Inpatient Hospital Care of Children With Trisomy 13 and Trisomy 18 in the United States

Nelson, Katherine; Hexem, Kari R.; Feudtner, Chris

doi:10.1542/peds.2011-2139

Cited by 115 publications

(103 citation statements)

References 30 publications

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“…However, compared with trisomy 21, PS and ES are less common and have much worse longterm prognoses [8]. The spectrum of clinical abnormalities associated with these two syndromes is substantial and includes CHD, with atrial septal defects (ASD), patent ductus arteriosus (PDA), and ventricular septal defects (VSD) among the most common cardiac anomalies observed [11]. Even though cardiac complications are a major cause of death in PS and ES patients, a large retrospective study demonstrated that cardiac interventions are less common than procedures not related to cardiac pathology, including gastrointestinal, orthopedic, and facial interventions [11].…”

Section: Discussionmentioning

confidence: 99%

“…The spectrum of clinical abnormalities associated with these two syndromes is substantial and includes CHD, with atrial septal defects (ASD), patent ductus arteriosus (PDA), and ventricular septal defects (VSD) among the most common cardiac anomalies observed [11]. Even though cardiac complications are a major cause of death in PS and ES patients, a large retrospective study demonstrated that cardiac interventions are less common than procedures not related to cardiac pathology, including gastrointestinal, orthopedic, and facial interventions [11]. There is currently no consensus among medical professionals regarding the advisability of operating on CHD in patients following the prenatal diagnosis of PS and ES [1].…”

Section: Discussionmentioning

confidence: 99%

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A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

et al. 2015

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The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

et al. 2015

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“…Interestingly, no cardiac surgeries were reported. Similarly, only 3% of Nelson et al [2012] sample underwent cardiac procedures (n ¼ 1075). The most common surgeries were related to gastrointestinal conditions including placement of gastrostomy tube (n ¼ 13).…”

Section: Confirm and Disconfirm Previous Findingsmentioning

confidence: 99%

“…Additional reports of better outcome are available [Hsu and Hou, 2007;Kaneko et al, 2008;Maeda et al, 2011]. There are also recent United States data that point to the increased delivery of surgical interventions and other hospital-based care for infants, toddlers, and older children with trisomy 13 [Nelson et al, 2012]. Peroos et al [2012] provide an account of an 8-year-old with trisomy 13.…”

Section: Introductionmentioning

confidence: 99%

Nine children over the age of one year with full trisomy 13: A case series describing medical conditions

Bruns

Campbell

2014

American J of Med Genetics Pt A

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Trisomy 13 (Patau syndrome), identified by Patau and colleagues [1960; Lancet 1: 790-793] is the third most common autosomal condition. Population studies indicate less than one in 10 children reaches their first birthday. In the face of mixed findings and recommendations for treatment, additional research is needed to further determine what contributes to longevity and implications for treatment for presenting medical conditions. The purpose of the present study is to report on presenting medical conditions and the presence or absence of the specific conditions (age at survey completion). Data on nine survivors (seven female, two male) with trisomy 13 indicated mean gestational age of approximately 36 weeks, birth weight ranging from 1100 to 3290 g and mean length of 45.3 cm. Length of hospital stay after birth varied. The majority of infants presented with well-known physical characteristics. Medical conditions and their treatment varied at birth and at survey completion. Notably, several infants' cardiac anomalies resolved without surgical intervention. Surgeries were provided for a range of conditions including gastrostomy tube placement to address feeding issues and removal of intestinal blockage. There were no reports of holoprosencephaly. Implications and recommendations are provided.

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“…Nelson et al [40] state "although diagnoses of trisomy 13 and 18 are generally assumed to be fatal within days to weeks after birth, a small but significant subgroup of children with trisomy 13 and 18 are alive over the age of 1 year, and at least some of these children receive substantial inpatient hospital care" (p. 874) Yates et al [41] point to a shift in interventions to address cardiac anomalies based on parent request. Yet, the impact of these studies is minimized by empirical reports emphasizing early mortality [42].…”

Section: Offering a Positive Viewpoint And Associated Recommendationsmentioning

confidence: 99%

Erring on the Side of Life: Children with Rare Trisomy Conditions, Medical Interventions and Quality of Life

Bruns¹

2013

J Genet Disor Genet

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Inpatient Hospital Care of Children With Trisomy 13 and Trisomy 18 in the United States

Cited by 115 publications

References 30 publications

A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

Nine children over the age of one year with full trisomy 13: A case series describing medical conditions

Erring on the Side of Life: Children with Rare Trisomy Conditions, Medical Interventions and Quality of Life

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