Search Results

You are looking at 1 - 10 of 21 items for

  • Author or Editor: Mark P. Johnson x
  • Refine by Access: all x
Clear All Modify Search
Restricted access

Ajith J. Thomas, Jack P. Rock, Christine C. Johnson, Linda Weiss, Gordon Jacobsen, and Mark L. Rosenblum

Object. It has been suggested that synchronous brain metastases (that is, those occurring within 2 months of primary cancer diagnosis) are associated with a shorter survival time compared with metachronous lesions (those occurring more than 2 months after primary cancer diagnosis). In this study the authors used data obtained from the National Cancer Institute's Surveillance, Epidemiology, and End Results program to determine the incidence of synchronous brain metastases and length of survival of patients in a defined population of southeastern Michigan residents.

Methods. Data obtained in 2682 patients with synchronous brain metastases treated between 1973 and 1995 were reviewed. Study criteria included patients in whom at least one brain metastasis was diagnosed within 2 months of the diagnosis of primary cancer and those with an unknown primary source. The incidence per 100,000 population increased fivefold, from 0.69 in 1973 to 3.83 in 1995. The most frequent site for the primary cancer was the lung (75.4%). The second largest group (10.7%) consisted of patients in whom the primary site was unknown. The median length of survival was 3.2 months. There was no significant difference in the median survival of patients with primary lung/bronchus and those with an unknown primary site (3.3 months and 3.2 months, respectively).

Conclusions. Patients who present with synchronous lesions have a poor prognosis, and the predominant cause of death, in more than 90% of cases, is related to systemic disease; however, despite poor median survival times, certain patients will experience prolonged survival.

Full access

Ajith J. Thomas, Jack P. Rock, Christine C. Johnson, Linda Weiss, Gordon Jacobsen, and Mark L. Rosenblum

Object

It has been suggested that synchronous brain metastases (that is, those occurring within 2 months of primary cancer diagnosis) are associated with a shorter survival time compared with metachronous lesions (those occurring greater than 2 months after primary cancer diagnosis). In this study the authors used data obtained from the National Cancer Institute's Surveillance, Epidemiology, and End Results Program to determine the incidence of synchronous brain metastases and length of survival of patients in a defined population of southeastern Michigan residents.

Methods

Data obtained in 2682 patients with synchronous brain metastases treated from 1973 to 1995 were reviewed. Study criteria included patients in whom at least one brain metastasis was diagnosed within 2 months of the diagnosis of primary cancer and those with an unknown primary source. The incidence per 100,000 increased fivefold, from 0.69 in 1973 to 3.83 in 1995. The most frequent site for the primary cancer was the lung (75.4%). The second largest group (10.7%) consisted of patients in whom the primary site was unknown. The median survival length was 3.3 months. There was no significant difference in the median survival in patients with primary lung/bronchus and those with an unknown primary site (3.2 months and 3.4 months, respectively).

Conclusions

Patients who present with synchronous lesions have a poor prognosis, and the predominant cause of death, in greater than 90% of cases, is related to systemic disease; however, despite poor median survival lengths, certain patients will experience prolonged survival.

Restricted access

Daniele Rigamonti, Burton P. Drayer, Peter C. Johnson, Mark N. Hadley, Joseph Zabramski, and Robert F. Spetzler

✓ The angiographic, computerized tomography (CT), and magnetic resonance imaging (MRI) findings were compared in 10 patients with a total of 16 pathologically verified cavernous angiomas. Only three lesions had abnormal vasculature in the form of venous pooling or a capillary blush. The CT scans were positive in seven patients and detected 14 lesions, while high-field strength (1.5 Tesla) MRI was positive in each case and demonstrated 27 distinct lesions. On T2-weighted MRI, the combination of a reticulated core of mixed signal intensity (SI) with a surrounding rim of decreased SI strongly suggests the diagnosis of a cavernous malformation. Smaller lesions appear as areas of decreased SI (black dots). The sensitivity of MRI is based on magnetic susceptibility and possibly diffusion effects related to field heterogeneity that is more conspicuous on high-field imaging and caused by the presence of excessive iron (hemosiderin).

Restricted access

Enrico Danzer, Linda M. Ernst, Natalie E. Rintoul, Mark P. Johnson, N. Scott Adzick, and Alan W. Flake

Object

The authors retrospectively investigated whether midgestational fetal myelomeningocele (fMMC) repair alters intrauterine meconium exposure.

Methods

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.

Results

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.

Conclusions

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.

Restricted access

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

Object

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.

Methods

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.

Results

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.

Conclusions

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.

Full access

Mario Teo, Jeremiah N. Johnson, Teresa E. Bell-Stephens, Michael P. Marks, Huy M. Do, Robert L. Dodd, Michael B. Bober, and Gary K. Steinberg

OBJECTIVE

Majewski osteodysplastic primordial dwarfism Type II (MOPD II) is a rare genetic disorder. Features of it include extremely small stature, severe microcephaly, and normal or near-normal intelligence. Previous studies have found that more than 50% of patients with MOPD II have intracranial vascular anomalies, but few successful surgical revascularization or aneurysm-clipping cases have been reported because of the diminutive arteries and narrow surgical corridors in these patients. Here, the authors report on a large series of patients with MOPD II who underwent surgery for an intracranial vascular anomaly.

METHODS

In conjunction with an approved prospective registry of patients with MOPD II, a prospectively collected institutional surgical database of children with MOPD II and intracranial vascular anomalies who underwent surgery was analyzed retrospectively to establish long-term outcomes.

RESULTS

Ten patients with MOPD II underwent surgery between 2005 and 2012; 5 patients had moyamoya disease (MMD), 2 had intracranial aneurysms, and 3 had both MMD and aneurysms. Patients presented with transient ischemic attack (TIA) (n = 2), ischemic stroke (n = 2), intraparenchymal hemorrhage from MMD (n = 1), and aneurysmal subarachnoid hemorrhage (n = 1), and 4 were diagnosed on screening. The mean age of the 8 patients with MMD, all of whom underwent extracranial-intracranial revascularization (14 indirect, 1 direct) was 9 years (range 1–17 years). The mean age of the 5 patients with aneurysms was 15.5 years (range 9–18 years). Two patients experienced postoperative complications (1 transient weakness after clipping, 1 femoral thrombosis that required surgical repair). During a mean follow-up of 5.9 years (range 3–10 years), 3 patients died (1 of subarachnoid hemorrhage, 1 of myocardial infarct, and 1 of respiratory failure), and 1 patient had continued TIAs. All of the surviving patients recovered to their neurological baseline.

CONCLUSIONS

Patients with MMD presented at a younger age than those in whom aneurysms were more prevalent. Microneurosurgery with either intracranial bypass or aneurysm clipping is extremely challenging but feasible at expert centers in patients with MOPD II, and good long-term outcomes are possible.

Restricted access

Eric M. Jackson, Daniel M. Schwartz, Anthony K. Sestokas, Deborah M. Zarnow, N. Scott Adzick, Mark P. Johnson, Gregory G. Heuer, and Leslie N. Sutton

Object

Fetal myelomeningocele closure has been shown to be advantageous in a number of areas. In this study, the authors report on neural function in patients who had previously undergone fetal myelomeningocele repair and returned to the authors' institution for further surgery that included intraoperative neurophysiological monitoring.

Methods

The authors retrospectively reviewed data obtained in 6 cases involving patients who underwent fetal myelomeningocele repair and later returned to their institution for spinal cord untethering. (In 4 of the 6 cases, the patients also underwent removal of a dermoid cyst [3 cases] or removal of an epidermoid cyst [1 case] during the untethering procedure.) Records and imaging studies were reviewed to identify the anatomical level of the myelomeningocele as well as the functional status of each patient. Stimulated electromyography (EMG) and transcranial motor evoked potential (tcMEP) recordings obtained during surgery were reviewed to assess the functional integrity of the nerve roots and spinal cord.

Results

During reexploration, all patients had reproducible signals at or below their anatomical level on stimulated EMG and tcMEP recordings. Corresponding to these findings, prior to tethering, all patients had antigravity muscle function below their anatomical level.

Conclusions

All 6 patients had lower-extremity function and neurophysiological monitoring recording signals at or below their anatomical level. These cases provide direct evidence of spinal cord and nerve root conductivity and functionality below the anatomical level of the myelomeningocele, further supporting that neurological status improves with fetal repair.

Free access

John S. Riley, Ryan M. Antiel, Alan W. Flake, Mark P. Johnson, Natalie E. Rintoul, John D. Lantos, Michael D. Traynor Jr., N. Scott Adzick, Chris Feudtner, and Gregory G. Heuer

OBJECTIVE

The Management of Myelomeningocele Study (MOMS) compared prenatal with postnatal surgery for myelomeningocele (MMC). The present study sought to determine how MOMS influenced the clinical recommendations of pediatric neurosurgeons, how surgeons’ risk tolerance affected their views, how their views compare to those of their colleagues in other specialties, and how their management of hydrocephalus compares to the guidelines used in the MOMS trial.

METHODS

A cross-sectional survey was sent to all 154 pediatric neurosurgeons in the American Society of Pediatric Neurosurgeons. The effect of surgeons’ risk tolerance on opinions and counseling of prenatal closure was determined by using ordered logistic regression.

RESULTS

Compared to postnatal closure, 71% of responding pediatric neurosurgeons viewed prenatal closure as either “very favorable” or “somewhat favorable,” and 51% reported being more likely to recommend prenatal surgery in light of MOMS. Compared to pediatric surgeons, neonatologists, and maternal-fetal medicine specialists, pediatric neurosurgeons viewed prenatal MMC repair less favorably (p < 0.001). Responders who believed the surgical risks were high were less likely to view prenatal surgery favorably and were also less likely to recommend prenatal surgery (p < 0.001). The management of hydrocephalus was variable, with 60% of responders using endoscopic third ventriculostomy in addition to ventriculoperitoneal shunts.

CONCLUSIONS

The majority of pediatric neurosurgeons have a favorable view of prenatal surgery for MMC following MOMS, although less so than in other specialties. The reported acceptability of surgical risks was strongly predictive of prenatal counseling. Variation in the management of hydrocephalus may impact outcomes following prenatal closure.

Restricted access

Mark D. Johnson, Uwe Stolz, Christopher P. Carroll, George L. Yang, Norberto Andaluz, Brandon Foreman, Natalie Kreitzer, Michael D. Goodman, and Laura B. Ngwenya

OBJECTIVE

The Surviving Penetrating Injury to the Brain (SPIN) score utilizes clinical variables to estimate in-hospital and 6-month mortality for patients with civilian cranial gunshot wounds (cGSWs) and demonstrated good discrimination (area under the receiver operating characteristic curve [AUC] 0.880) in an initial validation study. The goal of this study was to provide an external, independent validation of the SPIN score for in-hospital and 6-month mortality.

METHODS

To accomplish this, the authors retrospectively reviewed 6 years of data from their institutional trauma registry. Variables used to determine SPIN score were collected, including sex, transfer status, injury motive, pupillary reactivity, motor component of the Glasgow Coma Scale (mGCS), Injury Severity Score (ISS), and international normalized ratio (INR) at admission. Multivariable logistic regression analysis identified variables associated with mortality. The authors compared AUC between models by using a nonparametric test for equality.

RESULTS

Of the 108 patients who met the inclusion criteria, 101 had all SPIN score components available. The SPIN model had an AUC of 0.962. The AUC for continuous mGCS score alone (0.932) did not differ significantly from the AUC for the full SPIN model (p = 0.26). The AUC for continuous mGCS score (0.932) was significantly higher compared to categorical mGCS score (0.891, p = 0.005). Use of only mGCS score resulted in fewer exclusions due to missing data. No additional variable included in the predictive model alongside continuous mGCS score was a significant predictor of inpatient mortality, 6-month mortality, or increased model discrimination.

CONCLUSIONS

Given these findings, continuous 6-point mGCS score may be sufficient as a generalizable predictor of inpatient and 6-month mortality in patients with cGSW, demonstrating excellent discrimination and reduced bias due to missing data.

Free access

Noel Tulipan, John C. Wellons III, Elizabeth A. Thom, Nalin Gupta, Leslie N. Sutton, Pamela K. Burrows, Diana Farmer, William Walsh, Mark P. Johnson, Larry Rand, Susan Tolivaisa, Mary E. D’alton, and N. Scott Adzick

OBJECT

The Management of Myelomeningocele Study (MOMS) was a multicenter randomized trial comparing the safety and efficacy of prenatal and postnatal closure of myelomeningocele. The trial was stopped early because of the demonstrated efficacy of prenatal surgery, and outcomes on 158 of 183 pregnancies were reported. Here, the authors update the 1-year outcomes for the complete trial, analyze the primary and related outcomes, and evaluate whether specific prerandomization risk factors are associated with prenatal surgery benefit.

METHODS

The primary outcome was a composite of fetal loss or any of the following: infant death, CSF shunt placement, or meeting the prespecified criteria for shunt placement. Primary outcome, actual shunt placement, and shunt revision rates for prenatal versus postnatal repair were compared. The shunt criteria were reassessed to determine which were most concordant with practice, and a new composite outcome was created from the primary outcome by replacing the original criteria for CSF shunt placement with the revised criteria. The authors used logistic regression to estimate whether there were interactions between the type of surgery and known prenatal risk factors (lesion level, gestational age, degree of hindbrain herniation, and ventricle size) for shunt placement, and to determine which factors were associated with shunting among those infants who underwent prenatal surgery.

RESULTS

Ninety-one women were randomized to prenatal surgery and 92 to postnatal repair. The primary outcome occurred in 73% of infants in the prenatal surgery group and in 98% in the postnatal group (p < 0.0001). Actual rates of shunt placement were only 44% and 84% in the 2 groups, respectively (p < 0.0001). The authors revised the most commonly met criterion to require overt clinical signs of increased intracranial pressure, defined as split sutures, bulging fontanelle, or sunsetting eyes, in addition to increasing head circumference or hydrocephalus. Using these modified criteria, only 3 patients in each group met criteria but did not receive a shunt. For the revised composite outcome, there was a difference between the prenatal and postnatal surgery groups: 49.5% versus 87.0% (p < 0.0001). There was also a significant reduction in the number of children who had a shunt placed and then required a revision by 1 year of age in the prenatal group (15.4% vs 40.2%, relative risk 0.38 [95% CI 0.22–0.66]). In the prenatal surgery group, 20% of those with ventricle size < 10 mm at initial screening, 45.2% with ventricle size of 10 up to 15 mm, and 79.0% with ventricle size ≥ 15 mm received a shunt, whereas in the postnatal group, 79.4%, 86.0%, and 87.5%, respectively, received a shunt (p = 0.02). Lesion level and degree of hindbrain herniation appeared to have no effect on the eventual need for shunting (p = 0.19 and p = 0.13, respectively). Similar results were obtained for the revised outcome.

CONCLUSIONS

Larger ventricles at initial screening are associated with an increased need for shunting among those undergoing fetal surgery for myelomeningocele. During prenatal counseling, care should be exercised in recommending prenatal surgery when the ventricles are 15 mm or larger because prenatal surgery does not appear to improve outcome in this group. The revised criteria may be useful as guidelines for treating hydrocephalus in this group.