Table 4.
Causes and mechanisms of death among people with myelomeningocele in modern published series
| Paper | Data source | Country | Included patients born before 1972 | Number of deaths reported | MMC among dead patients | Shunted among dead patients | Median age at death (range, years) | Cause of death | Most common mechanism of death | ||||
| Neurological | Pulmonary | Urological (renal failure) | Other | Unknown | |||||||||
| McDonnell et al., 2000 | Single center | United Kingdom | Unknown | 18 | Unknown | Unknown | 28 (n/a) (adults only) | 22-39% * | 0-17% * | 33-44% * (22%) | 39-55% * | 0% | Infection, non-congenital cardiovascular, renal failure (22% each) |
| Bowman et al., 2001 | Single center | United States | No | 28 | 100% | Unknown (86% in 118 MMC patients) | 1 to 5 (range < 1->15) | > = 50% * | n/a | n/a | n/a | n/a | Hindbrain dysfunction (> = 46% *) |
| Jernigan et al., 2012 | Single center | United States | No | 11 | 100% | Unknown (92% in 106 MMC patients) | 24 (mean, SD 3) (adults only) | 18% | 18% | 0% (0%) | 64% | 0% | Unexplained death: unknown cause while asleep (55%) |
| Malakounides et al., 2013 | Single center | United Kingdom | Yes (> = 1970) | 7 | Unknown | Unknown | 14 (0-25) | 29-57% * | 0-29% * | 0-14% * (0%) | 43-71% * | 29% | Infection (57%) |
| Szymanski et al., 2015 | Single center | United States | No | 28 | 100% ** | 96% ** | 22 (9-38) | 25% | 18% | 14% (7%) | 39% | 4% | Infection (57%) |
| Borgstedt-Bakke et al., 2017 | National registry | Denmark | Yes (> = 1970) | 27 | 100% | Unknown | 1 (0-39) | 11% | 19% | 7% (0%) | 26% | 37% | Infection (48%) |
| Dicianno et al., 2018 | Single center | United States | Yes (∼half) | 36 | 100% | Unknown (73% hydrocephalus in 48 MMC/non-MMC patients) | 42 (mean, range 24-83) | 6-17% * | 8-25% * | 0-17% * (0%) | 3-19% * | 67% | Infection (17%) |
| North et al., 2018 | Single center | Canada | Yes (> = 1971) | 18 | 100% | Unknown (84% in all 101 MMC patients) | n/a (13 for entire cohort of 83 patients) | 22-28% * | 0-6% * | 6-11% * (6%) | 6-11% * | 61% | Infection (17-22%)* |
| Protzenko et al., 2019 | Single center | Brazil | No | 17 | 100% | Unknown (84% in all 231 MMC patients) | n/a (7 for entire cohort of 384 patients) | 35% | 0% | 29% (6-29%)* | 0% | 35% | Infection (29-53%)* |
| Peyronnet et al., 2020 | National registry of in-hospital deaths | France | Yes (∼half) | 138 | 95% | Unknown (39% in all 138 MMC/non-MMC patients) | 41 (IQR 25-52) | 14% | 17% | 17% (5%) | 38% | 14% | Infection (37%) |
| Current study | Multi-center | United States &Canada | No | 261 | 100% | 100% (shunted group) | <1 (IQR < 1-10) | 21% | 17% | 10% (3%) | 24% | 28% | Infection (34%) |
| 32 | 100% | 0% (unshunted group) | 20 (IQR 9-27) | 6% | 34% | 9% (6%) | 35% | 16% | Infection (38%) | ||||
*Exact number not calculable due to lack of data granularity. **Based on review of original data (not in published manuscript). Totals may not add up to 100% due to rounding. IQR: interquartile range; MMC: myelomeningocele; SD: standard deviation.