Patrick C. Hettinger

Double stellate tongue reduction: a new method of treatment for macroglossia in patients with Beckwith-wiedemann syndrome.

Background:Although multiple methods of tongue reduction have been described, recent literature suggests that the central reductions may be more favorable in patients with Beckwith-Wiedemann syndrome (BWS). In this case series, we review our experience with macroglossia associated with BWS, and we offer a new technique of central tongue reduction. Methods:Between 1993 and 2007, a retrospective chart review was conducted to include all patients with a diagnosis of BWS who have undergone stellate or double stellate tongue reduction at the Childrens Hospital of Wisconsin. Results:A total of 7 patients met all inclusion criteria. All patients had good tongue mobility at 1-year follow-up. One patient required speech therapy for persistent articulation errors postoperatively. A total of 2 patients required secondary procedures for recurrent macroglossia. There were no complaints of abnormal taste or sensation. Conclusions:The stellate and double stellate tongue reductions provide effective treatment in macroglossia associated with BWS.

Cortical plasticity induced by different degrees of peripheral nerve injuries: a rat functional magnetic resonance imaging study under 9.4 Tesla

Background Major peripheral nerve injuries not only result in local deficits but may also cause distal atrophy of target muscles or permanent loss of sensation. Likewise, these injuries have been shown to instigate long-lasting central cortical reorganization. Methods Cortical plasticity changes induced after various types of major peripheral nerve injury using an electrical stimulation technique to the rat upper extremity and functional magnetic resonance imaging (fMRI) were examined. Studies were completed out immediately after injury (acute stage) and at two weeks (subacute stage) to evaluate time affect on plasticity. Results After right-side median nerve transection, cortical representation of activation of the right-side ulnar nerve expanded intra-hemispherically into the cortical region that had been occupied by the median nerve representation After unilateral transection of both median and ulnar nerves, cortical representation of activation of the radial nerve on the same side of the body also demonstrated intra-hemispheric expansion. However, simultaneous electrical stimulation of the contralateral uninjured median and ulnar nerves resulted in a representation that had expanded both intra- and inter-hemispherically into the cortical region previously occupied by the two transected nerve representations. Conclusions After major peripheral nerve injury, an adjacent nerve, with similar function to the injured nerve, may become significantly over-activated in the cortex when stimulated. This results in intra-hemispheric cortical expansion as the only component of cortical plasticity. When all nerves responsible for a certain function are injured, the same nerves on the contralateral side of the body are affected and become significantly over-activated during a task. Both intra- and inter-hemispheric cortical expansion exist, while the latter dominates cortical plasticity.

Long-term vascular access ports as a means of sedative administration in a rodent fMRI survival model.

The purpose of this study is to develop a rodent functional magnetic resonance imaging (fMRI) survival model with the use of heparin-coated vascular access devices. Such a model would ease the administration of sedative agents, reduce the number of animals required in survival experiments and eliminate animal-to-animal variability seen in previous designs. Seven male Sprague-Dawley rats underwent surgical placement of an MRI-compatible vascular access port, followed by implantable electrode placement on the right median nerve. Functional MRI during nerve stimulation and resting-state functional connectivity MRI (fcMRI) were performed at times 0, 2, 4, 8 and 12 weeks postoperatively using a 9.4T scanner. Anesthesia was maintained using intravenous dexmedetomidine and reversed using atipamezole. There were no fatalities or infectious complications during this study. All vascular access ports remained patent. Blood oxygen level dependent (BOLD) activation by electrical stimulation of the median nerve using implanted electrodes was seen within the forelimb sensory region (S1FL) for all animals at all time points. The number of activated voxels decreased at time points 4 and 8 weeks, returning to a normal level at 12 weeks, which is attributed to scar tissue formation and resolution around the embedded electrode. The applications of this experiment extend far beyond the scope of peripheral nerve experimentation. These vascular access ports can be applied to any survival MRI study requiring repeated medication administration, intravenous contrast, or blood sampling.

Le Fort III distraction using rotation advancement of the midface in patients with cleft lip and palate.

Background: Patients with cleft lip and palate demonstrate a spectrum of maxillary growth deficiencies. The purpose of this study was to review the authors’ experience in the treatment of midface hypoplasia in nonsyndromic cleft lip–cleft palate patients using rotation advancement of the midface with Le Fort III distraction. Methods: A retrospective chart review was conducted to include all patients with nonsyndromic cleft lip and/or cleft palate who underwent Le Fort III rotation advancement of the midface from 1999 to 2011. Along with standard outcome measures, Amira imaging software was used to perform surface analysis on the last five consecutive patients in this series. Results: Forty-three consecutive patients met inclusion criteria. Diagnoses included unilateral complete cleft lip and palate (n = 25) and bilateral complete cleft lip and palate (n = 18). Average distraction distance measured 10.1 mm at the level of the zygoma (range, 6 to 15 mm). Preoperative and 6-month postoperative sella, nasion, A point angles measured 76.3 and 81.8 degrees; whereas sella, nasion, B point angles measured 79.9 and 78.7 degrees, respectively. Preoperative and 6-month postoperative overjet measured −5.4 and 3.2 mm, whereas overbite measured 1.9 and 1.1 mm, respectively. Six patients (13 percent) developed pseudorelapse, with five patients going on to have subsequent Le Fort I advancement at an average of 7 years after distraction (range, 2 to 11 years). Conclusions: Le Fort III rotation advancement of the midface addresses severe midface deficiencies in select patients. Whereas older techniques target occlusal correction alone, extending osteotomies to the Le Fort III level allows occlusal correction along with improvements in malar and nasal projection required to achieve facial harmony in this group of patients. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Early evaluation of nerve regeneration after nerve injury and repair using functional connectivity MRI.

Resting state functional connectivity magnetic resonance imaging studies in rat brain show brain reorganization caused by nerve injury and repair. In this study, distinguishable differences were found in healthy, nerve transection without repair (R−) and nerve transection with repair (R+) groups in the subacute stage (2 weeks after initial injury). Only forepaw on the healthy side was used to determine seed voxel regions in this study. Disturbance of neuronal network in the primary sensory region of cortex occurs within two hours after initial injury, and the network pattern was restored in R+ group in subacute stage, while the disturbed pattern remained in R− group. These are the central findings of the study. This technique provides a novel way of detecting and monitoring the effectiveness of peripheral nerve injury treatment in the early stage and potentially offers a tool for clinicians to avoid poor clinical outcomes.

C7 nerve root sensory distribution in peripheral nerves: a bold functional magnetic resonance imaging investigation at 9.4 T.

Introduction: In this study we used a rat model to elucidate the linear make‐up of each major nerve of the upper limb by the C7 root through sensory stimulation and functional magnetic resonance imaging (fMRI). Methods: The C7 nerve root and major nerves of the right forelimb were stimulated electrically. Blood oxygen level–dependent functional magnetic resonance imaging (BOLD fMRI) was performed concurrently. Voxel overlap within the primary sensory cortex was calculated. Results: C7 comprised sensation in <1% in the musculocutaneous nerve, 6% in the ulnar nerve, 16% in the radial nerve, and 19% in the median nerve (P < 0.005 for each). The overlap was always <25% for each major nerve. Conclusions: This study helps explain why C7 is a suitable donor for brachial plexus injury treatment and why there is only a transient sensory deficit after transfer. Muscle Nerve 49: 40–46, 2014

Extramammary Paget's disease: a novel approach to treatment using a modification of peripheral Mohs micrographic surgery.

Extramammary Pagets disease is a rare intraepithelial adenocarcinoma typified histologically by the presence of Paget cells. Treatment has historically been surgical, with wide excision being the standard of care. However, due to clinically indeterminate margins and diffuse spread, local recurrence rates remain high. Mohs micrographic surgery has been proposed to improve the rate of local recurrence. Application of Mohs technique to treat extramammary Pagets disease can be difficult because of the large size of these lesions. Reported excisions either involved lengthy procedures or peripheral Mohs modification. The peripheral technique does not evaluate the depth of the central lesion, yet prognosis and lymph node involvement are directly related to the degree of vertical invasion. In this study, we discuss our experience with extramammary Pagets disease, along with a novel approach to treatment by using a modification of peripheral Mohs micrographic surgery that incorporates histologic analysis of the central specimens depth.

Lipomas of the Brachial Plexus: A Case Series and Review of the Literature

Background: Lipomas are common benign tumors. When they develop in proximity to peripheral nerves, they can cause neurologic symptoms secondary to mass effect. Previous reports have shown symptom resolution after removal of lipomas compressing various upper extremity peripheral nerves. However, brachial plexus lipomas are relatively rare. Our multidisciplinary experience with brachial plexus lipoma resection is reviewed in the largest case series to date. Methods: A retrospective chart review of all patients undergoing resection of brachial plexus lipomatous tumors between 2006 and 2016 was performed. Patient demographic data, diagnostic imaging, clinical presentation, operative details, surgical pathology, and clinical outcomes were reviewed. Results: Twelve brachial plexus lipomatous tumors were resected in 11 patients: 10 lipomas, 1 hibernoma, and 1 atypical lipomatous tumor. The most common tumor location was supraclavicular (50%), followed by axillary (42%), and proximal medial arm (8%). The most common brachial plexus segment involved was the upper trunk (50%), followed by posterior cord (25%), lateral pectoral nerve (8%), lower trunk (8%), and proximal median nerve (8%). Most patients presented with an enlarging painless mass (58%). Of the patients who presented with neurologic symptoms, symptoms resolved in the majority (80%). Conclusions: Brachial plexus lipomas are rare causes of compression neuropathy in the upper extremity. Careful resection and knowledge of brachial plexus anatomy, which may be distorted by the tumor, are critical to achieving a successful surgical outcome with predictable symptom resolution. Finally, surveillance magnetic resonance imaging may be warranted for atypical lesions.

C7 nerve root sensory distribution in peripheral nerves: A bold functional magnetic resonance imaging investigation at 9.4 T: fMRI Study of C7 Nerve Distribution

Introduction: In this study we used a rat model to elucidate the linear make‐up of each major nerve of the upper limb by the C7 root through sensory stimulation and functional magnetic resonance imaging (fMRI). Methods: The C7 nerve root and major nerves of the right forelimb were stimulated electrically. Blood oxygen level–dependent functional magnetic resonance imaging (BOLD fMRI) was performed concurrently. Voxel overlap within the primary sensory cortex was calculated. Results: C7 comprised sensation in <1% in the musculocutaneous nerve, 6% in the ulnar nerve, 16% in the radial nerve, and 19% in the median nerve (P < 0.005 for each). The overlap was always <25% for each major nerve. Conclusions: This study helps explain why C7 is a suitable donor for brachial plexus injury treatment and why there is only a transient sensory deficit after transfer. Muscle Nerve 49: 40–46, 2014

C7 Nerve Root Sensory Distribution in Peripheral Nerves: A BOLD fMRI Investigation at 9.4T

Introduction: In this study we used a rat model to elucidate the linear make‐up of each major nerve of the upper limb by the C7 root through sensory stimulation and functional magnetic resonance imaging (fMRI). Methods: The C7 nerve root and major nerves of the right forelimb were stimulated electrically. Blood oxygen level–dependent functional magnetic resonance imaging (BOLD fMRI) was performed concurrently. Voxel overlap within the primary sensory cortex was calculated. Results: C7 comprised sensation in <1% in the musculocutaneous nerve, 6% in the ulnar nerve, 16% in the radial nerve, and 19% in the median nerve (P < 0.005 for each). The overlap was always <25% for each major nerve. Conclusions: This study helps explain why C7 is a suitable donor for brachial plexus injury treatment and why there is only a transient sensory deficit after transfer. Muscle Nerve 49: 40–46, 2014