Intramuscular myxomas (IMMs) are rare benign tumors of mesenchymal origin that are most often located in large skeletal muscles, particularly of the thigh. They have also been reported within the paraspinal musculature and should be considered in the differential diagnosis of a paraspinal mass. These lesions can cause neurological symptoms due to mass effect. This is a report of a 52-year-old man with multiple paraspinal tumors that exhibited concerning growth on serial imaging studies. To the authors’ knowledge, this represents the first report of a patient with multiple paraspinal myxomas. CT-guided biopsy followed by surgical excision of the largest mass was performed. Histopathological analysis was consistent with an IMM. Patients with multiple IMMs often have an underlying genetic syndrome such as Mazabraud syndrome, McCune-Albright syndrome, or Carney complex. Despite variable growth patterns and associations with genetic syndromes, multiple IMMs have had no documented cases of malignant transformation into myxoid sarcoma; therefore, surgical excision should be considered based on a patient’s individual symptoms.
Joseph S. Domino, Shane Weindel, and Sarah Woodrow
Aqueel Pabaney, Shawn L. Hervey-Jumper, Joseph Domino, Cormac O. Maher, and Lynda J. S. Yang
Neuropathic pain is rare in children, and few reports provide adequate guidelines for treatment. The authors describe the successful treatment of tardy neuropathic pain via macrovascular decompression in a 15-year-old boy who presented with progressive pain 11 years following trauma to the upper extremity that had required surgical repair of the brachial artery. Examination revealed mild chronic median and ulnar motor neuropathy as well as recent progressive lancinating pain and a Tinel sign at the prior scar. A soft tissue mass in the neurovascular bundle at the site of previous injury was noted on MRI. Surgical exploration demonstrated an altered anatomical relationship of the previously repaired brachial artery and the median nerve, resulting in pulsatile compression of the median nerve by the brachial artery. Neurolysis and decompression of the median nerve with physical separation from the brachial artery resulted in immediate pain relief.
This is the first report of macrovascular decompression of a major peripheral nerve with complete symptom resolution. Noninvasive imaging together with a thorough history and physical examination can support identification of this potential etiology of peripheral neuralgic pain. Recognition and treatment of this uncommon problem may yield improved outcomes for children with neuropathic pain.
Joseph Domino, Connie McGovern, Kate W. C. Chang, Noelle E. Carlozzi, and Lynda J. S. Yang
Perinatal disorders are prone to malpractice litigation. Neonatal brachial plexus palsy (NBPP) results from stretching the nerves in the perinatal period and may lead to paresis or paralysis and sensory loss in the affected arm. Little is known about the key factors associated with malpractice litigation by families of patients with NBPP and whether these factors reflect the practice environment or are inherent to the condition. In this study, the authors documented the percentage of families of NBPP patients at a specialty center that had filed a malpractice suit and described the key factors associated with that pursuit of legal action.
The families/caregivers of 51 patients with NBPP who had presented to the University of Michigan Interdisciplinary Brachial Plexus Program participated in this study. A qualitative research design was applied using both a questionnaire to examine psychosocial factors and a dynamic tool to measure health outcomes from the patient perspective via parent proxy (Patient-Reported Outcomes Measurement Information System [PROMIS] assessment instruments). Statistical analysis included the Fisher exact test, chi-square test, and Student t-test. The study protocol was approved by the University of Michigan institutional review board.
Forty-seven percent of the families pursued malpractice litigation. In comparing patient families that had pursued legal action with those that had not, significant differences were revealed in the perception that the sustained birth injury was unnecessary (p = 0.002), the information received in the perinatal period was inadequate (p = 0.003), family concerns were ignored in the perinatal period (p = 0.005), and family concerns were not adequately addressed (p < 0.001). Sixty-six percent of the families received external advice to pursue legal action. The PROMIS survey revealed significant group differences in depressive symptoms (p = 0.008), fatigue (p = 0.02), pain (p = 0.01), and anger (p = 0.004). In contrast, the extent of NBPP was not associated with malpractice litigation (p = 0.18). Age, sex, and race were not significantly different between litigation and nonlitigation groups.
Physician-controllable factors, such as communication in the perinatal period, are associated with malpractice litigation in NBPP. The perceived level of global disability may affect the pursuit of malpractice litigation, whereas the isolated extent of nerve root involvement and/or upper extremity dysfunction are not significant factors in pursuing litigation. Identifying and ameliorating these factors within the practice environment may decrease the animosity between families and health care providers and improve overall outcome for patients with NBPP.
Paige Lundy, Emmanuel Vlastos, Joseph Domino, Grace S. Mitchell, Kristin Fickenscher, and Paul Grabb
Prenatal closure of myelomeningocele is associated with a reduced rate of hydrocephalus treatment. This need for hydrocephalus treatment is positively correlated with fetal ventricular width. When ventricular width is 15 mm or greater, the benefits of prenatal closure, as a method to decrease hydrocephalus treatment, are reduced. Thus, fetal ventricular size is an important factor when counseling families who are considering intrauterine surgery with mitigation of hydrocephalus as the primary goal. This study sought to determine whether imaging modality (ultrasound [US] vs MRI) and interobserver variability were factors in any ventricular size disparity seen on imaging studies.
The imaging studies of 15 consecutive fetuses who underwent prenatal myelomeningocele repair at Children’s Mercy Fetal Health Center, Kansas City, Missouri, were reviewed. All fetuses were imaged with US and fetal MRI; on average (range), procedures were performed 3.8 (0–20) days apart. Three comparisons were performed to analyze interobserver and intermodality variability in ventricular width measurements: 1) retrospective comparison of dictated ventricular widths measured with MRI and US by pediatric radiologists (PRs) and maternal-fetal medicine specialists (MFMs), respectively; 2) blinded measurements obtained with US by PRs versus initial US-based measurements by MFMs, and blinded measurements obtained with MRI by PRs versus initial MRI-based measurements by PRs; and 3) blinded measurements obtained with MRI by PRs versus those obtained with US.
Retrospective comparison showed that measurements with MRI by PRs were on average 2.06 mm (95% CI 1.43–2.69, p < 0.001) larger than measurements with US by MFMs. Blinded measurements with US by PRs were on average larger than dictated measurements obtained with US by MFMs, but by only 0.6 mm (95% CI 0.31–0.84, p < 0.001). When PRs measured ventricular size in a blinded fashion with both US and MRI, the mean width determined with MRI was significantly larger by 2.0 mm (95% CI 1.26–2.67, p < 0.0001).
The ventricular width of these fetuses was larger when measured with MRI than US by an amount that could impact recommendations for fetal surgery. Every center involved in counseling families about the risks and benefits of fetal intervention for spina bifida needs to be aware of these possible imaging-based disparities.