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Jugular foramen stenosis in external hydrocephalus in infants
Giuseppe Cinalli1, Giuliana Di Martino2, Carmela Russo3
1Department of Pediatric Neurosurgery, Santobono-Pausilipon Children's Hospital (AORN), Naples, Italy. giuseppe.cinalli@gmail.com.
Insights
Infants with external hydrocephalus (EH) have significantly smaller jugular foramina (JF) than controls. This jugular foramen stenosis is linked to increased venous obstruction, suggesting a key role in EH pathophysiology.
Area of Science:
- Neuroimaging
- Pediatric Neurology
- Vascular Anatomy
Background:
- External hydrocephalus (EH) is a condition affecting infants.
- The pathophysiology of EH is not fully understood.
- Jugular foramen (JF) stenosis is hypothesized to contribute to dural venous sinus alterations and increased venous outflow resistance in EH.
Purpose of the Study:
- To measure and compare the size of jugular foramina (JF) in infants with EH and a control group.
- To investigate the association between JF size, dural venous sinus alterations, and venous outflow resistance in EH.
- To explore the potential impact of positional plagiocephaly on JF dimensions and venous obstruction in infants with EH.
Main Methods:
- Phase-contrast magnetic resonance venous angiography (angio MRV PCA3D) was used to measure JF areas.
- JF areas were analyzed in 81 infants with EH and compared to 54 control infants.
- Venous obstruction grading score (VOGS) and cranial vault asymmetry index were assessed.
Main Results:
- Infants with EH exhibited significantly smaller JF areas compared to controls (mean JF area: 51.6 mm² vs. 57.0 mm²).
- Smaller JF areas in EH patients were significantly associated with higher VOGS on both sides.
- Positional plagiocephaly in EH patients was linked to decreased JF area and increased VOGS on the flattened side.
Conclusions:
- The mean size of the jugular foramen ostium is significantly smaller in infants with EH compared to controls.
- Jugular foramen stenosis is associated with increased venous obstruction, suggesting a direct impact on dural sinus lumen and venous outflow resistance.
- Positional plagiocephaly may further contribute to reduced JF area and elevated venous obstruction on the flattened side in infants with EH.
Objective:
To measure the size of jugular foramina in infants affected by external hydrocephalus (EH) and in a control group, to support the hypothesis that a jugular foramen (JF) stenosis may determine dural venous sinus alterations and increased venous outflow resistance as main pathophysiological factor.
Methods:
Minimum, maximum, and mean values of JF areas were measured in a series of phase-contrast magnetic resonance venous angiography (angio MRV PCA3D) performed on 81 infants affected by EH. Results were compared with a group of 54 controls.
Results:
Smaller JF area was significantly smaller in patients versus controls (43.1 ± 14.6 vs. 52.7 ± 17.8; p < 0.001) resulting in a significantly smaller mean JF areas in patients vs. controls (51.6 ± 15.8 vs. 57.0 ± 18.3; p = 0.043). In patients, smaller JF areas were significantly associated with higher venous obstruction grading score (VOGS) both on the right (p = 0.018) and on the left side (p = 0.005). Positional plagiocephaly (cranial vault asymmetry index > 3.5%) was more frequent among EH patients than controls (38/17) but the difference was not significant (p = 0.07). In the 38 plagiocephalic patients, JF area was smaller on the flattened side than the contralateral in a significant number of cases both in right (21/7) and left (9/1) plagiocephaly (p < 0.0005) as well as the mean area (48.2 + 16.4 mm2 vs. 57.5 + 20.7 mm2, p = 0.002) and VOGS was significantly higher on the plagiocephalic side than on the contralateral side (1.6 ± 1.1 vs. 1.1 ± 0.9, p = 0.019).
Conclusion:
In this series of infants affected by EH, the mean size of the ostium of both JF resulted significantly smaller than controls. JF stenosis was significantly associated with higher degrees of venous obstruction on both sides, suggesting a direct extrinsic effect of JF size on dural sinus lumen and possible consequent effect on venous outflow resistance. Positional plagiocephaly, when present, was associated with a decreased JF area and increased VOGS on the flattened side.
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