Enrico Danzer, Linda M. Ernst, Natalie E. Rintoul, Mark P. Johnson, N. Scott Adzick, and Alan W. Flake
The authors retrospectively investigated whether midgestational fetal myelomeningocele (fMMC) repair alters intrauterine meconium exposure.
Prior to the National Institutes of Health Management of Myelomeningocele Study, 54 fetuses underwent fMMC repair at the authors' institution. Forty-six fMMC sacs were available for pathological examination and 53 MMC sacs from postnatally repaired MMCs (pMMCs) were available for comparison. The presence and distribution of meconium were blindly evaluated using a grading system defined as follows: absent (no meconium present), mild (< 10 meconium-positive histiocytes [MPHs]/hpf), moderate (10–25 MPHs/hpf), and severe (> 25 MPHs/hpf). Hall's bile stain was used to confirm meconium and Prussian blue and Fontana Masson stains to exclude hemosiderin and melanin, respectively.
Compared to pMMCs (79%), meconium histiocytosis was less prevalent in fMMC sacs (57%; p = 0.017). Meconium staining was completely absent in 43% of the fMMC sacs. Mild meconium histiocytosis was found in 35% fMMC and 61% pMMC sacs (p = 0.035). There was no statistical difference between groups with moderate and severe meconium histiocytosis.
Meconium passage in MMCs can occur early in fetal life. Fetal MMC repair may reduce the duration of meconium exposure, thereby potentially limiting the toxic injury to the vulnerable neural elements.
Enrico Danzer, N. Scott Adzick, Natalie E. Rintoul, Deborah M. Zarnow, Erin S. Schwartz, Jeanne Melchionni, Linda M. Ernst, Alan W. Flake, Leslie N. Sutton, and Mark P. Johnson
The goal in this study was to evaluate the incidence and clinical implications of the development of cutaneously derived intradural inclusion cysts (ICs) following fetal myelomeningocele (fMMC) closure.
Retrospective databases and responses to a parental questionnaire were reviewed to determine the incidence, clinical presentation, and outcomes of fMMCs in children in whom ICs developed at follow-up.
Prior to the National Institutes of Health (NIH)-sponsored Management of Myelomeningocele Study (MOMS), 54 patients underwent fMMC closure at the authors' institution. Sixteen (30%) presented with symptomatic tethered cord syndrome (TCS) at a median age of 27 months (range 4–93 months). Ten (63%) of the 16 (19% of the total) developed TCS in association with an intradural IC. In 9 (90%) of 10 patients, the IC was seen on preoperative MR imaging, and in 1 it was found during surgery. Four additional children (7% of the total) with evidence of an IC on surveillance MR imaging are currently asymptomatic at 94, 84, 60, and 60 months of age, respectively. All but 1 (an L-3 level lesion) IC developed in infants with L-4 and L-5 defects. After cyst removal, 6 children are asymptomatic at a median follow-up of 36 months (range 12–63 months). Following IC removal, 4 children lost normal bladder function and now require clean intermittent catheterization, and 1 lost normal leg function and now requires a walking aid for ambulation. Histologically, 8 lesions were dermoid, 1 was an epidermoid, and 1 was a mixed dermoid-epidermoid IC. Three patients developed another IC and required its removal at 24, 39, and 51 months, respectively. One required another tethered cord release within 57 months after IC removal.
Cutaneously derived intradural ICs can develop following fMMC surgery. Deterioration of bladder function, risk of recurrence, and loss of lower-extremity function appear to be the most important long-term complications of IC in children with fMMCs. The ongoing NIH-sponsored MOMS may help determine whether children with fMMC are at increased risk of IC development compared with children treated with postnatal MMC closure. Parents seeking fMMC closure should be informed about the possibility of IC formation and the potential clinical consequences.