Avoidance of emergency surgery in newborn infants with trisomy 18

Bos, A. Van Den; Broers, Cjm; Hazebroek, Fwj; Hemel, JO Van; Tibboel, Dick; Swaay, E. Wesby-van; Molenaar, J C

doi:10.1016/0020-7292(92)90716-v

Cited by 21 publications

(25 citation statements)

References 0 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Many care providers label the diagnoses of T13 and T18 as "lethal" and advocate providing palliative care only. 14,15 More recently however, a more active approach has been called for by some. [16][17][18] This approach includes engaging the parents in the choice of care options, avoiding language that assumes outcome and that presumes a family' s view of quality of life for their children, and informing parents of the possibility of long-term survival.…”

Section: Discussionmentioning

confidence: 99%

Mortality and Morbidity of VLBW Infants With Trisomy 13 or Trisomy 18

Boghossian

Hansen²,

Bell

et al. 2014

Pediatrics

View full text Add to dashboard Cite

WHAT'S KNOWN ON THIS SUBJECT: Infants with trisomy 13 (T13) or trisomy 18 (T18) are known to have poor survival. Little is known about how very low birth weight (VLBW) impacts survival and morbidities among infants with T13 or T18. WHAT THIS STUDY ADDS:We examined the risks of mortality and neonatal morbidities for VLBW infants with T13 or T18 compared with VLBW infants with trisomy 21 and VLBW infants without birth defects in a 16-year cohort from the Neonatal Research Network. abstract OBJECTIVE: Little is known about how very low birth weight (VLBW) affects survival and morbidities among infants with trisomy 13 (T13) or trisomy 18 (T18). We examined the care plans for VLBW infants with T13 or T18 and compared their risks of mortality and neonatal morbidities with VLBW infants with trisomy 21 and VLBW infants without birth defects. METHODS:Infants with birth weight 401 to 1500 g born or cared for at a participating center of the Eunice Kennedy Shriver National Institute of Child Health and Human Development Neonatal Research Network during the period 1994-2009 were studied. Poisson regression models were used to examine risk of death and neonatal morbidities among infants with T13 or T18. RESULTS:Of 52 262 VLBW infants, 38 (0.07%) had T13 and 128 (0.24%) had T18. Intensity of care in the delivery room varied depending on whether the trisomy was diagnosed before or after birth. The plan for subsequent care for the majority of the infants was to withdraw care or to provide comfort care. Eleven percent of infants with T13 and 9% of infants with T18 survived to hospital discharge. Survivors with T13 or T18 had significantly increased risk of patent ductus arteriosus and respiratory distress syndrome compared with infants without birth defects. No infant with T13 or T18 developed necrotizing enterocolitis. CONCLUSIONS:In this cohort of liveborn VLBW infants with T13 or T18, the timing of trisomy diagnosis affected the plan for care, survival was poor, and death usually occurred early. Pediatrics 2014;133:226-235

show abstract

Section: Discussionmentioning

confidence: 99%

Mortality and Morbidity of VLBW Infants With Trisomy 13 or Trisomy 18

Boghossian

Hansen²,

Bell

et al. 2014

Pediatrics

View full text Add to dashboard Cite

show abstract

“…Withdrawal of intensive treatment such as cesarean, resuscitation, respiratory support, and surgical procedures has been recommended because of the lethality and severe mental retardation [David and Glew, 1980;Schneider et al, 1981;Carter et al, 1985;Rochelson et al, 1986;Goldstein and Nielsen, 1988;Bos et al, 1992;Embleton et al, 1996].…”

Section: Introductionmentioning

confidence: 99%

Neonatal management of trisomy 18: Clinical details of 24 patients receiving intensive treatment

Kosho

Nakamura

Kawame

et al. 2006

American J of Med Genetics Pt A

175

200

View full text Add to dashboard Cite

Management of neonates with trisomy 18 is controversial, supposedly due to the prognosis and the lack of precise clinical information concerning efficacy of treatment. To delineate the natural history of trisomy 18 managed under intensive treatment, we reviewed detailed clinical data of 24 patients with full trisomy 18 admitted to the neonatal intensive care unit of Nagano Children's Hospital, providing intensive treatment to those with trisomy 18, from 1994 to 2003. Cesarean, resuscitation by intubation, and surgical operations were performed on 16 (67%), 15 (63%), and 10 (42%) of the patients, respectively. Mechanical ventilation was required by 21 (88%), and 6 (29%) of them were extubated. Survival rate at age 1 week, 1 month, and 1 year was 88%, 83%, and 25%, respectively. Median survival time was 152.5 days. Respiration was not stabilized in two patients with left diaphragmatic eventration and hypoplasia accompanied by lung hypoplasia, even with maximal ventilation. The common underlying factors associated with death were congenital heart defects and heart failure (96%), followed by pulmonary hypertension (78%). The common final modes of death were sudden cardiac or cardiopulmonary arrest (26%) and possible progressive pulmonary hypertension-related events (26%). These data of improved survival, through neonatal intensive treatment, are helpful for clinicians to offer the best information on treatment options to families of patients with trisomy 18. ß 2006 Wiley-Liss, Inc.

show abstract

“…Double aneuploidy, structural aberration and mosaicism involving chromosome 18 have also been recorded. 18 The birth prevalence rate of Edwards' syndrome varies considerably, from 1:3000 to 1:11,000 live births. 2,3,7,[19][20][21][22][23][24] In the present study, the T18 average birth prevalence rate was 8.95 per 10,000 live births, which is significantly higher than the previous rate reported from Kuwait during 1984-1985 (1.1 per 10,000) and in 1986 (4.61 per 10,000).…”

Section: Discussionmentioning

confidence: 99%

Clustering of Trisomy 18 in Kuwait: Genetic Predisposition or Environmental?

et al. 1999

View full text Add to dashboard Cite

Background: This study describes 59 newborns with regular trisomy 18 (Edwards' syndrome, T18) who were ascertained clinically and cytogenetically at the Kuwait Medical Genetic Centre from 1994 to 1997, out of 118 T18 cases identified from 1980 to 1997. Materials and Methods: T18 cases were ascertained clinically and cytogenetically shortly after birth. In addition to assessing the T18 birth prevalence rate and confidence limits during the years 1994-1997, we investigated the possible etiological factors by a case-control study with normal healthy newborns. Studied factors included gender, parental age, birth order, abortion, clinical variables (presentation, amniotic fluid and mode of delivery), and survival. Results: The average T18 birth prevalence rate during the period was 8.95 per 10,000 live births (95% confidence limits 6.66-11.23). The T18 cases were mostly females, with a male:female ratio of 1:2.1, and the majority (53%) died before the second week of life. Maternal age above 30 years was found to be a significant factor for T18. Conclusion: This high T18 birth prevalence rate suggests clustering of T18 in the highly inbred population of Kuwait. Such clustering may indicate a possible environmental, and to a lesser extent, genetic predisposition to aneuploidy nondisjunction.

show abstract

Avoidance of emergency surgery in newborn infants with trisomy 18

Cited by 21 publications

References 0 publications

Mortality and Morbidity of VLBW Infants With Trisomy 13 or Trisomy 18

Mortality and Morbidity of VLBW Infants With Trisomy 13 or Trisomy 18

Neonatal management of trisomy 18: Clinical details of 24 patients receiving intensive treatment

Clustering of Trisomy 18 in Kuwait: Genetic Predisposition or Environmental?

Contact Info

Product

Resources

About