Perioperative risk factors in patients with 22q11.2 deletion syndrome requiring surgery for velopharyngeal dysfunction.

Objective : To determine the prevalence of cardiac, cervical spine, and carotid artery abnormalities in patients with 22q11.2 deletion syndrome (22q11.2DS) undergoing surgery for velopharyngeal dysfunction (VPD), associations between the presence of these abnormalities, and whether these abnormalities caused changes in surgical management or perioperative complications. Design : Retrospective review. Setting : Tertiary pediatric hospital. Patients : Seventy patients with 22q11.2DS with complete preoperative cervical vascular and spine imaging and cardiac evaluation between 1998 and 2011. Main Outcome Measures : Incidence of cardiac, cervical spine, and vascular abnormalities; related perioperative complications; and resulting changes in surgical, anesthetic, or perioperative management plan. Results : Cardiac abnormalities occurred in 45 patients (64.3%), and 8 patients required cardiac anesthesia. Thirty-eight patients (54.3%) had at least one vascular abnormality of the neck, and 14% had medial deviation of the internal carotid artery. Surgery was not performed in one patient, and the surgical plan was altered in three patients because of carotid anomalies. Cervical spine abnormalities were found in 24 patients (34.3%); 8 patients demonstrated radiographic evidence of cervical instability and were treated with spinal precautions during surgery. The presence of one anomaly was not predictive of any other finding, and there were no complications related to the heart, cervical spine, or carotid arteries. Conclusions : Anomalies of the heart, cervical spine, and cervical vasculature occur frequently in 22q11.2DS, vary drastically in severity, and are impossible to predict based on other features of the syndrome. Preoperative diagnosis of these comorbidities with routine imaging can minimize the risk of avoidable surgical complications.

Long term speech outcomes following late cleft palate repair using the modified Furlow technique.

OBJECTIVES: Published reports and previous studies from our institution have reported worse overall speech results, including significantly higher rates of persistent articulation errors, in patients undergoing palatoplasty at age >18 months. This study further investigates the effects of late repair on long term speech outcomes. METHODS: A retrospective review was performed of non-syndromic patients undergoing primary palatoplasty at age >18 months between 1980 and 2006 at our institution. Longitudinal speech results were compared based on reason for late repair and age at repair. RESULTS: Forty-one patients were greater than 18 months of age at the time of palatoplasty, and 24 fit criteria for longitudinal data analysis. There was a statistically significant improvement in nasality scores at Time Point 1 for international adoptees compared to the non-adopted population (p=0.04). Patients with submucosal clefts were found to have significantly less severe nasal emission scores at Time Point 1 compared to those with overt clefts (p=0.04), but not at Time Point 2. There were no significant differences between scores if repair was performed between 18 and 36 months or >36 months, nor any difference in incidence of articulation errors between subgroups of patients with late repair at either Time Point. CONCLUSION: Our experience demonstrates that cleft palate repair after 18 months of age is associated with a significantly increased incidence of articulation errors associated with VPI, irrespective of reason for late repair, highlighting the persistence of learned compensatory behaviors in speech and the importance of proceeding with early repair.

Reexamining free flap breast reconstruction in the community and university setting: is there a difference?

BACKGROUND: This study aimed to compare free flap breast reconstruction outcomes in community and university settings to determine whether the latter is necessary for successful performance of this complex procedure. METHODS: Free tissue transfer procedures for breast reconstruction from 1 university and 1 community hospital performed between 2005 and 2011 were included. Procedures were performed by the same 2 surgeons at both institutions. Demographics and outcome measures were evaluated. RESULTS: Of the 1293 free tissue breast reconstructions performed, 99 (7.7%) were performed in a community hospital and 1194 (92.3%) were performed in a university center. No differences were noted in demographics, comorbidities, or type of free flap reconstruction. However, a number of perioperative characteristics differed. In the community setting, operative time was less (7.3 vs 8.3 hours, P < 0.0001), estimated blood loss was higher (330 vs 248 mL, P < 0.0001), and blood transfusions were more prevalent (24.6% vs 8.3%, P < 0.0001). Furthermore, no significant differences were noted in overall postoperative complications, although a higher rate of abdominal wound infections was noted in the community setting (7.2% vs 2.6%, P = 0.03). The mean number of hospital days was similar between the community and the university (should include value P = 0.44). CONCLUSIONS: Although slight differences were noted in a number of perioperative variables and wound complications, we conclude that the key to a successful free tissue transfer reconstruction is in the expertise of the surgeon and not the setting in which it is performed. Despite its complexity, free flap breast reconstruction can be safely and successfully performed in the community setting.

A 35-year experience with syndromic cleft palate repair: operative outcomes and long-term speech function.

BACKGROUND: Associated comorbidities can put syndromic patients with cleft palate at risk for poor speech outcomes. Reported rates of velopharyngeal insufficiency (VPI) vary from 8% to 64%, and need for secondary VPI surgery from 23% to 64%, with few studies providing long-term follow-up. The purpose of this study was to describe our institutional long-term experience with syndromic patients undergoing cleft palatoplasty. METHODS: A retrospective review was conducted of all patients with syndromic diagnoses undergoing primary Furlow palatoplasty from 1975 to 2011. Outcomes included postoperative oronasal fistula (ONF) and need for secondary VPI surgery. Speech scores for verbal patients 5 years or older were collected via the Pittsburgh scale for speech assessment. Aggregate scores categorized the velopharyngeal mechanism as competent, borderline, or incompetent. Outcomes were analyzed by patient and operative factors. RESULTS: One hundred thirty-two patients were included with average age at repair of 20.7 months. Cleft type was 9% submucosal, 16% Veau class I, 50% class II, 12% class III, and 13% class IV. Forty-five syndromes were recorded, most commonly Stickler syndrome (n = 32) and 22q11.2 deletion syndrome [22q11.2DS (n = 19)]. Forty-four patients also had associated Pierre Robin sequence (PRS). The overall ONF rate was 4.5% and was highest in Veau class IV clefts (P = 0.048). Seventy-six patients were included in speech analysis, with an average age at last assessment of 10.4 years. Overall, 60.5% of patients had a competent velopharyngeal mechanism, 23.7% borderline, and 15.8% incompetent mechanism. Fifty percent of 22q11.2DS patients had borderline speech and none had competent speech, compared to 73.3% with Stickler syndrome (P = 0.01) and 71.4% of patients with associated PRS (P = 0.02). Secondary VPI surgery was performed in 11.4% of patients overall. Patients with PRS (13.6%) and with Stickler syndrome (15.6%) had secondary VPI surgery, compared to 31.6% of patients with 22q11.2DS (P = 0.01). CONCLUSIONS: This study demonstrates low rates of postoperative ONF after modified Furlow palatoplasty in syndromic patients. Speech outcomes were comparable to nonsyndromic patients at our institution, but patients with 22q11.2DS consistently had borderline-incompetent speech and a 3-fold higher incidence of secondary VPI surgery.

Transfusions in autologous breast reconstructions: an analysis of risk factors, complications, and cost.

PURPOSE: Free tissue transfer requires lengthy operative times and can be associated with significant blood loss. The goal of our study was to determine independent risk factors for blood transfusions and transfusion-related complications and costs. METHODS: We reviewed our prospectively maintained free flap database and identified all patients undergoing breast reconstruction receiving blood transfusions. These patients were compared with those not receiving a postoperative transfusion. We examined baseline patient comorbidities, preoperative and postoperative hemoglobin (HgB) levels, intraoperative and postoperative complications, and blood transfusions. Factors associated with transfusion were identified using univariate analyses, and multivariate logistic regression was used to determine independently associated factors. RESULTS: A total of 70 (8.2%) patients received postoperative blood transfusions. Multivariate analysis revealed associations between length of surgery (P=0.01), intraoperative arterial thrombosis [odds ratio (OR), 6.75; P=0.01], major surgical complications (OR, 25.9; P<0.001), medical complications (OR, 7.2; P=0.002), and postoperative HgB levels (OR, 0.2; P<0.001). Transfusions were independently associated with higher rates of medical complications (OR, 2.7; P=0.03). A significantly lower rate of medical complications was observed when a restrictive transfusion (HgB level, <7 g/dL) was administered (P=0.04). A cost analysis demonstrated that each blood transfusion was independently associated with an added $1,500 in total cost. CONCLUSIONS: Several key perioperative factors are associated with allogenic transfusion, including intraoperative complications, operative time, HgB level, and postoperative medical and surgical complications. Blood transfusions were independently associated with greater morbidity and added hospital costs. Overall, a restrictive transfusion strategy (HgB level, <7 g/dL or clinically symptomatic) may help minimize medical complications. LEVEL OF EVIDENCE: Prognostic/risk category, level III.

Do patients with Pierre Robin sequence have worse outcomes after cleft palate surgery?

BACKGROUND: Pierre Robin sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, airway obstruction, and often clefting of the soft and hard palate. The purpose of this study was to compare oronasal fistula rates, long-term speech outcomes, and rates of secondary surgery for velopharyngeal insufficiency (VPI) after primary cleft palate repair using the modified Furlow technique between nonsyndromic patients with and without PRS. This study also sought to determine if type of airway management in infancy correlated with speech outcomes in patients with PRS. METHODS: A retrospective review was performed of all nonsyndromic patients with clefts of the palate only who underwent repair between 1981 and 2006 at The Children's Hospital of Philadelphia using the modified Furlow technique. Patient outcomes were evaluated by the rate of postoperative oronasal fistula, speech scores at a minimum of age 5 years using the Pittsburgh Weighted Values for Speech Symptoms Associated with VPI, and the need for secondary pharyngeal surgery for VPI. RESULTS: Fifty-five patients with PRS and 129 without PRS were included in this study. There was no significant difference in oronasal fistula rate between groups. Speech outcomes were worse in patients with PRS with 52.7% demonstrating a competent velopharyngeal mechanism, and 30.9% a borderline mechanism, compared to 72.1% and 19.4% in the non-PRS group, respectively (P = 0.035). In addition, only 76.3% of patients with PRS had no or mild hypernasality compared to 91.5% of patients without PRS (P = 0.01). The rates of nasal emission, articulation errors associated with VPI, and secondary surgery for VPI did not differ between groups. Of the patients with PRS, 36 were managed in infancy with positioning alone and 15 underwent surgical intervention for airway obstruction, and there were no significant differences in speech scores or rates of secondary surgery for VPI between these subgroups. CONCLUSIONS: Nonsyndromic patients with PRS had worse speech outcomes after modified Furlow cleft palate repair, but no significant differences were seen in the rates of secondary surgery for VPI or postoperative oronasal fistula. In addition, initial airway management in patients with PRS did not correlate with speech outcomes.

The Children's Hospital of Philadelphia modification of the Furlow double-opposing Z-palatoplasty: 30-year experience and long-term speech outcomes.

BACKGROUND: The purpose of this study was to evaluate the long-term outcomes following modified Furlow palatoplasty over a 30-year experience at a single institution. METHODS: A retrospective review was performed of all nonsyndromic patients undergoing primary modified Furlow palatoplasty at The Children's Hospital of Philadelphia between 1980 and 2011. Surgical success was evaluated by the rate of postoperative oronasal fistula, speech outcomes assessed at a minimum of age 5 years, and the need for secondary surgery for velopharyngeal dysfunction. Results were analyzed by surgeon, Veau cleft type, age at repair, and over time. RESULTS: Eight hundred sixty-nine patients who underwent modified Furlow repair were included in this study. The mean age at palatoplasty was 1.1 years, and the mean length of follow-up was 6.7 years. Repairs were performed by 11 surgeons, with 44.3 percent performed by a single surgeon. The overall oronasal fistula rate was 5.2 percent, and this improved significantly over the 30-year period (p = 0.003). Speech results at age 5 or older were available for 559 patients. Of these 559 patients, 72.4 percent had a competent velopharyngeal mechanism, and 21.5 percent had a borderline mechanism consistent with socially acceptable speech. Secondary pharyngeal surgery was indicated in 8.1 percent of patients. Speech results were stable (p = 0.10), and rates of secondary surgery for velopharyngeal dysfunction declined significantly over the study period (p = 0.005). CONCLUSIONS: The authors' 30-year experience with the modified Furlow palatoplasty shows stable speech results, decreasing rates of secondary surgery, and an acceptable rate of postoperative oronasal fistula.

Fat grafting's past, present, and future: why adipose tissue is emerging as a critical link to the advancement of regenerative medicine.

Fat grafting is a common reconstructive and aesthetic procedure with extensive clinical applications. Recently, significant strides have been made in investigating the biology behind the success of this procedure. Surgeons and scientists alike have advanced this field by innovating fat graft harvesting and injection techniques, expanding the use of adipose tissue and its stem cell components, and broadening our understanding of the viability of fat grafting at the molecular and cellular levels. The objectives of this review are to (1) discuss the clinical applications of fat grafting, (2) describe the cellular biology of fat and the optimization of fat graft preparation, (3) illustrate the significance of adipose-derived stem cells and the potentiality of fat cells, (4) highlight the clinical uses of adipose-derived stem cells, and (5) explore the current and future frontiers of the study of fat grafting. Although collaborative knowledge has increased exponentially, many of the biological mechanisms behind fat grafting are still unknown. Plastic surgeons are in a unique position to pioneer both the scientific and clinical frontiers of fat grafting and to ultimately further this technology for the benefit of our patients.

Beyond fat grafting: what adipose tissue can teach us about the molecular mechanisms of human aging.

BACKGROUND: The concept of aging and the mechanisms responsible for soft tissue aging have become progressively more important as the world's population ages and demands a higher quality of life. Although molecular mechanisms of aging have been evaluated in model organisms, specific genomic, genetic, and epigenetic modifications that can be translated to normal human tissue aging have yet to be identified. We propose that adipose tissue is an excellent model with which to investigate molecular aging pathways. The goal of this study is to demonstrate that primary human adipose tissue can serve as a model of human aging, and further, can be used to detect differences in genomic transcriptional profiling between cell types in adipose tissue as well as between youthful and older age groups. METHODS: Subcutaneous adipose tissue was excised during cosmetic procedures from healthy patients. Adipocytes and stromal vascular fractions from the anterior abdomen were isolated from 3 young (26-39 years) and 3 old (52-64 years) patients and analyzed for genome-wide transcriptional differences between varying ages and cell types using the Affymetrix GeneChip Human Gene Chip 1.0ST. RESULTS: Genes specific to adipocytes were more highly expressed in adipocytes than in stromal vascular fractions, validating that adipose tissue should be examined in a cell-specific manner. An increase in overall gene expression was observed among patients in the older age group, consistent with senescence-related chromatin dysregulation. Principal components analysis revealed no clear delineation between age groups and a clear separation by cell type. Analysis of variance revealed cell type as the most significant variable in transcriptional differences, whereas age-related differences were a distant second. Gene Ontology categories of the most significantly modified genes included RNA splicing and mRNA metabolism, plasma membrane, and mitochondrial metabolism. CONCLUSIONS: Primary adipose tissue is an effective model for the study of the molecular mechanisms of human aging. Our findings are consistent with the hypothesis that epigenetic modifications play a more important role than transcriptional modifications in early human adipose tissue aging. Our future studies will examine the contribution of specific epigenetic markers to human adipose tissue aging and promise to advance approaches in regenerative medicine, and the prevention and treatment of aging.

Preoperative FDG-PET/CT Is an Important Tool in the Management of Patients with Thick (T4) Melanoma.

The yield of preoperative PET/CT (PET/CT) for regional and distant metastases for thin/intermediate thickness melanoma is low. Objective of this study is to determine if PET/CT performed for T4 melanomas helps guide management and alter treatment plans. Methods. Retrospective cohort of 216 patients with T4 melanomas treated at two tertiary institutions. Fifty-six patients met our inclusion criteria (T4 lesion, PET/CT and no clinical evidence of metastatic disease). Results. Fifty-six patients (M: 32, F: 24) with median tumor thickness of 6 mm were identified. PET/CT recognized twelve with regional and four patients with metastatic disease. Melanoma-related treatment plan was altered in 11% of the cases based on PET/CT findings. PET/CT was negative 60% of the time, in 35% of the cases; it identified incidental findings that required further evaluation. Conclusion. Patients with T4 lesions, PET/CT changed the treatment plan 18% of the time. Regional findings changed the surgical treatment plan in 11% and the adjuvant plan in 7% of our cases due to the finding of metastatic disease. Additionally 20 patients had incidental findings that required further workup. In this subset of patients, we feel there is a benefit to PET/CT, and further studies should be performed to validate our findings.