Hybrid lung lesions in children with segmental infantile hemangiomas, a new association?

Infantile hemangiomas (IHs) are the most common tumors of infancy and, in rare instances, can present in the setting of congenital structural anomalies or as part of syndromic disorders. In this study, we present three cases of children with segmental IHs born with concurrent pulmonary anomalies: congenital pulmonary airway malformations and bronchopulmonary sequestration. To date, no known association between these entities and hemangiomas has been described.

Effect of Gene Mutation on Seizures in Surgery for Tuberous Sclerosis Complex.

BACKGROUND: Tuberous sclerosis complex (TSC) is a rare genetic disorder that commonly leads to drug-resistant epilepsy in affected patients. This study aimed to determine whether the underlying genetic mutation (TSC1 vs. TSC2) predicts seizure outcomes following surgical treatments for epilepsy. METHODS: We retrospectively assessed TSC patients using the TSC Natural History Database core registry. Data review focused on outcomes in patients treated with surgical resection or vagus nerve stimulation. RESULTS: A total of 42 patients with a TSC1 mutation, and 145 patients with a TSC2 mutation, were identified. We observed a distinct clinical phenotype: children with TSC2 mutations tended to be diagnosed with TSC at a younger age than those with a TSC1 mutation (p < 0.001), were more likely to have infantile spasms (p < 0.001), and to get to surgery at a later age (p = 0.003). Among this TSC2 cohort, seizure control following resective epilepsy surgery was achieved in less than half (47%) the study sample. In contrast, patients with TSC1 mutations tended to have more favorable postsurgical outcomes; seizure control was achieved in 66% of this group. CONCLUSION: TSC2 mutations result in a more severe epilepsy phenotype that is also less responsive to resective surgery. It is important to consider this distinct clinical disposition when counseling families preoperatively with respect to seizure freedom. Larger samples are required to better characterize the independent effects of genetic mutation, infantile spasms, and duration of epilepsy as they relate to seizure control following resective or neuromodulatory epilepsy surgery.

Soft Tissue Contouring at the Time of Osseointegrated Implant Reconstruction for Lower Extremity Amputation.

INTRODUCTION: Patients with lower extremity amputations using a classic socket prosthesis face many challenges related to the socket-limb interface. The adaptation of osseointegration has allowed for the attachment of a prosthesis directly to bone, eliminating this interface and providing mechanical benefits. Contrary to the socket prosthesis, the osseointegrated prosthesis requires reducing and minimizing the soft tissue envelope. Studies have shown that patients who have undergone placement of these implants have high rates of reoperation for soft tissue redundancy. The purpose of our study was to evaluate complication rates and need for revisional surgery using our technique of soft tissue closure around the prosthesis at the time of implant placement. METHODS: An institutional review board-approved, retrospective chart review was performed on all patients who underwent implantation of an osseointegrated prosthesis for lower extremity amputation with concomitant plastic surgery closure at our institution during a 2-year period from June 2017 to June 2019. Patient demographics, health status descriptors, operative data, length of admission, and rates of postoperative complications were gathered from the electronic medical record and coded into a HIPAA-compliant database. Specific outcomes tracked included minor and major infection, osteomyelitis, implant failure, hematoma, seroma, delayed wound healing, and rates of reoperation and readmission. RESULTS: There were a total of 14 patients who underwent osseointegrated implant placement with concomitant plastic surgical coverage of the prosthesis during the study period. The average patient age was 50 years (range, 26-70 years), and average body mass index was 32.2 kg/m (range, 19.7-44.8 kg/m). Average follow-up time was 28 weeks (range, 10-73 weeks). There were 2 cases of local infection resolved with a course of oral antibiotics. There were no instances of infection requiring procedural intervention or hospital admission, nor any cases of osteomyelitis. Two patients required outpatient surgery for exchange of implant abutment, one required revision of a prosthesis for hardware loosening, and one required targeted muscle reinnervation of a sciatic nerve neuroma. There were no patients who required revisional surgery for soft tissue redundancy and no cases of delayed wound healing. CONCLUSIONS: Adequate planning of incisions and soft tissue contouring is important in the care of osseointegrated patients. Plastic surgery involvement can decrease soft tissue complications and lead to improved patient outcomes.

Infantile Hemangiomas Lose Vascular Endothelial Cadherin During Involution: Potential Role in Cell Death?

Background: Infantile hemangiomas (IHs) are benign endothelial cell (EC) tumors that undergo a predictable natural history, with rapid proliferation, stabilization, and involution. However, mechanisms regulating these transitions are not well understood. We have observed loss of vascular endothelial cadherin (VECAD) in involuting/involuted IHs. VECAD plays a critical role in angiogenesis, cell cycle progression, and EC survival. We hypothesize that loss of VECAD is associated with apoptosis occurring during IH involution. Methods: Resected IH samples were clinically categorized as proliferating (n = 4), stable (n = 4), or involuting/involuted (n = 5). Neonatal dermal tissues were used as controls (n = 5). Immunohistochemistry was conducted on sectioned specimens using antibodies against EC markers VECAD and CD31. Apoptosis was assessed with terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling assay. Results: CD31 signal intensity in proliferating, stable, and involuting/involuted IH ECs was unchanged relative to each other and to control ECs. VECAD signal significantly and progressively diminished as IHs progressed from proliferation to involution. Involuting/involuted IHs had significantly reduced VECAD expression compared with control ECs (P < 0.0001), proliferating IHs (P < 0.0001), and stable IHs (P < 0.001). As expected, the number of terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling-positive ECs was significantly higher in involuting/involuted IHs (P < 0.05) relative to control ECs and proliferating IHs. Conclusions: Loss of VECAD expression in IH endothelium corresponded to IH involution and increased apoptosis. It is unclear whether loss of VECAD is causative of IH involution; further studies are needed to elucidate the role of VECAD function in EC survival.

Propranolol Therapy in Infantile Hemangioma: It Is Not Just About the Beta.

BACKGROUND: Propranolol, a nonselective ß-adrenergic receptor antagonist, is approved by the U.S. Food and Drug Administration to treat problematic infantile hemangiomas, but a subset of patients experience treatment complications. Parents wary of long-term use and side effects consult plastic surgeons on surgical options or as a second opinion. Understanding the mechanism(s) of action of propranolol will allow plastic surgeons to better inform parents. METHODS: A systemic literature search was performed to query published translational and basic science studies on propranolol effects on infantile hemangiomas and cells derived from these lesions. RESULTS: In experimental studies, propranolol was antiproliferative and cytotoxic against hemangioma endothelial and stem cells and affected infantile hemangioma perivascular cell contractility. Propranolol inhibited migration, network formation, vascular endothelial growth factor A production, and vascular endothelial growth factor receptor 2 activation and down-regulated PI3K/AKT and mitogen-activated protein kinase signaling in hemangioma endothelial cells, but it increased ERK1/2 activity in hemangioma stem cells. At effective clinical doses, measured propranolol plasma concentration is 100 times higher than necessary for complete ß-adrenergic receptor blockade, yet was 10 to 100 times less than required to induce hemangioma stem cell death. CONCLUSIONS: Propranolol targets multiple cell types in infantile hemangiomas by means of ß-adrenergic receptor-dependent and -independent mechanisms. Plasma concentration played a significant role. At clinically relevant doses, incomplete infantile hemangioma suppression may explain the rebound phenomenon and worsening ulceration, and propranolol off target effects may lead to commonly reported adverse effects, such as sleep and gastrointestinal disturbances. Propranolol limitations and complications underscore the importance of surgical treatment options in cases of rebound and severe adverse effects. Surgical intervention remains an important treatment choice when parents are hesitant to use propranolol.

Readability and quality of wikipedia pages on neurosurgical topics.

OBJECTIVES: Wikipedia is the largest online encyclopedia with over 40 million articles, and generating 500 million visits per month. The aim of this study is to assess the readability and quality of Wikipedia pages on neurosurgical related topics. PATIENTS AND METHODS: We selected the neurosurgical related Wikipedia pages based on the series of online patient information articles that are published by the American Association of Neurological Surgeons (AANS). We assessed readability of Wikipedia pages using five different readability scales (Flesch Reading Ease, Flesch Kincaid Grade Level, Gunning Fog Index, SMOG) Grade level, and Coleman-Liau Index). We used the Center for Disease Control (CDC) Clear Communication Index as well as the DISCERN Instrument to evaluate the quality of each Wikipedia article. RESULTS: We identified a total of fifty-five Wikipedia articles that corresponded with patient information articles published by the AANS. This constitutes 77.46% of the AANS topics. The mean Flesch Kincaid reading ease score for all of the Wikipedia articles we analyzed is 31.10, which indicates that a college-level education is necessary to understand them. In comparison to the readability analysis for the AANS articles, the Wikipedia articles were more difficult to read across every scale. None of the Wikipedia articles meet the CDC criterion for clear communications. CONCLUSION: Our analyses demonstrated that Wikipedia articles related to neurosurgical topics are associated with higher grade levels for reading and also below the expected levels of clear communications for patients. Collaborative efforts from the neurosurgical community are needed to enhance the readability and quality of Wikipedia pages related to neurosurgery.