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Web neck anomaly and its association with congenital heart disease
L D Berdahl1, K D Wenstrom, J W Hanson
1Department of Obstetrics and Gynecology, University of Oklahoma Health Sciences Center, Oklahoma City 73190, USA.
Insights
Web neck anomaly in infants is strongly linked to congenital heart defects (CHD), particularly flow-related issues. This association suggests a need for CHD screening in infants diagnosed with web neck.
Area of Science:
- Pediatrics
- Cardiology
- Genetics
Background:
- Jugular lymphatic obstruction can manifest as web neck anomaly.
- Congenital heart defects (CHD) are common birth anomalies.
- The relationship between web neck and CHD requires further investigation.
Purpose of the Study:
- To determine the incidence of CHD in infants with and without web neck.
- To explore the association between web neck, genetic syndromes, and specific heart defects.
- To understand the pathogenetic relationship between web neck and CHD.
Main Methods:
- Utilized the Iowa Birth Defects Registry for data analysis.
- Compared incidence of CHD in infants with and without web neck.
- Analyzed the prevalence of genetic syndromes and lymphatic obstruction features in infants with web neck.
Main Results:
- Sixty percent of infants with web neck had CHD, including hypoplastic left heart, coarctation, and atrial septal defects.
- Sixty-eight percent of infants with web neck had genetic syndromes (Down, Ullrich-Turner, Noonan).
- Infants with web neck and specific syndromes showed a higher likelihood of flow-related heart defects, with Ullrich-Turner syndrome and web neck having an 11-fold increased risk of coarctation.
Conclusions:
- Web neck anomaly is associated with both flow and non-flow-related heart defects.
- The findings suggest an independent pathogenetic relationship between web neck and CHD.
- Web neck or nuchal cystic hygroma findings warrant a prompt search for CHD.
Abstract:
To investigate the relationship between congenital heart disease and jugular lymphatic obstruction as manifested in web neck anomaly, we used the Iowa Birth Defects Registry to determine the incidence of congenital heart defects (CHD) in infants with and without web neck. Sixty percent of infants with web neck had CHD, with a high incidence of flow-related defects such as hypoplastic left heart, coarctation, and secundum atrial septal defect. Sixty-eight percent of infants with web neck had a genetic syndrome (37% Down syndrome, 13% Ullrich-Turner syndrome, and 5% Noonan syndrome), and 24% had dysmorphic features consistent with lymphatic obstruction sequence. When infants with Down, Ullrich-Turner, and Noonan syndrome and web neck were compared to infants with the same syndrome but without web neck, those with web neck were significantly more likely to have flow-related heart defects. Infants with Ullrich-Turner syndrome and web neck had an 11-fold higher incidence of coarctation, compared to those with a normal neck. Our data suggests web neck is associated with both flow and nonflow-related heart defects. This association implies a pathogenetic relationship and appears to be independent of causal factors. The finding of web neck or nuchal cystic hygroma on a prenatal ultrasound or newborn examination should prompt a search for CHD.