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Minimally Conjoined Craniopagus Twins: Case Report, Review of the Literature, and 28-Year Follow-upHoward, Rebecca A, Billington, Alicia R, MD, PhD, Powers, Jeremy M, MD, FACS 07 April 2022 (has links)
Conjoined twinning has always been a phenomenon of great interest to the medical community because of its rarity and complexity. Each additional documented case bolsters our knowledge of embryologic development, anatomic anomalies, and strategic approaches to surgical challenges. Twins conjoined at the head, that is, craniopagi, are the rarest subtype of conjoined twins, and rates of survival to birth and through surgical separation are low. Those that do survive usually require multiple intensive procedures and often face lifelong developmental challenges due to shared brain tissue that must be parsed at time of separation. Here we present a case study of partial angular frontal craniopagus twin females with a soft-tissue-only conjuncture currently unrepresented in medical literature. Documentation of this case, in which a one-stage surgical separation occurred at day of life 1 without complication or any long-term disability, helps inform those that provide care for conjoined twins alongside their expectant parents of the wide possible range of clinical presentations and prognosis for craniopagi. A 28-year follow-up investigates how the anatomic configuration of the craniopagi and mechanical forces in utero impacted craniofacial development and their phenotypic characteristics as adults. Using clinical examination, CT imaging with 3D reconstruction, and Vectra® H1 stereophotogrammetry, overall symmetry and congenital deformities of both women’s external features and underlying craniofacial skeleton were characterized. Both twins exhibit near-identical skull deformities including right frontal bone flattening, ipsilateral parietal and occipital bossing, flattened malar eminence, and curvature of sagittal plane through the skull. Reported right anterolateral displacement of anterior fontanelles directly under the twins’ junctional area during infancy is reflected by asymmetry of the coronal suture withanterior displacement on the right side in both cases. In addition, Twin B has a persistent metopic suture that also shows lateral displacement toward site of junction. Soft tissue deformities include minor cosmetic asymmetries such as a sharp hairline irregularity below previous site of conjuncture, misaligned ears, sparse right eyebrow growth and weakened movement of this brow, and deviation of nasal tip in one twin. Both twins also sport a 5cm by 1.5-2cm surgical scar. Overall symmetry is good and most of these irregularities go unnoticed by laypeople; the women manage most of them with cosmetics and side-parting of the hair. Though some minor plastic surgery procedures could be offered to optimize symmetry, none are recommended or desired by the patients at this time. Through careful evaluation of their adult state, we conclude that minimally conjoined craniopagus twins sharing only soft tissue of the scalp at time of birth can still exhibit a number of lifelong cranial deformities and asymmetry of facial landmarks.
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Referrals to Cleft Lip and Palate Teams: Practices of School-Based Speech-Language PathologistsBuckles, Rachael, Burrows, Allison, Deel, Caitlyn, Holley, Elizabeth, Monroe, Ellen, Page, Olivia, Louw, Brenda 31 March 2020 (has links)
Cleft lip and palate (CLP) has been determined to be the second most common birth defect in the United States, affecting 1 in every 940 births (Parker et al., 2010). The team approach is the accepted best practice for children with CLP (Kummer, 2020) and the school-based Speech-Language Pathologist (SLP) has an important role to play in assessment and intervention of children with repaired CLP, however there is little research to describe their collaboration. This research aimed to explore and describe the referral practices of school-based SLP’s to CLP teams. A survey titled “Referral to Cleft Lip and Palate Teams: Practice of School-Based Speech Language Pathologist’s” was developed and distributed to members of the American Speech-Language Hearing Association’s (ASHA’s) Special Interest Groups (SIGs) 15 and 16 following an in depth literature review on the topic. A total of 57 practicing school-based SLPs acted as respondents. The results of the survey suggested VPD was the main reason for making a referral to a CLP team (89.72%), which validates the response that clients mostly referred had suspected VPD (89.47%). Making a team referral was not common practice, as 58.7% had never made a CLP team referral in the schools. ENTs (51.06%) were the preferred choice of referral in comparison to a CLP team (25.53%). Barriers to making CLP team referrals varied and obtaining permission from the school was experienced by some respondents (36.36%). Respondents made valuable comments which centered on positive experiences with working with CLP teams (11/56). The process of making referrals to CLP teams and collaboration between school-based SLPs and CLP teams needs to be addressed in graduate training and CE. According to Vallino et al., (2019) such communication enhances care, bridges the perceived gap between school-based SLPs and CLP teams, and will ensure that children with CLP and VPD receive the best care possible.
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Young Adults with Cleft Lip and Palate: Personal Perspectives on Transition of CareBuchanan, Courtney, Johnson, Bethany, Morgan, Jade, Morgan, Jessica, Padgett, Carissa, Louw, Brenda 12 April 2019 (has links)
Children with cleft lip/palate receive team care which typically ends at eighteen. Young adults then need to transition into an adult centered model of care. A paucity of literature exists regarding their perspective on transition of care experience. This research explores the experiences young adults with CLP regarding their transition of care process, within the person centered ICF framework.
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Modern Technology Applied to Classic Technique: Virtual Surgical Planning for Design of Split-Calvarial Bone Graft for Frontal Reconstruction after Osteoma Resection in a Fifteen-Year-Old MaleMartin, Taylor, Johnson, Abbey, Condra, Alex, Horsley, Neil, Powers, Jeremy 25 April 2023 (has links)
Osteoid osteomas are common, painful, osteoblast tumors that frequently present in adolescents and young adults. Surgical management is indicated for cases with associated symptoms, such as headaches, dizziness, diplopia, proptosis, sinusitis, and facial deformity. The authors report a case of a 15-year-old male with an enlarging frontal sinus osteoma. Virtual surgical planning was utilized to design a KLS Martin Resorbable plate for the graft site, as well as a cutting guide for the graft procedure. A split-calvarial bone graft was used to reconstruct the central bony defect in the anterior table of the frontal sinus. Virtual surgical planning sessions were initiated with KLS Martin biomedical engineers to design a graft. Design cutting guides were created for frontal craniotomy, and an interlocking cutting guide was created for the split calvarial bone graft. The graft was harvested from the non-dominant right parietal hemisphere to best match the curvature of the frontal bone. Post-operatively, the patient has not had any complications to date. This procedure proves to be a valuable consideration for the surgical treatment of pediatric osteomas.
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Osteonecrosis of Jaw: Common etiologies, uncommon treatmentsPanta, Utsab, chan, Adam, Das, Debalina 12 April 2019 (has links)
Introduction
First described in 2002, osteonecrosis of the jaw (ONJ, or avascular necrosis of the jaw) is an uncommon but potentially serious side effect of treatment with bisphosphonates. Although typically identified in patients with multiple myeloma and other malignancies, a few cases have been reported in patients taking bisphosphonates - a potent drug class used in the treatment of osteoclast-mediated bone resorption issues, including postmenopausal osteoporosis, Paget's disease, multiple myeloma, and malignant hypercalcemia. The clinical diagnosis of ONJ can be obscured by jaw pain, abscess, swelling, and fistulas, but exposed bone is a distinctive sign. This reports a case of ONJ secondary to bisphosphonate use in a 65-year-old woman and clinical management complications.
Case Presentation
A 65-year-old lady with history of age-related osteoporosis and compression fractures on alendronate for 4 years, squamous cell carcinoma of neck status post excision and radiotherapy 11-years prior, Sjogren's syndrome and discoid lupus on hydroxychloroquine, diabetes, hypertension, stroke and multiple dental abscesses presents with persistent neck pain. Initial CT neck with contrast showed diffuse fat stranding. Subsequently, alendronate was discontinued due to jaw necrosis suspicion. Eight months later, repeat CT scan showed new non-mass-like soft tissue thickening in the subcutaneous fat abutting the right anterior mandible with mandibular teeth cavities and periapical lucencies, likely to be periodontal cellulitis versus radiation osteonecrosis. Later, patient complained of a piece of bone penetrating the skin of her chin and presented with continuous drainage from sinus tract in her mandible, which was diagnosed as osteonecrosis attributed to bisphosphonates, previous radiation therapy, and dental abscesses. Patient was started on abaloparatide, an osteo-anabolic medication for osteoporosis and enrolled in hyperbaric oxygen therapy which immensely helped in controlling sinus drainage. Patient is currently awaiting mandibular reconstruction surgery.
Discussion
ONJ, often associated with pain, swelling, exposed bone, local infection, and pathologic fracture of the jaw, is a rare complication of bisphosphonate therapy. Currently, no prospective data exists to advise the benefits of therapy discontinuation however most clinical practices tend to discontinue at least temporarily. The incidence increases with longer treatment duration, particularly when therapy exceeds four years. Risk factors for developing ONJ while taking bisphosphonates include IV administration, anticancer therapy, dose and duration of exposure, dental extractions/implants, glucocorticoids, smoking, diabetes, and preexisting dental disease.
Case reports and series suggest benefit from hyperbaric oxygen therapy in wound healing, pain, and quality of life at three months, however no significant differences exist with outcomes beyond three months. Patients being considered for therapy with a bisphosphonate should be thoroughly evaluated for dental issues, prior to initiating therapy. Conservative management with limited debridement, antibiotic therapy as needed, and topical mouth rinses rather than aggressive surgical resection are recommended. Conservative therapy may result in healing in a significant proportion of patients. Surgical resection of necrotic bone should be reserved for refractory or advanced cases. Providers should remain cautious while prescribing high doses of bisphosphonates in patients with increased risk factors to prevent, timely diagnose and treat this condition.
References
Edwards BJ, Gounder M, McKoy JM, et al. Pharmacovigilance and reporting oversight in US FDA fast-track process: bisphosphonates and osteonecrosis of the jaw. Lancet Oncol 2008; 9:1166.
Khosla S, Burr D, Cauley J, et al. Bisphosphonate-associated osteonecrosis of the jaw: report of a task force of the American Society for Bone and Mineral Research. J Bone Miner Res 2007; 22:1479.
Hoff AO, Toth BB, Altundag K, et al. Frequency and risk factors associated with osteonecrosis of the jaw in cancer patients treated with intravenous bisphosphonates. J Bone Miner Res 2008; 23:826.
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