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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.

Facial feminization surgery (FFS) is one of many gender affirming surgeries that can reduce gender dysphoria and rate of mis-gendering. A multidisciplinary team with experience caring for transgender patients is valuable for generating high quality outcomes and patient satisfaction. In particular, specific technical pearls can assist in improving outcomes of this procedure. This article provides a review of patient and procedure selection for a range of FFS procedures including chondrolaryngoplasty, hairline advancement, forehead reduction and recontouring, orbital opening and brow lift, rhinoplasty, lip lift or augmentation, malar augmentation, and mandibular recontouring and genioplasty. The authors share technical insights and pre- and postoperative management recommendations gained from experiences at 2 institutions performing high volume FFS.

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.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.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.We recommend using a gracilis flap for RUF repair given its association with lower recurrence without increased surgical complications.

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).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.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 (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.

Purpose The aim of this study was to measure the cortical bone thickness of the infrazygomatic crest area in individuals with unilateral cleft lip and palate using cone beam computed tomography for placement of miniplates used for bone anchored maxillary protraction. Materials and methods CBCT scans were obtained from 31 non-syndromic UCLP children diagnosed with maxillary hypoplasia (17 males, 14 females, mean age: 11.9 years). 5 horizontal and 5 vertical reference planes were drawn at the infrazygomatic crest area. The cortical bone thickness at 25 intersection points on the cleft side and the non-cleft side was measured. Results The mean cortical bone thickness of the 25 measured points was 1.19 mm on the cleft side and 1.17 mm on the non-cleft side with no significant difference. The greatest cortical bone thickness was found to be at the most superior, posterior point (H+6, V+0), which was 1.49 mm on the cleft side and 1.47 mm on the non-cleft side. The thinnest mean cortical bone thickness was measured at the most inferior, anterior point (H-2, V-8), which was 0.94 mm on the cleft side and 0.95 mm on the non-cleft side. There was no significant difference between males and females.

Supplemental Digital Content is available in the text., Background: 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.

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.

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.

Minimally invasive gender-affirming procedures (MIGAPs), which aim to align gender identity and expression for transgender and gender-nonbinary patients in a way that is safe, effective, and semipermanent or reversible, are gaining in popularity. This article assesses the current amount of trainee exposure in clinic and didactic sessions in core procedural specialties nationwide via survey study of program directors. Low exposure of residents and fellows to MIGAPs was observed overall and a lack of procedure-specific education. In an effort to provide excellent patient care, promote cultural humility, and improve patients' quality of life, further education regarding these procedures is necessary.

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.

Objective:Study the prevalence of otologic disease in a pediatric post-palatoplasty population with no prior ear tube placement in resource-deprived countries and assess patient characteristics associated with these abnormal results.Design:Retrospective data review.Participants:Ecuadorian and Chinese children identified during humanitarian cleft lip and palate repair trips with cleft palates undergoing palatoplasty from 2007 to 2010.Interventions:Tympanometry and otoacoustic emission (OAE) testing performed following palatoplasty. Patients’ parents administered surveys regarding perceived hearing deficits.Main Outcome Measures:Age, gender, Veau classification, follow-up time, laterality, and country of origin were evaluated for possible association with type B tympanogram, “Refer” Otoacoustic results, and presence of hearing difficulty as identified by a parent. Significant predictors were further evaluated with multivariate analysis.Results:The cohorts included 237 patients (129 Ecuadorian, 108 Chinese); mean age: 3.9 years; mean follow-up: 4.2 years. Thirty-nine percent scored type B, 38% failed OAE testing, and 8% of parents noted hearing deficits. The country of origin and a younger age were identified as predictive variables regarding type B tympanogram. Follow-up time, country of origin, and bilateral OAE “Refer” results all significantly predicted parental questionnaire results. Subsequent multivariable analysis further demonstrated effect modification between the 2 variables of age at palatoplasty and country of origin when predicting type B vs type A tympanometry.Conclusion:Without otologic intervention, cleft palate children in resource-deprived settings suffer type B tympanometry and failed OAE results with similar to increased incidences to other studied cleft palate populations with otologic interventions available.

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.

Author(s): Gautam, Ayushi; Sun, Zhengda; Winkler, Ethan; Su, Hua; McCalmont, Timothy H; Kim, Helen; Tihan, Tarik; Hoffman, William Y; Dowd, Chris F; Frieden, Ilona J; Cooke, Daniel L | Abstract: BackgroundWe 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 descriptionShe 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.ConclusionsPathological 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.

Summary 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., Highlights • High-efficiency purification permits serial transplantation of human satellite stem cells • Cryopreservation preserves satellite cell function and phenotype • 1 gram of adult skeletal muscle yields 104 highly purified satellite cells • Purified uncultured endogenous human satellite cells can be stored and shared, Garcia and colleagues report methods for efficient purification of satellite cells from human skeletal muscle. They use their approaches to demonstrate stem cell functions of endogenous satellite cells and to make human satellite cells accessible for sharing among researchers.

BACKGROUND The poorly healing perineal wound is a significant complication of abdominoperineal resection. The authors examined criteria for immediate flap coverage of the perineum and long-term cross-sectional surgical outcomes. METHODS Patients who underwent abdominoperineal resection or pelvic exenteration for anorectal cancer were retrospectively analyzed. Demographic characteristics, premorbid and oncologic data, surgical treatment, reconstruction method, and recovery were recorded. Outcomes of successful wound healing, surgical complications necessitating intervention (admission or return to the operating room), and progression to chronic wounds were assessed. RESULTS The authors identified 214 patients who underwent this procedure from 1995 to 2013. Forty-seven patients received pedicled flaps and had higher rates of recurrence and reoperation, active smoking, Crohn disease, human immunodeficiency virus, and anal cancers, and had higher American Joint Committee on Cancer tumor stages. Thirty-day complication rates were equivalent in the two groups. There were no complete flap losses or reconstructive failures. Perineal wound complication rates were marginally but not significantly higher in the flap group (55 percent versus 41 percent; p = 0.088). Infectious complications, readmissions for antibiotics, and operative revision were more frequent in the flap cohort. A larger proportion of the primary closure cohort developed chronic draining perineal wounds (23.3 versus 8.5 percent; p = 0.025). CONCLUSIONS Immediate flap coverage of the perineum was less likely to progress to a chronic draining wound, but had higher local infectious complication rates. The authors attribute this to increased comorbidity in the selected patient population, reflecting the surgical decision making in approaching these high-risk closures and ascertainment bias in diagnosis of infectious complications with multidisciplinary examination. CLINICAL QUESTION/LEVEL OF EVIDENCE Risk, III.

Venous malformations (VMs) are congenital anomalies of the venous vasculature, but not all are evident at birth. The factors that lead to presentation later in life are not well understood. The objective of this retrospective cohort study of patients with VMs evaluated at the University of California at San Francisco Birthmarks and Vascular Anomalies Center from 2005 to 2009 was to investigate the clinical presentation of VMs and correlate these features with different types of tissues (e.g., skin, subcutis, intramuscular). Main outcomes included the age at which lesions were first noticed, tissue type involved, presenting signs and symptoms, aggravating factors, and morbidities. A total of 115 subjects was included. The mean age when VM was first noted was 6.7 ± 0.9 years. Tissue types involved included skin/subcutaneous (46%); intramuscular (40%); and bone, tendon, or joint (14%). Presenting signs/symptoms included soft tissue swelling (44%), discrete mass (34%), pain (33%), and skin discoloration (26%). When compared with VMs limited to the skin or subcutis, those restricted to the intramuscular compartment were less likely to present at birth (27% vs 53%, p < 0.05) but were more frequently painful (79% vs 60%, p < 0.05) and contained more phleboliths (28% vs 11%, p < 0.05), and were associated with more exercise limitation (35% vs 16%, p < 0.05). VMs differ in age of onset, clinical features, and complications based on differing tissues and sites of involvement, with isolated intramuscular involvement associated with later presentation and greater morbidity.

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.

Background Mandibular osteoradionecrosis is the most devastating complication after radiation therapy for head and neck malignancies. Quality of life (QOL) after surgical treatment is unclear. Methods A retrospective cohort analysis (1997–2007) was conducted of all patients treated at our institution for stage II and III mandibular osteoradionecrosis. Nineteen of 35 patients responded to a modified University of Washington QOL questionnaire. Twenty had undergone reconstruction using free flaps, and the remainder with plates, plates and local flaps, or debridement alone. Results Complications included 3 infections, 5 with hardware, 5 flap-specific, and 1 nonunion. Four patients had recurrent squamous cell carcinoma (SCC). The factors of greatest concern to patients were appearance, swallowing, and chewing. Average overall QOL was good to very good, and very good compared to preoperative. Conclusion Despite a 37% complication rate, a multidisciplinary team approach with adequate debridement, resection, and reconstruction can greatly improve QOL. © 2011 Wiley Periodicals, Inc. Head Neck, 2012

Free flap success depends on rapid identification and subsequent salvage of failing flaps. Conventional free flap monitoring techniques require an external component, whereas an implantable monitor readily indicates changes in free flap perfusion, especially in buried flaps used in head and neck reconstruction.This is a retrospective review of 169 consecutive head and neck free flaps reconstructed mostly for oncologic surgical defects in 155 patients from April of 2000 to December of 2006, all of which were monitored by an implantable venous Doppler device.There were 25 buried flaps, representing 14.8 percent of 169 flaps. Flap ischemia caused by thrombosis (n = 16), hematoma (n = 2), or tight closure (n = 1) occurred in 11.2 percent of the cases. The Doppler probe detected all of the failing free flaps, and we were able to salvage 18 of 19 ischemic flaps (94.7 percent). All Doppler-detected ischemic nonburied flaps (100 percent) and three of the four buried free flaps were salvaged (75 percent). There were 33 total complications (19.5 percent), with thrombosis occurring in 9.5 percent of the flaps, whereas 12 flaps required reoperation for vascular revision (7.1 percent). The mortality rate was less than 1 percent (0.6 percent). The overall success rate using the implantable Doppler probe was 98.2 percent, which was similar to that of the most recent reported cases of all free flaps in the literature, with significant improvement in the salvage rate for both buried and nonburied head and neck free flaps.The implantable Doppler probe is a useful monitoring device in buried free flaps and should be considered for use in head and neck reconstruction.

Objective:To assess the radiographic outcome of secondary alveolar bone grafting in individuals with nonsyndromic unilateral or bilateral cleft lip and palate using cone beam computed tomography.Methods:This prospective study was conducted at the University of California at San Francisco Center for Craniofacial Anomalies on 21 consecutive nonsyndromic complete cleft lip and palate individuals between 8 and 12 years of age who required alveolar bone grafting. Seventeen unilateral and four bilateral cleft lip and palate individuals had preoperative and postoperative cone beam computed tomography scans that were analyzed using Amira 3.1.1 software.Results:The average volume of the preoperative alveolar cleft defect in unilateral cleft lip and palate was 0.61 cm3, and the combined average volume of the right and left alveolar cleft defects in bilateral cleft lip and palate was 0.82 cm3. The average percentage bone fill in both unilateral cleft lip and palate and bilateral cleft lip and palate was 84%. The outcome of alveolar bone grafting was assessed in relation to (1) type of cleft, (2) size of preoperative cleft defect, (3) presence or absence of lateral incisor, (4) root development stage of the maxillary canine on the cleft side, (5) timing, and (6) surgeon. None of these parameters significantly influenced the radiographic outcome of alveolar bone grafting.Conclusions:Secondary alveolar bone grafting of the cleft defect in our center was successful, based on radiographic outcome using cone beam computed tomography scans. Volume rendering using cone beam computed tomography and Amira software is a reproducible and practical method to assess the preoperative alveolar cleft volume and the adequacy of bone fill postoperatively.

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.

© 2015, Springer-Verlag Berlin Heidelberg. Background: The incidence of localized intravascular coagulopathy (LIC) in venous malformations varies with lesion size and location, as well as the presence of palpable phleboliths. The development of LIC can cause pain and hemorrhage and can progress to disseminated intravascular coagulopathy (DIC) and thromboembolic disease resulting in death in some cases. Early recognition of LIC can relieve symptoms and prevent progression to life-threatening complications. Objective: The aim of this work was to identify MRI features of venous malformation associated with LIC. We hypothesized that venous malformations with larger capacitance, slower flow and less physiological compression (greater stasis) were more likely to be associated with LIC. Materials and methods: In this HIPAA-compliant and IRB-approved study, we retrospectively reviewed clinical records and MRI for consecutive patients undergoing evaluation of venous malformations at our multidisciplinary Birthmarks and Vascular Anomalies Center between 2003 and 2013. Inclusion required consensus diagnosis of venous malformation and availability of laboratory data and MRI; patients on anticoagulation or those previously undergoing surgical or endovascular treatment were excluded. LIC was diagnosed when D-dimer exceeded 1,000 ng/mL and/or fibrinogen was less than 200 mg/dL. Two board-certified radiologists assessed the following MRI features for each lesion: morphology (spongiform vs. phlebectatic), presence of phleboliths, size, location (truncal vs. extremity), and tissue type(s) involved (subcutis, muscle, bone and viscera). Univariate logistic regression analyses were used to test associations between LIC and MRI findings, and stepwise regression was applied to assess the significance of the individual imaging predictors. Results: Seventy patients, 37 with LIC, met inclusion criteria during the 10-year study period (age: 14.5 +/- 13.6 years [mean +/- standard deviation]; 30 male, 40 female). Both elevated D-dimer and low fibrinogen were associated with the presence of phleboliths, larger lesion sizes and visceral involvement on MRI (all P < 0.05). In stepwise regressions, lesion size (P < 0.001), the presence of phleboliths (P = 0.005) and lesion morphology (P = 0.006) were all significant predictors of LIC. Conclusion: LIC is associated with larger lesion size, visualized phleboliths, truncal location and spongiform morphology on MRI in venous malformations, suggesting that lesions with larger capacitance, slower flow and less physiological compression are more likely to be associated with coagulopathy.

BackgroundThe absence of a discrete mass, surrounding signal abnormality and solid enhancement are imaging features that have traditionally been used to differentiate soft-tissue arteriovenous malformations from vascular tumors on MRI. We have observed that these findings are not uncommon in arteriovenous malformations, which may lead to misdiagnosis or inappropriate treatment.ObjectiveTo estimate the frequency of atypical MRI features in soft-tissue arteriovenous malformations and assess their relationship to lesion size, location, tissue type involved and vascular architecture.Materials and methodsMedical records, MRI and histopathology were reviewed in consecutive patients with soft-tissue arteriovenous malformations in a multidisciplinary vascular anomalies clinic. Arteriovenous malformations were divided into those with and without atypical MRI findings (perilesional T2 signal abnormality, enhancement and/or a soft-tissue mass). Lesion location, size, tissue involved and vascular architecture were also compared between groups. Tissue stains were reviewed in available biopsy or resection specimens to assess relationships between MRI findings and histopathology.ResultsThirty patients with treatment-naïve arteriovenous malformations were included. Fifteen lesions demonstrated atypical MRI. There was no difference in age, gender, lesion size or involved body part between the groups. However, more than half of the atypical lesions demonstrated multicompartmental involvement, and tiny intralesional flow voids were more common in atypical arteriovenous malformations. Histopathology also differed in atypical cases, showing densely packed endothelial cells with connective tissue architectural distortion and edema.ConclusionArteriovenous malformations may exhibit features of a vascular tumor on MRI, particularly when multicompartmental and/or containing tiny internal vessels. These features are important to consider in suspected fast-flow vascular malformations and may have implications with respect to their treatment.

Homeobox (Hox) genes are transcriptional regulators which modulate embryonic morphogenesis and pathological tissue remodeling in adults via regulation of genes associated with cell-cell or cell extracellular matrix (ECM) interactions. We previously showed that while Hox 3 genes promote angiogenesis, Hox D10 inhibits this process.Here we show that another Hox family gene, Hox A5, also blocks angiogenesis but accomplishes this by targeting different downstream genes than Hox D10. Sustained expression of Hox A5 leads to down regulation of many pro-angiogenic genes including VEGFR2, ephrin A1, Hif1alpha and COX-2. In addition, Hox A5 also upregulates expression of anti-angiogenic genes including Thrombospondin-2. Furthermore, we show that while Hox A5 mRNA is expressed in quiescent endothelial cells (EC), its expression is diminished or absent in active angiogenic EC found in association with breast tumors or in proliferating infantile hemangiomas.Together our results suggest that restoring Hox A5 expression may provide a novel means to limit breast tumor growth or expansion of hemangiomas.

Background/Purpose: Neonates with large congenital diaphragmatic hernias (CDH) require prosthetic patch closure of the defect because of the paucity of native diaphragmatic tissue. As the child grows, patch separation can occur necessitating reoperation. Use of vascularized autologous tissue may decrease the incidence of reherniation as tissue incorporation and growth may be improved. The authors report our early experience using a local muscle advancement flap with microneural anastomosis for those children in whom reherniation develops after prosthetic patch placement. Methods: Seven patients with CDH (6 left and 1 right) whose synthetic diaphragmatic patch separated from the chest wall resulting in a clinically significant recurrent hernia were followed up with prospectively. After dissecting the ipsilateral latissimus dorsi off the chest wall and dividing the thoracodorsal neurovascular bundle (based on its lumbar blood supply), the synthetic patch was removed via an eighth intercostal incision. The muscle flap was placed into the hemithorax through the bed of the tenth rib and sutured in place over a Vicryl mesh scaffold. The thoracodorsal nerve was anastomosed to the phrenic nerve. Functional analysis of the flap was performed in 4 patients. Results: Age at placement of the muscle graft ranged from 2 months to 48 months (median, 24 months). There has been no evidence of reherniation after placement of the muscle graft. Long-term outcome and functional analysis of the flap was available in 4 patients (mean, 19 months). Two infants had fluoroscopic and sonographic evidence of nonparadoxical neodiaphragmatic motion. In one of these, electromyographic evidence of function was documented with a phrenic nerve conduction velocity of 22 meters per second. The third infant showed no evidence of neodiaphragmatic motion, and the fourth infant had paradoxical motion. Conclusions: This is the first direct documentation of phrenic nerve function in an infant with CDH. An innervated reversed latissimus dorsi (RLD) flap reconstruction for recurrent CDH provides an alternative to prosthetic patch repair. This technique offers the advantages of autologous vascularized tissue with potential phrenic nerve innervation and physiologic neodiaphragmatic motion. J Pediatr Surg 38:296-300. Copyright 2003, Elsevier Science (USA). All rights reserved.

Temporal hollowing is a disfiguring complication after a craniectomy operation resulting from temporalis muscle atrophy, contraction, and adhesion to the underlying dura. We describe a technique for temporalis muscle re-suspension utilizing multi-hole cranial plates and screws to recreate the superior temporal line on an otherwise smooth synthetic cranioplasty implant.

Venous malformations (VMs) are often painful and may enlarge over time. Chronic coagulopathy is common in VMs and may contribute to phleboliths and potentially to disease progression. Few studies have examined the effects of anticoagulation on VMs and to our knowledge none have examined the use of aspirin therapy. A survey was administered to patients and parents of patients with VMs who attended the University of California at San Francisco Vascular Anomalies Center over a 4-year period (2008-2012) to whom aspirin had been recommended. They were surveyed regarding whether they were taking aspirin and, if yes, whether aspirin had resulted in any appreciable benefit. Sixty-five letters were sent to potential subjects: 38 participated and 27 declined to participate or could not be contacted. Twenty-eight of the 38 had begun aspirin and 22 reported current use. Seventeen reported some benefit, including less aching (n = 2), less shooting pain (n = 15), less fullness and swelling (n = 13), and shrinking of the VM (n = 1). Discontinuation of aspirin was associated with worsening VM symptoms in five of six patients. Side effects were reported in 6 of 28 patients, including five episodes of minor bleeding or excessive bruising and one of nausea and vomiting. This study suggests that aspirin may be a beneficial treatment for VM, with a reduction in pain and soft tissue swelling and an acceptable side-effect profile, but the retrospective nature of the study and the small size of the cohort limited our conclusions. Larger prospective studies of aspirin for VM using clinical and laboratory outcome measures are needed to confirm these observations.

Background Reported 10-year patient survival after liver transplantation is nearing 70 percent, with similar trends seen for kidney transplantation. With increasing life expectancy, these patients have an increased need for posttransplant reconstructive surgery. Thus, plastic surgeons must know the effects of immunosuppressive medications on outcomes in microvascular reconstruction. Methods A retrospective review was performed on all immunosuppressed solid-organ transplant patients who underwent subsequent free tissue transfer of all types, over an 8-year period. Patient demographics, type of solid organ transplant, immunosuppressive regimen, anticoagulation regimen, associated medical comorbidities, and complication rates were analyzed. Results Twenty-four microvascular free tissue transfers were performed over an 8-year period on immunosuppressed patients, for head and neck and lower extremity reconstruction. Fifteen patients (63 percent) underwent prior kidney transplant, seven (29 percent) underwent prior liver transplant, and two (8 percent) underwent prior lung transplant. Prednisone (79 percent of patients), cyclosporine (33 percent), and tacrolimus (33 percent) were the most commonly used immunosuppressive medications. Complications included one total flap loss (4 percent), three intraoperative arterial thromboses (13 percent), and two postoperative venous thromboses (8 percent). Univariate analysis illustrated a statistically significant association between prednisone and overall complication rate (p = 0.032). Conclusions Microvascular free tissue transfer is a challenge in immunosuppressed patients. Univariate analysis showed prednisone to correlate statistically with operative morbidity. Transplant patients receiving prednisone at the time of their elective free flap procedure will require additional care to lower the additional risk of delayed wound healing, partial flap loss, and anastomotic thrombosis. Clinical question/level of evidence Therapeutic IV.

Regulatory T cells (Tregs), which are characterized by expression of the transcription factor Foxp3, are a dynamic and heterogeneous population of cells that control immune responses and prevent autoimmunity. We recently identified a subset of Tregs in murine skin with properties typical of memory cells and defined this population as memory Tregs (mTregs). Due to the importance of these cells in regulating tissue inflammation in mice, we analyzed this cell population in humans and found that almost all Tregs in normal skin had an activated memory phenotype. Compared with mTregs in peripheral blood, cutaneous mTregs had unique cell surface marker expression and cytokine production. In normal human skin, mTregs preferentially localized to hair follicles and were more abundant in skin with high hair density. Sequence comparison of TCRs from conventional memory T helper cells and mTregs isolated from skin revealed little homology between the two cell populations, suggesting that they recognize different antigens. Under steady-state conditions, mTregs were nonmigratory and relatively unresponsive; however, in inflamed skin from psoriasis patients, mTregs expanded, were highly proliferative, and produced low levels of IL-17. Taken together, these results identify a subset of Tregs that stably resides in human skin and suggest that these cells are qualitatively defective in inflammatory skin disease.

Patients with a giant congenital melanocytic nevus can develop melanotic tumors characterized by central nervous system involvement, termed leptomeningeal melanocytosis or neurocutaneous melanosis. Although symptomatic neurocutaneous melanosis is rare, we previously reported distinct magnetic resonance (MR) findings of T1 shortening, strongly suggestive of neurocutaneous melanosis, in 30 percent (6 of 20) of children with giant congenital melanocytic nevi who presented initially without neurological symptoms. The purpose of this study was to determine the incidence of neurocutaneous melanosis in high-risk patients and its long-term clinical significance. Magnetic resonance imaging was recommended for all 46 patients with "at-risk" giant congenital melanocytic nevi involving the skin overlying the dorsal spine or scalp. The clinical histories and follow-up of these patients were evaluated by retrospective chart review. Forty-two underwent MR imaging of the brain and 11 underwent additional MR scanning of the spinal cord. Abnormalities were identified in 14 of 43 MR studies, and 23 percent (n = 10) had T1 shortening indicative of melanotic rests within the brain or meninges. None had associated masses or leptomeningeal thickening. The most common areas of involvement in these 10 included the amygdala (n = 8), cerebellum (n = 5), and pons (n = 3). In the group of 11 patients with spinal MR scans, a tethered spinal cord was demonstrated in one. Additional abnormalities were detected by MR scanning, including a middle cranial fossa arachnoid cyst, a Chiari type I malformation, and a crescentic enhancement that subsequently resolved. Clinical follow-up averaging 5 years (range, 2 to 8 years) revealed that only one of the 46 patients evaluated developed neurological symptoms, manifested as developmental delay, hypotonia, and questionable seizures but no other signs of neurocutaneous melanosis. No patient has developed a cutaneous or central nervous system melanoma. Magnetic resonance findings of neurocutaneous melanosis are relatively common, even in asymptomatic children with giant congenital melanocytic nevi. Although these findings suggest an increased lifetime risk of central nervous system melanoma, they do not signify the eventual development of symptomatic neurocutaneous melanosis during childhood.

Objective To analyze a series of patients treated for recurrent or chronic abdominal wall hernias and determine a treatment protocol for defect reconstruction. Background Data Complex or recurrent abdominal wall defects may be the result of a failed prior attempt at closure, trauma, infection, radiation necrosis, or tumor resection. The use of prosthetic mesh as a fascial substitute or reinforcement has been widely reported. In wounds with unstable soft tissue coverage, however, the use of prosthetic mesh poses an increased risk for extrusion or infection, and vascularized autogenous tissue may be required to achieve herniorrhaphy and stable coverage. Methods Patients undergoing abdominal wall reconstruction for 106 recurrent or complex defects (104 patients) were retrospectively analyzed. For each patient, hernia etiology, size and location, average time present, technique of reconstruction, and postoperative results, including recurrence and complication rates, were reviewed. Patients were divided into two groups based on defect components: Type I defects with intact or stable skin coverage over hernia defect, and Type II defects with unstable or absent skin coverage over hernia defect. The defects were also assigned to one of the following zones based on primary defect location to assist in the selection and evaluation of their treatment: Zone 1A, upper midline; Zone IB, lower midline; Zone 2, upper quadrant; Zone 3, lower quadrant. Results A majority of the defects (68%) were incisional hernias. Of 50 Type I defects, 10 (20%) were repaired directly, 28 (56%) were repaired with mesh only, and 12 (24%) required flap reconstruction. For the 56 Type II defects reconstructed, flaps were used in the majority of patients (n = 48; 80%). The overall complication and recurrence rates for the series were 29% and 8%, respectively. Conclusions For Type I hernias with stable skin coverage, intraperitoneal placement of Prolene mesh is preferred, and has not been associated with visceral complications or failure of hernia repair. For Type II defects, the use of flaps is advisable, with tensor fascia lata representing the flap of choice, particularly in the lower abdomen. Rectus advancement procedures may be used for well-selected midline defects of either type. The concept of tissue expansion to increase both the fascial dimensions of the flap and zones safely reached by flap transposition is introduced. Overall failure is often is due to primary closure under tension, extraperitoneal placement of mesh, flap use for inappropriate zone, or technical error in flap use. With use of the proposed algorithm based on defect analysis and location, abdominal wall reconstruction has been achieved in 92% of patients with complex abdominal defects.

The availability of multiple reconstructive options is often a factor in the decision to undertake complex cranial base resections. Flaps provide an adequate seal between the dura and the paranasal sinuses and may also greatly enhance the postoperative appearance of the patient. This article reviews the wide array of alternatives for cranial base reconstruction, from local pericranial and galeal flaps through regional musculocutaneous flaps to microvascular flap reconstructions. The use of cranial bone grafts and the planning of incisions to allow for appropriate reconstruction is discussed.

The prospect of fetal surgery for cleft lip is predicated on our ability to accurately identify fetuses with clefts and exclude those that have associated anomalies. Prenatal ultrasound is currently the most appropriate means with which to do this. We reviewed the ultrasonographic data from two large perinatal referral institutions to determine the natural history of fetuses with cleft lip who may be candidates for fetal surgery. Forty fetuses had a cleft lip diagnosed prenatally by ultrasound. In this group, severe associated anomalies were common (30 of the 40) and multiple (23 of the 40) in a majority of fetuses. Life-threatening anomalies, such as central nervous system and cardiac anomalies, were the most common defects. As a result, many fetuses were aborted therapeutically or died in the perinatal period. Out of 12 surviving fetuses, only six had isolated clefts, and two surviving fetuses, diagnosed with isolated cleft lip, had no defect identified postnatally. This information has important implications for the perinatal management of fetuses with cleft lip and the potential role of fetal intervention.