients with a myelomeningocele treated in a nonselective, prospective manner.
METHODS: Of the initial 118 children, 71 patients were available for our most recent review. Nineteen patients have been lost to follow-up and 28 patients have died. Data were collected on: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy and/or gastrostomy tube.
RESULTS: Mortality (24%) continues to climb into young adulthood. Eighty-six percent of the cohort have cerebrospinal fluid diversion, with 95% having undergone at least one shunt revision. Thirty-two percent have undergone a tethered cord release, with 97% having an improvement or stabilization in their preoperative symptoms. Forty-nine percent have scoliosis, with 43% eventually requiring a spinal fusion. Sixteen patients (23%) have had at least one seizure. Eighty-five percent are attending or have graduated from high school and/or college. More than 80% of young adults have social bladder continence. Approximately 1/3 of patients are allergic to latex, with 6 patients having experienced a life-threatening reaction.
CONCLUSION: At least 75% of children born with a myelomeningocele can be expected to reach their early adult years. Late deterioration is common. One of the greatest challenges in medicine today is establishing a network of care for these adults with spina bifida. Copyright 2001 S. Karger AG, Basel
PMID: 11359098
BACKGROUND: Open spina bifida is the most complex congenital abnormality compatible with long-term survival. This report outlines the 20- to 25-year outcome for our original cohort of patients with a myelomeningocele treated in a nonselective, prospective manner.
METHODS: Of the initial 118 children, 71 patients were available for our most recent review. Nineteen patients have been lost to follow-up and 28 patients have died. Data were collected on: motor level, shunt status, education/employment, seizure history, mobility, bladder/bowel continence, tethered cord, scoliosis, latex allergy, posterior cervical decompression, tracheostomy and/or gastrostomy tube.
RESULTS: Mortality (24%) continues to climb into young adulthood. Eighty-six percent of the cohort have cerebrospinal fluid diversion, with 95% having undergone at least one shunt revision. Thirty-two percent have undergone a tethered cord release, with 97% having an improvement or stabilization in their preoperative symptoms. Forty-nine percent have scoliosis, with 43% eventually requiring a spinal fusion. Sixteen patients (23%) have had at least one seizure. Eighty-five percent are attending or have graduated from high school and/or college. More than 80% of young adults have social bladder continence. Approximately 1/3 of patients are allergic to latex, with 6 patients having experienced a life-threatening reaction.
CONCLUSION: At least 75% of children born with a myelomeningocele can be expected to reach their early adult years. Late deterioration is common. One of the greatest challenges in medicine today is establishing a network of care for these adults with spina bifida. Copyright 2001 S. Karger AG, Basel
PMID: 11359098
Tuesday, July 24, 2007
Long-term outcome of hydrocephalus management in myelomeningoceles.
Tuli S, Drake J, Lamberti-Pasculli M. Long-term outcome of hydrocephalus management in myelomeningoceles. Child's Nervous System. 2003 Jun;19(5-6):286-91. Epub 2003 May 23.
BACKGROUND: The cerebrospinal fluid (CSF) shunt remains an important ongoing management problem in myelomeningocele (MMC) patients. We reviewed the long-term shunt treatment outcome in a prospectively followed group of MMC patients from a single institution.
METHOD: Patients prospectively entered into a hydrocephalus database with a diagnosis of MMC from the years 1987 to 1996 were selected. All data was verified from the medical records and additional details about the shunt surgery were collected. The outcome of shunt failure was categorized as shunt obstruction, shunt infection, presence of loculated ventricles, overdrainage, and other. All deaths were recorded and causation identified. Univariate analysis for shunt failure risk factors was accomplished using Log rank statistics. Multivariable analysis was performed for each repeated failure level using a conditional Cox regression model.
RESULTS: One hundred and twenty (64%) out of 189 MMC patients experienced a first shunt failure with a median time of 303 days; 29 (24%) of the failures were due to shunt infection (the procedure infection rate being 15%). Sixty-one patients experienced a second shunt failure, 38 a third and 36 had four or more. Multivariable analysis of risk factors failed to demonstrate any clear risk factors for either first or subsequent shunt failure. Fifteen patients died, 13 from either shunt or Chiari 1 complications.
CONCLUSION: Shunt complications remain an important cause of morbidity and mortality in MMC patients, particularly shunt infection.
PMID: 12764629
BACKGROUND: The cerebrospinal fluid (CSF) shunt remains an important ongoing management problem in myelomeningocele (MMC) patients. We reviewed the long-term shunt treatment outcome in a prospectively followed group of MMC patients from a single institution.
METHOD: Patients prospectively entered into a hydrocephalus database with a diagnosis of MMC from the years 1987 to 1996 were selected. All data was verified from the medical records and additional details about the shunt surgery were collected. The outcome of shunt failure was categorized as shunt obstruction, shunt infection, presence of loculated ventricles, overdrainage, and other. All deaths were recorded and causation identified. Univariate analysis for shunt failure risk factors was accomplished using Log rank statistics. Multivariable analysis was performed for each repeated failure level using a conditional Cox regression model.
RESULTS: One hundred and twenty (64%) out of 189 MMC patients experienced a first shunt failure with a median time of 303 days; 29 (24%) of the failures were due to shunt infection (the procedure infection rate being 15%). Sixty-one patients experienced a second shunt failure, 38 a third and 36 had four or more. Multivariable analysis of risk factors failed to demonstrate any clear risk factors for either first or subsequent shunt failure. Fifteen patients died, 13 from either shunt or Chiari 1 complications.
CONCLUSION: Shunt complications remain an important cause of morbidity and mortality in MMC patients, particularly shunt infection.
PMID: 12764629
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