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1.
Ann Plast Surg ; 92(5S Suppl 3): S320-S326, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38689413

ABSTRACT

PURPOSE: Resection of sacral neoplasms such as chordoma and chondrosarcoma with subsequent reconstruction of large soft tissue defects is a complex multidisciplinary process. Radiotherapy and prior abdominal surgery play a role in reconstructive planning; however, there is no consensus on how to maximize outcomes. In this study, we present our institution's experience with the reconstructive surgical management of this unique patient population. METHODS: We conducted a retrospective review of patients who underwent reconstruction after resection of primary or recurrent pelvic chordoma or chondrosarcoma between 2002 and 2019. Surgical details, hospital stay, and postoperative outcomes were assessed. Patients were divided into 3 groups for comparison based on reconstruction technique: gluteal-based flaps, vertical rectus abdominus myocutaneous (VRAM) flaps, and locoregional fasciocutaneous flaps. RESULTS: Twenty-eight patients (17 males, 11 females), with mean age of 62 years (range, 34-86 years), were reviewed. Twenty-two patients (78.6%) received gluteal-based flaps, 3 patients (10.7%) received VRAM flaps, and 3 patients (10.7%) were reconstructed with locoregional fasciocutaneous flaps. Patients in the VRAM group were significantly more likely to have undergone total sacrectomy (P < 0.01) in a 2-stage operation (P < 0.01) compared with patients in the other 2 groups. Patients in the VRAM group also had a significantly greater average number of reoperations (2 ± 3.5, P = 0.04) and length of stay (29.7 ± 20.4 days, P = 0.01) compared with the 2 other groups. The overall minor and major wound complication rates were 17.9% and 42.9%, respectively, with 17.9% of patients experiencing at least 1 infection or seroma. There was no association between prior abdominal surgery, surgical stages, or radiation therapy and an increased risk of wound complications. CONCLUSIONS: Vertical rectus abdominus myocutaneous flaps are a more suitable option for patients with larger defects after total sacrectomy via 2-staged anteroposterior resections, whereas gluteal myocutaneous flaps are effective options for posterior-only resections. For patients with small- to moderate-sized defects, local fasciocutaneous flaps are a less invasive and effective option. Paraspinous flaps may be used in combination with other techniques to provide additional bulk and coverage for especially long postresection wounds. Furthermore, mesh is a useful adjunct for any reconstruction aimed at protecting against intra-abdominal complications.


Subject(s)
Chordoma , Plastic Surgery Procedures , Sacrum , Humans , Male , Female , Middle Aged , Retrospective Studies , Plastic Surgery Procedures/methods , Aged , Adult , Aged, 80 and over , Chordoma/surgery , Sacrum/surgery , Chondrosarcoma/surgery , Surgical Flaps , San Francisco , Spinal Neoplasms/surgery
2.
Oral Maxillofac Surg Clin North Am ; 36(2): 237-245, 2024 May.
Article in English | MEDLINE | ID: mdl-38402140

ABSTRACT

Facial feminization is a complex undertaking requiring skill in both craniofacial and aesthetic plastic surgery. As in aesthetic procedures, understanding the patient's goals and setting realistic expectations in light of an individual's anatomy is critical. Both soft tissue and bone must be addressed to adequately soften masculine facial features. This article delves into specific anatomic areas and delineates some of the pathways to successful outcomes.


Subject(s)
Plastic Surgery Procedures , Sex Reassignment Surgery , Surgery, Plastic , Humans , Esthetics, Dental , Face/surgery , Surgery, Plastic/methods
3.
Plast Reconstr Surg Glob Open ; 11(9): e5259, 2023 Sep.
Article in English | MEDLINE | ID: mdl-37691705

ABSTRACT

Background: Side effects of opioid pain management after surgical repair of cleft lips are numerous and affect postoperative course. We compared opioid versus opioid-free pain management regimens for infants who underwent cleft lip repair to evaluate the impact on postoperative recovery. Methods: Cleft lip repairs at our institution from December 2016 to February 2021 were retrospectively reviewed, comparing patients who received opioids to patients receiving a nonopioid pain control regimen. Data collected include length of stay, oral morphine equivalents (OME) received on day of surgery (DOS)/postoperative day (POD) 1, time to and volume of first oral feed, and Face/Legs/Activity/Cry/Consolability (FLACC) scores. Results: Seventy-three infants were included (47 opioid and 26 nonopioid). The opioid group received average 1.75 mg OME on DOS and 1.04 mg OME on POD1. Average DOS FLACC scores were similar between groups [1.57 ±â€…1.18 nonopioid versus 1.76 ±â€…0.94 (SD) opioid; P = 0.46]. Average POD1 FLACC scores were significantly lower for the nonopioid group (0.73 ±â€…1.05 versus 1.35 ±â€…1.06; P = 0.022). Median time to first PO (min) was similar [178 (interquartile range [IQR] 66-411) opioid versus 147 (IQR 93-351) nonopioid; P = 0.65]. Median volume of first feed (mL) was twice as high for the nonopioid group [90 (IQR 58-120) versus 45 (IQR 30-60); P = 0.003]. Conclusions: Nonopioid postoperative pain management was more effective than opioids for pain management in infants after cleft lip repair, as evidenced by FLACC scores and increased volume of the first oral feed.

5.
J AAPOS ; 27(3): 165-166, 2023 06.
Article in English | MEDLINE | ID: mdl-37031917

ABSTRACT

Facial feminization surgery (FFS), or gender-affirming facial surgery, is a common procedure for patients with gender dysphoria. One goal of FFS involves extensive contouring of the frontal and nasal bones to reduce supraorbital bossing. Ophthalmic complications after FFS have been rarely reported. We report 2 cases of superior oblique palsy after FFS producing persistent vertical and torsional diplopia. One case was successfully treated with prism spectacles; the other required surgical management. Both cases likely involved surgical trauma to or disinsertion of the trochlea during orbital bony reshaping.


Subject(s)
Feminization , Trochlear Nerve Diseases , Male , Humans , Feminization/surgery , Eye , Trochlear Nerve Diseases/surgery , Diplopia/etiology , Paralysis
7.
Pediatr Dermatol ; 40(4): 755-758, 2023.
Article in English | MEDLINE | ID: mdl-36807931

ABSTRACT

Microtia is the term for congenital malformation of the ear in which the external and internal ear are absent or malformed. Surgical reconstruction is a common management approach and occasionally requires hair reduction of the newly constructed auricle. Few studies have investigated lasers for this purpose. We conducted a retrospective chart review of patients seen at a single institution between 2012 and 2021 who underwent laser hair reduction with long-pulsed neodymium-doped yttrium aluminum garnet laser (Nd:YAG). Efficacy ratings were done through review of clinical photographs. Twelve patients were identified with 14 total ears treated. The number of laser treatments varied from 1 to 9 sessions with an average of 5.1 treatments. The majority (8/12) had an "excellent" or "very good" response, one patient had a "good" response, and three were lost to follow-up. Other than pain, there were no side effects documented. Nd:YAG laser was both effective and safe in our pediatric cohort, without any cutaneous side effects in patients with darker skin.


Subject(s)
Congenital Microtia , Lasers, Solid-State , Humans , Child , Neodymium , Congenital Microtia/etiology , Retrospective Studies , Aluminum , Hair , Lasers, Solid-State/therapeutic use , Treatment Outcome
8.
Surg Endosc ; 37(3): 2290-2294, 2023 03.
Article in English | MEDLINE | ID: mdl-35982283

ABSTRACT

BACKGROUND: Perineal hernias can be secondarily acquired following abdominoperineal resection of the rectum. While transabdominal minimally invasive techniques have traditionally used laparoscopy, there are few studies published on the robotic platform, which has been gaining popularity for other types of hernia repairs. We review the existing literature, share a video vignette, and provide practical tips for surgeons interested in adopting this approach. METHODS: A literature search in Pubmed was performed to include all articles in English describing robotic repair of perineal hernias with identification of variables of interest related to repair. A case presentation with an accompanying video vignette and lessons learned from the experience are provided. RESULTS: Seven case reports (four containing video) published between 2019 and 2022 were included. Most articles (n = 5) utilized the Da Vinci Si or Xi, and most patients (n = 5) had undergone abdominoperineal resection with neoadjuvant chemotherapy to treat rectal cancer. Patients were positioned in Trendelenburg with rightward tilt (n = 2), modified lithotomy (n = 1), or a combination of the two (n = 1). All articles (n = 7) reported closing the defect and using mesh. Three articles describe placing five ports (one camera, three robotic, one assistant). There were no significant intraoperative or postoperative complications reported, and no recurrence noted at 3-27 months follow-up. Based on our experience, as shown in the video vignette, we recommend lithotomy positioning, using porous polypropylene mesh anchored to the periosteum of the sacrum and peritoneum overlying the bladder and side wall, and placing a drain above the mesh. CONCLUSIONS: A robotic transabdominal approach to perineal hernia repair is a viable alternate to laparoscopy based on low complication rates and lack of recurrence. Prospective and longer duration data are needed to compare the techniques.


Subject(s)
Hernia, Abdominal , Laparoscopy , Robotic Surgical Procedures , Humans , Prospective Studies , Hernia, Abdominal/surgery , Herniorrhaphy/methods , Surgical Mesh/adverse effects
9.
Ann Plast Surg ; 88(4 Suppl 4): S316-S319, 2022 05 01.
Article in English | MEDLINE | ID: mdl-35180755

ABSTRACT

BACKGROUND: Rectourethral fistula (RUF) is an uncommon serious condition with various etiologies including neoplasm, radiation therapy, and surgery. Treatment for RUF remains problematic with a high recurrence rate. Although studies have suggested the recurrence rate of RUF is lower after surgical repair using a gracilis flap, outcomes have varied and the studies were small and inadequately controlled. Here, we compare outcomes of RUF repair with and without gracilis flap to evaluate its efficacy in preventing fistula recurrence and identify risk factors for recurrence. METHODS: We retrospectively reviewed patients who had undergone surgical repair for RUF between 2007 and 2018 at our institution and had at least 30 days of follow-up. Patient demographics, comorbidities, and surgical outcomes were recorded and compared for patients who had gracilis flap repair and those who did not (controls). Single variable logistic regression analysis was used to identify risk factors for recurrence. RESULTS: The gracilis group (n = 24) and control group (n = 12) had similar demographics and comorbidities. Fistula recurrence was far less frequent in the gracilis group (8% vs 50%, P = 0.009). There were no significant differences in other outcomes including length of hospitalization and surgical complications. When recurrent RUF was treated with a muscle flap (gracilis or inferior gluteus), 83% of the group had no additional fistula recurrence. In the control group, history of radiation ( P = 0.04) and urinary incontinence ( P = 0.015) were associated with fistula recurrence. CONCLUSIONS: We recommend using a gracilis flap for RUF repair given its association with lower recurrence without increased surgical complications.


Subject(s)
Rectal Fistula , Urethral Diseases , Urinary Fistula , Humans , Retrospective Studies , Rectal Fistula/prevention & control , Rectal Fistula/surgery , Rectal Fistula/etiology , Surgical Flaps , Urethral Diseases/etiology , Urethral Diseases/prevention & control , Urethral Diseases/surgery , Urinary Fistula/etiology , Urinary Fistula/prevention & control , Urinary Fistula/surgery
10.
Plast Reconstr Surg Glob Open ; 10(2): e4097, 2022 Feb.
Article in English | MEDLINE | ID: mdl-35169528

ABSTRACT

BACKGROUND: Strip craniectomy with orthotic helmet therapy (SCOT) is an increasingly supported treatment for metopic craniosynostosis, although the long-term efficacy of deformity correction remains poorly defined. We compared the longterm outcomes of SCOT versus open cranial vault reconstruction (OCVR). METHODS: Patients who underwent OCVR or SCOT for isolated metopic synostosis with at least 3 years of follow-up were identified at our institution. Anthropometric measurements were used to assess baseline severity and postoperative skull morphology. Independent laypersons and craniofacial surgeons rated the appearance of each patient's 3D photographs, compared to normal controls. RESULTS: Thirty-five patients were included (15 SCOT and 20 OCVR), with similar follow-up between groups (SCOT 7.9 ± 3.2 years, OCVR 9.2 ± 4.1 years). Baseline severity and postoperative anthropometric measurements were equivalent. Independent adolescent raters reported that the forehead, eye, and overall appearance of SCOT patients was better than OCVR patients (P < 0.05, all comparisons). Craniofacial surgeons assigned Whitaker class I to a greater proportion of SCOT patients with moderate-to-severe synostosis (72.2 ± 5.6%) compared with OCVR patients with the same severity (33.3 ± 9.2%, P = 0.02). Parents of children who underwent SCOT reported equivalent satisfaction with the results of surgery (100% versus 95%, P > 0.99), and were no more likely to report bullying (7% versus 15%, P = 0.82). CONCLUSIONS: SCOT was associated with superior long-term appearance and perioperative outcomes compared with OCVR. These findings suggest that SCOT should be the treatment of choice for patients with a timely diagnosis of metopic craniosynostosis.

11.
Plast Reconstr Surg Glob Open ; 9(1): e3351, 2021 Jan.
Article in English | MEDLINE | ID: mdl-33564582

ABSTRACT

Whether treatment of cleft palate (CP) associated with Robin sequence (RS) should attain outcomes similar to those of isolated cleft palate (ICP) remains unknown. This study compares treatment and outcomes in both conditions and delineates predictors of long-term outcome. METHODS: This retrospective case series of consecutive syndromic and isolated RS- and ICP-patients (1990-2016) includes indications and outcomes of straight-line repair with intravelar veloplasty (SLIV) or Furlow repair depending on cleft and airway characteristics. RESULTS: Seventy-five RS and 83 ICP patients underwent CP repair. Velopharyngeal insufficiency (VPI) occurred in 41% of RS versus 17% of ICP patients (P = 0.012), and in 60% of patients with syndromic RS versus 16% with isolated RS (P = 0.005). In multivariable logistic regression analysis, wider and more severe CP anatomy was the only factor independently associated with VPI (P = 0.028), in contrast to age at repair, syndromic RS compared with isolated RS, and isolated RS compared with ICP and initial tongue-lip adhesion. Secondary Furlow after primary SLIV was used to treat VPI in all groups, and more frequently in syndromic versus isolated RS patients (P = 0.025). CONCLUSIONS: Variability of RS anatomy and airway compromise necessitates individualized treatment protocols. Despite differing CP etiology and other variables, our findings demonstrate cleft anatomy as the only independent variable predictive of VPI comparing RS and ICP patients. Patients with isolated RS should ultimately attain similar VPI outcomes compared with ICP patients. Obstructive speech operations in RS patients can be avoided without compromising speech outcome by reserving the prsocedure for secondary cases.

12.
Ann Plast Surg ; 87(2): 123-125, 2021 08 01.
Article in English | MEDLINE | ID: mdl-33346559

ABSTRACT

BACKGROUND: The Internet has a plethora of online patient education resources for many symptoms and diseases. National medical governing bodies recommend that patient education materials are written at or below the eighth-grade level, and the literature suggests that health literacy has been linked to increased adherence to treatment regimens and improved outcomes. The primary aim of the study is to assess the readability of online patient materials relating to gynecomastia and ascertain the availability of patient materials in non-English languages. METHODS: The readability of patient education materials relating to gynecomastia for academic-based websites and nonacademic websites was assessed using the Flesch Reading Ease (FRE), Flesch-Kincaid Grade Level (FKGL), and Simple Measure of Gobbledygook (SMOG). The prevalence of non-English patient education materials was assessed for both academic-based and nonacademic websites. RESULTS: Fifty-eight documents were collected across academic websites. Overall median values were 10.7 for the FKGL, 47.0 for the FRE, and 11.4 for the SMOG. For the 10 nonacademic institutions, the overall median values were 10.6 for the FKGL, 45.2 for the FRE, and 10.8 for the SMOG. No appreciable differences were observed for readability when stratified by region or source. The prevalence of non-English patient materials was 19.1% across institutions. None of the noninstitutional materials had information in non-English languages. CONCLUSIONS: The readability of patient education materials related to gynecomastia is at higher levels than recommended by national organizations. There are limited non-English patient education materials. Future efforts should focus on improving the readability and accessibility of patient materials.


Subject(s)
Gynecomastia , Health Literacy , Comprehension , Humans , Internet , Male , Patient Education as Topic , Reading
13.
BMJ Case Rep ; 13(12)2020 Dec 09.
Article in English | MEDLINE | ID: mdl-33298502

ABSTRACT

For patients whose vasculitis is managed with biologic medications, no reports or evidence-based guidance exists regarding the perioperative management of microvascular flaps. We present a case of a 78-year-old patient with Takayasu's arteritis (TA) and diabetes mellitus who was taking infliximab and underwent wide local excision of squamous cell carcinoma, craniectomy and reconstruction with a latissimus dorsi flap. TA, an immune-mediated large cell vasculitis characterised by granuloma formation, tends to affect larger vessels and aortic branches. The typical localisation of this condition raises concerns about potentially compromised pedicle and recipient vessels (ie, superficial temporal arteries), which could hinder postoperative flap success. Discontinuation of infliximab 4 weeks before surgery and resumption 6 weeks after led to favourable results. This case addresses the gap in the literature concerning stopping and restarting biologic drugs in the perioperative setting and documents a successful course of a microvascular procedure in a patient with vasculitis.


Subject(s)
Infliximab/administration & dosage , Squamous Cell Carcinoma of Head and Neck/surgery , Surgical Flaps/transplantation , Takayasu Arteritis/drug therapy , Aged , Craniotomy , Diabetes Mellitus, Type 2 , Drug Administration Schedule , Female , Humans , Male , Middle Aged , Plastic Surgery Procedures/methods
14.
Article in English | MEDLINE | ID: mdl-34307056

ABSTRACT

BACKGROUND: We report a rare case of a 19-year-old female progressively affected by a peripheral arteriovenous malformation (pAVM), a midline cerebellar astrocytoma, and a brain arteriovenous malformation (bAVM). CASE DESCRIPTION: She presented with a pulsatile mass on her left cheek, which was classified as a pAVM through angiography. Following treatment with embolization and surgical resection, she returned with enlargement of the mass and imaging incidentally identified a cerebellar astrocytoma. Suboccipital craniotomy, C1 laminectomy, and endoscopic third ventriculostomy were subsequently performed. She was later treated again for growth of her pAVM, and angiography revealed the presence of a left temporal bAVM, which was resected via a pterional craniotomy. CONCLUSIONS: Pathological staining identified activation of mTOR and RAS/MAPK pathway in the patient's pAVM and bAVM tissue samples. Furthermore, genetic sequencing demonstrated an activating MAPK21 (K57N) mutation in the pAVM and a gain of distal chromosome 7q in the pilocytic astrocytoma. No germline mutation was identified to explain all pathologies. This case demonstrates the need for continued development and further integration of multi-disciplinary genetic, radiological, and neurological treatment teams to effectively care for such complex presentations.

15.
Stem Cell Reports ; 10(3): 1160-1174, 2018 03 13.
Article in English | MEDLINE | ID: mdl-29478895

ABSTRACT

Investigation of human muscle regeneration requires robust methods to purify and transplant muscle stem and progenitor cells that collectively constitute the human satellite cell (HuSC) pool. Existing approaches have yet to make HuSCs widely accessible for researchers, and as a result human muscle stem cell research has advanced slowly. Here, we describe a robust and predictable HuSC purification process that is effective for each human skeletal muscle tested and the development of storage protocols and transplantation models in dystrophin-deficient and wild-type recipients. Enzymatic digestion, magnetic column depletion, and 6-marker flow-cytometric purification enable separation of 104 highly enriched HuSCs per gram of muscle. Cryostorage of HuSCs preserves viability, phenotype, and transplantation potential. Development of enhanced and species-specific transplantation protocols enabled serial HuSC xenotransplantation and recovery. These protocols and models provide an accessible system for basic and translational investigation and clinical development of HuSCs.


Subject(s)
Satellite Cells, Skeletal Muscle/cytology , Animals , Cell Separation/methods , Cells, Cultured , Dystrophin/metabolism , Humans , Mice , Muscle, Skeletal/cytology , Muscle, Skeletal/metabolism , Regeneration/physiology , Satellite Cells, Skeletal Muscle/metabolism , Stem Cell Transplantation/methods , Stem Cells/cytology , Stem Cells/metabolism , Transplantation, Heterologous/methods
16.
Cleft Palate Craniofac J ; 55(6): 807-813, 2018 07.
Article in English | MEDLINE | ID: mdl-28001101

ABSTRACT

BACKGROUND: Humanitarian surgical organizations provide palatoplasties for patients without access to surgical care. Few organizations have evaluated the outcomes of these trips. This study evaluates the palatal fistula rate in patients from two cohorts in rural China and one in the United States. METHODS: This study compared the odds of fistula formation among three cohorts whose palates were repaired between 2005 and 2009. One cohort included 97 Chinese patients operated on by teams from the United States and Canada under the auspices of Resurge International. They were compared to cohorts at Huaxi Stomatology Hospital and the University of California San Francisco (UCSF). Age, fistula presence, and Veau class were compared among cohorts using Chi-square tests. Logistic regression was used to analyze predictors of fistula formation. RESULTS: The fistula risk was 35.4% in patients treated by humanitarian teams, 12.8% at Huaxi University Hospital and 2.5% at UCSF ( P < 0.001). Age and Veau class were associated with fistula formation (Age P = 0.0015; Veau P < 0.001). ReSurge and Huaxi patients had 20.2 and 5.6 times the odds of developing a fistula, respectively, compared to UCSF patients ( P < 0.01, both). A multivariable model controlling for surgical group, age, and gender showed an association between Veau class and the odds of fistula formation. CONCLUSIONS: Chinese children undergoing palatoplasty by international teams had higher odds of palatal fistula than children treated by Chinese surgeons in established institutions and children treated in the United States. More research is required to identify factors affecting complication rates in low-resource environments.


Subject(s)
Cleft Palate/surgery , Oral Fistula/etiology , Organizations, Nonprofit , Plastic Surgery Procedures/methods , Practice Patterns, Physicians'/statistics & numerical data , Tertiary Care Centers , Canada , Child , Child, Preschool , China , Clinical Competence , Female , Humans , Infant , Male , Postoperative Complications , Retrospective Studies , Treatment Outcome , United States
17.
J Craniofac Surg ; 28(2): 325-330, 2017 Mar.
Article in English | MEDLINE | ID: mdl-28045814

ABSTRACT

BACKGROUND: Recurrent invasive skin cancer of the scalp and calvarium is a difficult problem for which universally accepted treatment protocols have not been established. The authors present their 10-year experience with treatment of this specific subset of scalp reconstruction patients and present a successful treatment algorithm that is well suited to this patient population. METHODS: The authors retrospectively reviewed all patients of microsurgical scalp reconstruction performed from 2005 to 2015 that involved invasive cutaneous malignancies of the scalp and calvarium. RESULTS: Eleven patients met inclusion criteria. There were 9 squamous cell carcinoma, 1 basal cell carcinoma, and 1 melanoma. Seven received radiation prior to resection, 2 were irradiated postoperatively, and 2 were immunosuppressed. Seven had a history of prior scalp reconstruction. The median scalp defect size was 141 cm. All the patients underwent craniectomy and the median cranial defect size was 71 cm. Cranioplasty was not performed in any patient. There were no intraoperative complications or flap loss. Recipient site complications included hematoma in 1, 1 seroma, 2 cerebral spinal fluid leaks, 3 partial skin graft loss. There was 1 donor site seroma in a patient who had a latissimus dorsi flap. All the patients reported satisfaction with the overall result and none were limited in activities by the existing cranial defect. CONCLUSIONS: This is the largest series published to date that focuses exclusively on management of cutaneous malignancies with intracranial invasion. Wide resection with craniectomy, and reconstruction with microvascular free tissue transfer without cranioplasty provides safe and reliable treatment of recalcitrant invasive scalp skin cancers with low morbidity and without major complications. Pre and postoperative radiation is well tolerated with this approach. The patients in this series were of advanced age and of a lifestyle for which cranioplasty is unnecessary for return to regular activities.


Subject(s)
Dermatologic Surgical Procedures/methods , Head and Neck Neoplasms/surgery , Plastic Surgery Procedures/methods , Scalp/surgery , Skin Neoplasms/surgery , Skull/surgery , Adult , Aged , Aged, 80 and over , Algorithms , Craniotomy , Female , Humans , Male , Middle Aged , Neoplasm Invasiveness , Plastic Surgery Procedures/adverse effects , Retrospective Studies , Skin Neoplasms/pathology , Surgical Flaps/adverse effects
18.
Pediatrics ; 137(3): e20152053, 2016 Mar.
Article in English | MEDLINE | ID: mdl-26908658

ABSTRACT

Acquired thoracic dystrophy is a complication associated with early open repair of pectus excavatum resulting from extensive cartilage resection. The condition can cause serious functional and physiologic impairments, including cardiac compression and restrictive pulmonary function. We describe a 17-year-old boy with acquired thoracic dystrophy after Ravitch repair of pectus excavatum during infancy, whom we treated with distraction osteogenesis. The patient had a marked deformity of the chest wall and general hypoplasia of the central portion of the ribcage, with resultant symptomatic dyspnea on exertion and reduced pulmonary function. After osteotomies and distraction osteogenesis of bilateral ribs 4-8 using customized distraction devices, he had improved thoracic contour, resolution of dyspnea, and decreased restrictive pulmonary symptoms. This case suggests that distraction osteogenesis, already used extensively in craniomaxillofacial and orthopedic surgery, may be a novel method for management of this condition.


Subject(s)
Osteogenesis, Distraction/methods , Ribs/surgery , Thoracic Wall/pathology , Thoracic Wall/surgery , Child , Forced Expiratory Volume , Funnel Chest/pathology , Funnel Chest/surgery , Humans , Male , Osteotomy/methods , Ribs/diagnostic imaging , Sternum/diagnostic imaging , Sternum/pathology , Sternum/surgery , Thoracic Wall/diagnostic imaging , Tomography, X-Ray Computed , Total Lung Capacity , Treatment Outcome , Vital Capacity
19.
Pediatr Dermatol ; 33(2): e129-30, 2016.
Article in English | MEDLINE | ID: mdl-26763884

ABSTRACT

We report the case of a mixed infantile hemangioma (IH) involving the right breast that resulted in pronounced hypoplasia of the affected breast, which became apparent after breast development at puberty. No treatment had been performed in infancy or childhood. This case demonstrates that the presence of an IH may affect development of the mammary gland bud and that systemic therapy should be considered in an attempt to minimize this adverse sequela.


Subject(s)
Breast/abnormalities , Diseases in Twins , Hemangioma/complications , Skin Neoplasms/complications , Cohort Studies , Female , Gestational Age , Humans , Male , Prospective Studies , Sex Factors , Twins, Dizygotic , Twins, Monozygotic
20.
Stem Cell Reports ; 5(3): 419-34, 2015 Sep 08.
Article in English | MEDLINE | ID: mdl-26352798

ABSTRACT

Identification of human satellite cells that fulfill muscle stem cell criteria is an unmet need in regenerative medicine. This hurdle limits understanding how closely muscle stem cell properties are conserved among mice and humans and hampers translational efforts in muscle regeneration. Here, we report that PAX7 satellite cells exist at a consistent frequency of 2-4 cells/mm of fiber in muscles of the human trunk, limbs, and head. Xenotransplantation into mice of 50-70 fiber-associated, or 1,000-5,000 FACS-enriched CD56(+)/CD29(+) human satellite cells led to stable engraftment and formation of human-derived myofibers. Human cells with characteristic PAX7, CD56, and CD29 expression patterns populated the satellite cell niche beneath the basal lamina on the periphery of regenerated fibers. After additional injury, transplanted satellite cells robustly regenerated to form hundreds of human-derived fibers. Together, these findings conclusively delineate a source of bona-fide endogenous human muscle stem cells that will aid development of clinical applications.


Subject(s)
Muscle, Skeletal/metabolism , Regeneration , Regenerative Medicine , Satellite Cells, Skeletal Muscle/transplantation , Animals , Antigens, Differentiation/metabolism , Heterografts , Humans , Mice , Muscle, Skeletal/pathology , Satellite Cells, Skeletal Muscle/metabolism , Satellite Cells, Skeletal Muscle/pathology
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