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1.
Cleft Palate Craniofac J ; : 10556656241277694, 2024 Sep 09.
Artigo em Inglês | MEDLINE | ID: mdl-39252504

RESUMO

OBJECTIVE: To identify associations between scores on the CLEFT-Q and Columbia-Suicide Severity Rating Scale (C-SSRS) Lifetime Version in patients with cleft lip and/or palate (CLP). DESIGN: Prospective. SETTING: Tertiary care center. PATIENTS, PARTICIPANTS: Patients ages six and older administered both the CLEFT-Q questionnaire and C-SSRS survey between 2019 and 2024. INTERVENTIONS: Multidisciplinary care coordination facilitated by the team psychologist. MAIN OUTCOME MEASURE(S): Associations among demographics, CLEFT-Q responses, and suicidality. RESULTS: A total of 305 patients were included, 141 females (46.2%) and 164 males (53.8%). Fifty-one (16.7%) endorsed lifetime incidence of suicidal ideation, four (1.3%) endorsed suicidal behavior, 12 (3.9%) endorsed non-suicidal self-injury (NSSI), and one (0.3%) endorsed self-injurious behavior, intent unknown. Patients endorsing suicidal ideation had lower PROs in 12/13 categories on the CLEFT-Q questionnaire (p < 0.001). Those with suicidal behavior had lower PROs in three health-related quality of life categories (psychological function, p = 0.018; social function, p = 0.005; school function, p = 0.007), but no difference in other domains. A cutoff of ≤70 in the CLEFT-Q psychological function domain identified suicidal ideation with 72.9% sensitivity and 65.9% specificity and suicidal behavior with 100.0% sensitivity and 62.2% specificity. CONCLUSIONS: Patients with cleft lip and/or palate have increased risks for psychosocial challenges that are often missed by healthcare providers. This study reveals that patient-reported outcomes are worse in those with CLP who endorsed suicidal ideation and behavior. Low PRO responses identify suicidality with moderate sensitivity and specificity. Patients with low scores should be offered safety screenings and psychosocial support, ideally by mental healthcare professionals.

2.
Cleft Palate Craniofac J ; : 10556656241272736, 2024 Sep 11.
Artigo em Inglês | MEDLINE | ID: mdl-39262157

RESUMO

OBJECTIVE: To examine the impact of primary rhinoplasty on subsequent rhinoplasties for patients with cleft lip nasal deformity. DESIGN: Retrospective cohort study. SETTING: Tertiary care pediatric hospital. PATIENTS/PARTICIPANTS: Individuals who underwent definitive cleft lip repair at our institution from 2000 to 2006 with a current age of 18 or older. MAIN OUTCOME MEASURES: Number and timing of subsequent rhinoplasties. RESULTS: Among 199 individuals, 94 (47.2%) underwent primary rhinoplasty. Follow-up was 15.0 ± 4.8 years in the PR cohort and 15.0 ± 5.1 years in the NPR cohort (p = 0.993). In bilateral cleft lip, interdomal suture predicted fewer subsequent rhinoplasties (ß=-0.310, p = 0.033), while history of primary rhinoplasty predicted greater age at subsequent rhinoplasty (ß=1.800, p = 0.040). Among individuals with follow-up beyond age 18, intranasal stenting predicted fewer subsequent rhinoplasties (ß=-0.609, p = 0.015). Most underwent subsequent nasal correction aside from 7 (19.4%) and 9 (20.9%) in the PR and NPR cohorts, respectively (p = 0.536). There was no difference in mean subsequent rhinoplasties between cohorts (1.1 ± 0.8 versus 1.3 ± 1.1, p = 0.284). Individuals with complete cleft lip underwent more lifetime rhinoplasties (1.9 ± 1.0 versus 1.2 ± 1.2, p = 0.007). CONCLUSIONS: Primary rhinoplasty with interdomal tip sutures in bilateral cleft lip was associated with fewer subsequent rhinoplasties. Primary rhinoplasty may delay subsequent nasal correction, though most who underwent primary rhinoplasty ultimately required nasal correction later in childhood. Postoperative nasal stenting may provide longer-term nasal benefits and should be considered at time of definitive cleft lip repair.

3.
Cleft Palate Craniofac J ; : 10556656241236369, 2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38436069

RESUMO

OBJECTIVE: To describe how the psychosocial status of patients with cleft lip and/or palate (CL/P) relates to patient-reported outcomes (PROs). DESIGN: Cross-sectional retrospective chart review. SETTING: Tertiary care pediatric hospital. PATIENTS/PARTICIPANTS: Patients aged 8 to 29 years attending cleft team evaluations during a 1-year period. MAIN OUTCOME MEASURES: CLEFT-Q. RESULTS: Patients (N = 158) with isolated or syndromic CL/P and mean age 13.4 ± 3.0 years were included. Fifteen (9%) patients had siblings who also had CL/P. Of 104 patients who met with the team psychologist, psychosocial concerns were identified in 49 (47%) patients, including 25 (24%) with Attention-Deficit/Hyperactivity Disorder or behavior concerns, 28 (27%) with anxiety, and 14 (13%) with depression or mood concerns. Younger age and having siblings with cleft were associated with better PROs, while psychosocial concerns were associated with worse PROs on Speech, Psychosocial, and Face Appearance scales. CONCLUSIONS: Patient perception of cleft outcomes is linked to psychosocial factors.

4.
Genet Med ; 25(3): 100338, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36729053

RESUMO

This review aimed to update the clinical practice guidelines for managing children and adolescents with 22q11.2 deletion syndrome (22q11.2DS). The 22q11.2 Society, the international scientific organization studying chromosome 22q11.2 differences and related conditions, recruited expert clinicians worldwide to revise the original 2011 pediatric clinical practice guidelines in a stepwise process: (1) a systematic literature search (1992-2021), (2) study selection and data extraction by clinical experts from 9 different countries, covering 24 subspecialties, and (3) creation of a draft consensus document based on the literature and expert opinion, which was further shaped by survey results from family support organizations regarding perceived needs. Of 2441 22q11.2DS-relevant publications initially identified, 2344 received full-text reviews, including 1545 meeting criteria for potential relevance to clinical care of children and adolescents. Informed by the available literature, recommendations were formulated. Given evidence base limitations, multidisciplinary recommendations represent consensus statements of good practice for this evolving field. These recommendations provide contemporary guidance for evaluation, surveillance, and management of the many 22q11.2DS-associated physical, cognitive, behavioral, and psychiatric morbidities while addressing important genetic counseling and psychosocial issues.


Assuntos
Síndrome de DiGeorge , Adolescente , Humanos , Criança , Síndrome de DiGeorge/genética , Síndrome de DiGeorge/terapia , Aconselhamento Genético , Inquéritos e Questionários
5.
Vascular ; : 17085381231164015, 2023 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-36917226

RESUMO

BACKGROUND: Ocular Ischemic Syndrome (OIS) is a rare, vision threatening condition associated with severe carotid artery disease. There are few cases of OIS reported in the literature. METHODS: We present the case of a 54-year-old male with history of multiple previous carotid interventions including a right carotid stent, who presented with right-sided OIS. RESULTS: CTA and angiogram showed a severe calcific plaque causing restenosis of the right carotid stent, with a patent right internal carotid artery (ICA) in the very distal neck. The right common carotid artery (CCA) was patent but diseased with ulcerated plaque extending proximally to below the level of the clavicle. The left CCA was chronically occluded from its origin all the way to the bifurcation. Given our patient's surgical history, the imperative to revascularize the ipsilateral carotid, and a diffusely diseased ipsilateral CCA, he was successfully treated with an ipsilateral subclavian to internal carotid bypass. CONCLUSION: There is paucity of data regarding the best approach for carotid revascularization in OIS. This case report discusses our unique perioperative decision making as well as relevant literature.

6.
J Craniofac Surg ; 34(7): 1978-1984, 2023 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-37449578

RESUMO

Orofacial clefts (OFC) remain among the most prevalent congenital abnormalities worldwide. In the United States in 2010 to 2014, 16.2 of 10,000 live births are born with OFC compared with 23.6 of 10,000 in Alta Verapaz, Guatemala in 2012. Demographics and cleft severity scores were retrospectively gathered from 514 patients with isolated OFC at the Children's Hospital of Philadelphia scheduled for surgery from 2012 to 2019 and from 115 patients seen during surgical mission trips to Guatemala City from 2017 to 2020. Risk factors were also gathered prospectively from Guatemalan families. The Guatemalan cohort had a significantly lower prevalence of cleft palate only compared with the US cohort, which may be a result of greater cleft severity in the population or poor screening and subsequent increased mortality of untreated cleft palate. Of those with lip involvement, Guatemalan patients were significantly more likely to have complete cleft lip, associated cleft palate, and right-sided and bilateral clefts, demonstrating an increased severity of Guatemalan cleft phenotype. Primary palate and lip repair for the Guatemalan cohort occurred at a significantly older age than that of the US cohort, placing Guatemalan patients at increased risk for long-term complications such as communication difficulties. Potential OFC risk factors identified in the Guatemalan cohort included maternal cooking-fire and agricultural chemical exposure, poor prenatal vitamin intake, poverty, and risk factors related to primarily corn-based diets. OFC patients who primarily rely on surgical missions for cleft care would likely benefit from more comprehensive screening and investigation into risk factors for more severe OFC phenotypes.

7.
Cleft Palate Craniofac J ; : 10556656231198647, 2023 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-37649261

RESUMO

OBJECTIVE: To compare patient-reported outcomes (PROs) in internationally adopted patients with cleft lip and palate to those in non-adopted peers. DESIGN: Cross-sectional study. SETTING: Multidisciplinary cleft team at tertiary care hospital. PATIENTS: Patients aged ≥ 8 with cleft lip and palate attending routine cleft team evaluations September 2021 - September 2022. MAIN OUTCOME MEASURE: CLEFT-Q PRO scores. RESULTS: Sixty-four internationally adopted patients and 113 non-adopted patients with a mean age of 13 years were included. Compared to non-adopted peers, adopted patients demonstrated worse satisfaction with face appearance (mean 59 vs. 66, p = .044), speech function (mean 69 vs. 78, p = .005), and speech distress (mean 80 vs. 84, p = .032). No significant differences were observed on the nose, nostrils, teeth, lips, lip scar, jaws, psychological function, or social function scales (p > .05). Objective clinical evaluation corroborated these findings, with adopted patients demonstrating worse Pittsburgh Weighted Speech scores (mean 3.0 vs 1.9, p = .027) and greater incidence of articulation errors (64% vs 46%, p = .021). No significant differences were observed in rates of mood, anxiety, or behavior concerns identified on psychosocial assessment (p = .764). Among adopted patients, undergoing palatoplasty prior to adoption was associated with worse satisfaction with speech, appearance, school, and social function (p < .05). CONCLUSIONS: Patient-reported outcomes among internationally adopted adolescents and young adults with cleft lip and palate show slightly lower satisfaction with facial appearance and speech but otherwise demonstrate similar results to non-adopted peers on most appearance and psychosocial measures. PRO data correlated well with objective speech assessment and did not portend worse psychosocial function.

8.
Cleft Palate Craniofac J ; : 10556656231154808, 2023 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-36749038

RESUMO

OBJECTIVE: Approximately 30% of patients with a history of repaired cleft palate (CP) go on to suffer from velopharyngeal dysfunction (VPD). This study discusses the operative management of VPD and postoperative speech outcomes in a cohort of CP patients. SETTING: An academic tertiary pediatric care center. METHODS: Retrospective cohort study. PATIENTS: Patients with history of repaired CP (Veau I-IV) who underwent operative management of VPD between January 1st, 2010 and December 31st, 2020. Operative modalities were posterior pharyngeal flap (PPF), sphincter pharyngoplasty (SPP), Furlow palate re-repair, and buccal myomucosal flap palate lengthening (PL). OUTCOME MEASURES: The primary outcome measure is postoperative speech improvement evaluated by the Pittsburgh Weighted Speech Scale (PWSS). RESULTS: 97 patients met inclusion criteria. 38 patients with previous straight-line primary palatoplasty underwent Furlow re-repair; these patients were significantly younger (7.62 vs 11.14, P < .001) and were more likely to have severe VPD per PWSS (OR 4.28, P < .01, 95% CI 1.46-12.56) when compared to VPD patients with previous Furlow repair. 21.1% of these patients required an additional non-revisional VPD procedure. The remaining patients underwent a non-revision procedure (26 PPF, 22 SPP, 11 PL); all experienced significant (P < .001 on paired t-test) reductions in PWSS total and subgroup VPD severity scores without difference in improvement between operation types. SPP was statistically associated with all-cause complication (OR 2.79, 95% CI 1.03-7.59, P < .05) and hyponasality (OR 3.27, 95% CI 1.112-9.630, P < .05). CONCLUSION: Furlow re-repair reduced need for additional VPD operations. Speech outcomes between non-revisional operations are comparable, but increased complications were seen in SPP.

9.
Cleft Palate Craniofac J ; : 10556656231181359, 2023 Jul 13.
Artigo em Inglês | MEDLINE | ID: mdl-37441787

RESUMO

OBJECTIVE: Surgical treatment of velopharyngeal insufficiency (VPI) includes a wide array of procedures. The purpose of this study was to develop a classification for VPI procedures and to describe variations in how they are performed.Design/participants/setting/outcomes: We completed an in-depth review of the literature to develop a preliminary schema that encompassed existing VPI procedures. Forty-one cleft surgeons from twelve hospitals across the USA and Canada reviewed the schema and either confirmed that it encompassed all VPI procedures they performed or requested additions. Two surgeons then observed the conduct of the procedures by surgeons at each hospital. Standardized reports were completed with each visit to further explore the literature, refine the schema, and delineate the common and unique aspects of each surgeon's technique. RESULTS: Procedures were divided into three groups: palate-based surgery; pharynx-based surgery; and augmentation. Palate-based operations included straight line mucosal incision with intravelar veloplasty, double-opposing Z-plasty, and palate lengthening with buccal myomucosal flaps. Many surgeons blended maneuvers from these three techniques, so a more descriptive schema was developed classifying the maneuvers employed on the oral mucosa, nasal mucosa, and muscle. Pharynx-based surgery included pharyngeal flap and sphincter pharyngoplasty, with variations in design for each. Augmentation procedures included palate and posterior wall augmentation. CONCLUSIONS: A comprehensive schema for VPI procedures was developed incorporating intentional adaptations in technique. There was substantial variation amongst surgeons in how each procedure was performed. The schema may enable more specific evaluations of surgical outcomes and exploration of the mechanisms through which these procedures improve speech.

10.
Cleft Palate Craniofac J ; 60(8): 1010-1020, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-35711155

RESUMO

OBJECTIVE: To determine the impact of illustrated postoperative instructions on patient-caregiver knowledge and retention. DESIGN: Prospective study with all participants receiving an educational intervention. SETTING: Pediatric plastic surgical missions in Guatemala City, Guatemala, between 2019 and 2020. PARTICIPANTS: A total of 63 majority-indigenous Guatemalan caregivers of patients receiving cleft lip and/or palate surgery. INTERVENTION: Illustrated culturally appropriate postoperative care instructions were iteratively developed and given to caregivers who were surveyed on illustration-based and text-based information at preoperative, postoperative, and four-week follow-up time points. MAIN OUTCOME MEASURE: Postoperative care knowledge of illustration-based versus text-based information as determined by the ability to answer 11 illustration- and 8 text-based all-or-nothing questions, as well as retention of knowledge as determined by the same survey given at four weeks follow-up. RESULTS: Scores for illustration-based and text-based information both significantly increased after caregivers received the postoperative instructions (+13.30 ± 3.78 % SE, + 11.26 ± 4.81 % SE; P < .05). At follow-up, scores were unchanged for illustration-based (-3.42 ± 4.49 % SE, P > .05), but significantly lower for text-based information (-28.46 ± 6.09 % SE, P < .01). Retention of text-based information at follow-up correlated positively with education level and Spanish literacy, but not for illustration-based. CONCLUSIONS: In the setting of language and cultural barriers on a surgical mission, understanding of illustration-based and text-based information both increased after verbal explanation of illustrated postoperative instructions. Illustration-based information was more likely to be retained by patient caregivers after four weeks than text-based information, the latter of which correlated with increased education and literacy.


Assuntos
Fenda Labial , Fissura Palatina , Missões Médicas , Humanos , Criança , Fenda Labial/cirurgia , Fissura Palatina/cirurgia , Estudos Prospectivos
11.
Ann Plast Surg ; 89(5): 538-542, 2022 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-36279580

RESUMO

BACKGROUND: Facial palsy may have deleterious effects for pediatric patients. The most common reconstruction is 2-stage free gracilis muscle transfer (FGMT) after cross-face nerve graft (CFNG). This requires a prolonged period from time of surgery to smile. New techniques using both a CFNG and motor nerve to masseter (MNM) as dual power sources in a single-stage surgery have been described in adults. Here, we examine our experience with this technique in children. METHODS: A retrospective study was performed examining patients who underwent dual-innervated single-stage FGMT at 2 pediatric hospitals from 2016 to 2019. Demographics, etiology, perioperative characteristics, time to mandibular and emotional smile, and Sunnybrook scores were recorded. RESULTS: Five patients met inclusion criteria with a mean age of 11.8 (range, 8-20). Two patients had congenital facial palsy while 3 had acquired facial palsy. Four patients (80%) received dual end-to-end neural coaptations of the CFNG and MNM to the obturator nerve. One (20%) had end-to-side coaptation of the CFNG to the obturator nerve and end-to-end of the MNM to the obturator nerve. The average time to mandibular smile was 103 ± 15.4 days. The average time to emotional smile was 245 ± 48.1 days. The preoperative Sunnybrook scale was 32 ± 7.5 and improved to 55.3 ± 20.6 at 8 months postoperatively. CONCLUSIONS: Dual-innervated FGMT is effective for facial reanimation in children with unilateral facial palsy. Patients can harness a stronger motor source (MNM) and the component of an emotional stimulus (CFNG). This approach may be the new criterion standard pediatric facial reanimation.


Assuntos
Paralisia de Bell , Paralisia Facial , Músculo Grácil , Procedimentos de Cirurgia Plástica , Adulto , Humanos , Criança , Músculo Grácil/transplante , Paralisia Facial/cirurgia , Estudos Retrospectivos , Procedimentos de Cirurgia Plástica/métodos , Sorriso/fisiologia , Paralisia de Bell/cirurgia
12.
13.
Am J Med Genet A ; 185(12): 3694-3700, 2021 12.
Artigo em Inglês | MEDLINE | ID: mdl-34291880

RESUMO

Robin sequence (RS) has many genetic and nongenetic causes, including isolated Robin sequence (iRS), Stickler syndrome (SS), and other syndromes (SyndRS). The purpose of this study was to determine if the presence and type of cleft palate varies between etiologic groups. A secondary endpoint was to determine the relationship of etiologic group, cleft type, and mortality. Retrospective chart review of patients with RS at two high-volume craniofacial centers. 295 patients with RS identified. CP was identified in 97% with iRS, 95% with SS, and 70% of those with SyndRS (p < .0001). U-shaped CP was seen in 86% of iRS, 82% with SS, but only 27% with SyndRS (p < .0001). At one institution, 12 children (6%) with RS died, all from the SyndRS group (p < .0001). All died due to medical comorbidities related to their syndrome. Only 25% of children who died had a U-shaped CP. The most common palatal morphology among those who died was an intact palate. U-shaped CP was most strongly associated with iRS and SS, and with a lower risk of mortality. RS with submucous CP, cleft lip and palate or intact palate was strongly suggestive of an underlying genetic syndrome and higher risk of mortality.


Assuntos
Artrite/genética , Fenda Labial/genética , Fissura Palatina/genética , Doenças do Tecido Conjuntivo/genética , Perda Auditiva Neurossensorial/genética , Síndrome de Pierre Robin/genética , Descolamento Retiniano/genética , Artrite/diagnóstico por imagem , Artrite/mortalidade , Artrite/patologia , Criança , Pré-Escolar , Fenda Labial/diagnóstico por imagem , Fenda Labial/mortalidade , Fenda Labial/patologia , Fissura Palatina/diagnóstico por imagem , Fissura Palatina/mortalidade , Fissura Palatina/patologia , Doenças do Tecido Conjuntivo/diagnóstico por imagem , Doenças do Tecido Conjuntivo/mortalidade , Doenças do Tecido Conjuntivo/patologia , Feminino , Perda Auditiva Neurossensorial/diagnóstico por imagem , Perda Auditiva Neurossensorial/mortalidade , Perda Auditiva Neurossensorial/patologia , Humanos , Lactente , Masculino , Síndrome de Pierre Robin/diagnóstico por imagem , Síndrome de Pierre Robin/mortalidade , Síndrome de Pierre Robin/patologia , Descolamento Retiniano/diagnóstico por imagem , Descolamento Retiniano/mortalidade , Descolamento Retiniano/patologia , Estudos Retrospectivos
14.
J Craniofac Surg ; 32(3): 892-895, 2021 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-33086295

RESUMO

BACKGROUND: Facial palsy can have significant functional and psychological impact. Dynamic facial reanimation methods have provided means of restoration of smile. There remains a dearth of quality of life data in children. The authors aim to assess patient reported outcomes in this underreported population. METHODS: A retrospective review was performed of pediatric patients with facial palsy who had dynamic facial reanimation from 2009 to 2017 at a single institution. Patients were sent a digital Facial Clinimetric Evaluation (FaCE) questionnaire, a validated tool to assess patient quality of life. FaCE statements are grouped into 6 categories: facial movement, facial comfort, oral function, eye comfort, lacrimal control, and social control. RESULTS: Twenty patients met inclusion criteria. Fourteen successfully completed the survey. With normal function being 100, our cohort scored: facial movement 38.7 ±â€Š14.3; facial comfort 67.3 ±â€Š23, oral function 75 ±â€Š22.7, eye comfort 68.75 ±â€Š18.2, lacrimal control 53.6 ±â€Š32.5, social function 64.3 ±â€Š25.6. The overall FaCE score was 61.07 ±â€Š10.85. The facial movement subscores were lower for brow raise 16.1 ±â€Š33.4 relative to smiling 53.6 ±â€Š29.2 and lip pucker, 46.4 ±â€Š23.7. No correlation was found between facial movement scores and total scores (R2 < 0.01). There was a strong negative correlation between total scores and time since procedure (Spearman's rho R =  -0.45). CONCLUSION: Our pediatric facial reanimation cohort demonstrated excellent social function, in contrast to a relatively lower facial movement score. The low facial movement score was mediated by low score for brow raise and was not correlated to overall FaCE score. Interestingly, overall FaCE score tended to decrease with time from procedure.


Assuntos
Paralisia de Bell , Paralisia Facial , Criança , Paralisia Facial/cirurgia , Humanos , Qualidade de Vida , Estudos Retrospectivos , Sorriso
15.
Ann Vasc Surg ; 69: 217-223, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32497616

RESUMO

BACKGROUND: Definitive treatment of Paget-Schroetter syndrome (PSS) involves first rib resection (FRR), division of the anterior scalene muscle, and resection of the subclavius muscle. This is a single-institution experience with PSS, according to a treatment algorithm of preoperative venogram (accompanied by lysis and percutaneous mechanical thrombectomy as needed) followed by transaxillary FRR. In the later period of this experience, patients have often been discharged on aspirin only, with no plan for anticoagulation postoperatively. We sought to evaluate outcomes in light of this experience and these practice patterns. METHODS: Between 2007 and 2018, 125 transaxillary FRRs were performed in 123 patients. All patients presented with documented venous thrombosis, underwent diagnostic venography and-if indicated-lysis and percutaneous mechanical thrombectomy (VPT) before FRR. The patient was not offered FRR if the vein could not be crossed with a wire and patency was not re-established during percutaneous treatment. The experience was divided into early (before 2012, n = 50) and late (n = 75) periods. RESULTS: Mean patient age was 28.4 (12-64 years) years. Of the cohort, 33 were high-level competitive athletes, 13 presented with documented pulmonary embolism in addition to local symptoms, and 3 had a cervical rib fused to the first rib. Patients underwent FRR a median of 50 (4 days to 18 years) days after their initial symptoms, and a median of 22 (1 day to 9 months) days after their percutaneous intervention. Postoperative VPT was required in 23 patients and performed a median of 5 (1-137 days) days postoperatively; in 19 of these patients, postoperative VPT was required for postoperative re-thrombosis, whereas in 4 patients, postoperative VPT was planned before FRR due to vein stenosis or residual thrombus. All these patients were prescribed postoperative anticoagulation. No operative venous reconstruction or bypass was performed. Median follow-up time after FRR was 242 days; at last follow-up, 98.4% (123/125) of axillosubclavian veins were patent by duplex ultrasound (and all those patients were asymptomatic). Postoperative anticoagulation was less frequently prescribed in the late experience, with no difference in the rate of early re-thrombosis or follow-up patency. CONCLUSIONS: This experience demonstrates 98.4% patency at last follow-up with standard preoperative percutaneous venography and intervention, transaxillary FRR, and postoperative endovascular re-intervention only in cases with persistent symptoms, stenosis, or re-thrombosis. Patients presenting with both acute and chronic PSS did not require surgical venous reconstruction. In the later experience, patients frequently have not been anticoagulated postoperatively. Advantages of this algorithm include the following: (1) the cosmetic benefits of the transaxillary approach, (2) the preoperative assessment of the ability to recanalize the vein to determine which patients will benefit from surgery, (3) the capacity to use thrombolysis preoperatively, and (4) potential elimination of the risk and inconvenience of postoperative anticoagulation.


Assuntos
Anticoagulantes/administração & dosagem , Osteotomia , Cuidados Pós-Operatórios , Costelas/cirurgia , Trombose Venosa Profunda de Membros Superiores/cirurgia , Adolescente , Adulto , Anticoagulantes/efeitos adversos , Criança , Esquema de Medicação , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteotomia/efeitos adversos , Philadelphia , Flebografia , Cuidados Pós-Operatórios/efeitos adversos , Recidiva , Estudos Retrospectivos , Fatores de Risco , Trombectomia , Terapia Trombolítica , Fatores de Tempo , Resultado do Tratamento , Trombose Venosa Profunda de Membros Superiores/diagnóstico por imagem , Trombose Venosa Profunda de Membros Superiores/fisiopatologia , Grau de Desobstrução Vascular , Adulto Jovem
16.
Ann Plast Surg ; 84(6): 665-671, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-31913900

RESUMO

PURPOSE: The purpose of this study was to characterize airway problems, speech outcomes, and facial growth in patients with Stickler syndrome undergoing cleft palate repair. METHODS: A retrospective, longitudinal study was performed at the Children's Hospital of Philadelphia on 25 patients with Stickler syndrome and 53 nonsyndromic patients with clefts of the secondary palate repaired between 1977 and 2000. Airway problems were characterized by the incidence of Pierre Robin Sequence (PRS) and the necessity for surgical airway management. Speech was analyzed using the Pittsburgh weighted values for speech symptoms associated with velopharyngeal incompetence (VPI). Longitudinal anthropometric measurements represented up to 12 years of longitudinal cephalofacial growth. RESULTS: Seventy-two percent of patients with Stickler syndrome were diagnosed with PRS, 55.6% of whom required surgical airway management. Conversely, 20.8% of nonsyndromic patients were diagnosed with PRS (P < 0.0001), 18% of whom required surgical intervention (P < 0.05). Speech outcomes were poorer in patients with Stickler syndrome with 40% demonstrating borderline VPI and 13.3% demonstrating VPI, compared with 21.8% and 9.1%, respectively, in the nonsyndromic group. Both groups exhibited significantly shallower upper and mid facial depths and wider upper facial breadths when compared with normal standards of facial growth. Although there was a tendency toward decreased facial depths in patients with Stickler syndrome relative to nonsyndromic patients, the differences were nonsignificant. CONCLUSIONS: Patients with Stickler syndrome show significant potential for early airway compromise and a poorer prognosis for speech outcome after cleft palate repair. Their cephalofacial growth does not differ significantly from that of nonsyndromic cleft palate patients.


Assuntos
Fissura Palatina , Insuficiência Velofaríngea , Artrite , Criança , Fissura Palatina/cirurgia , Doenças do Tecido Conjuntivo , Perda Auditiva Neurossensorial , Humanos , Estudos Longitudinais , Philadelphia , Descolamento Retiniano , Estudos Retrospectivos , Fala , Resultado do Tratamento , Insuficiência Velofaríngea/etiologia , Insuficiência Velofaríngea/cirurgia
17.
J Craniofac Surg ; 31(1): 230-233, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31821211

RESUMO

PURPOSE: Postoperative hip pain is commonly reported after anterior iliac crest harvest for alveolar bone grafting. The goal of this study is to describe our institution's experience and examine the efficacy of our pain management protocols. METHODS: A retrospective review was performed by abstracting demographic, operative, and pain management data from January 2011 to April 2013. Paired t-tests and Fisher exact tests were used to examine differences when comparing 2 groups, while ANOVA was used to examine difference between the 3 protocols for harvest and pain management: trapdoor technique and local anesthetic injection (TD+LAI), TD and pain catheter (TD+PC), and split crest and LAI. RESULTS: Eighty-four patients, 52 males (61.9%), averaging 8.8 years old (±2.9) were included. Postoperatively, 17 (71%) patients in the PC group received IV narcotics compared to 27 (45%) in those without a PC (P = .03). When comparing all 3 protocols, no significant difference was found in IV morphine usage or duration of IV morphine treatment. In subgroup analysis, when patients in the groups TD+PC versus TD+LAI were examined, those in the TD+PC group had significantly shorter hospital stays and were more likely to go home postoperative day 1 (P = .03; P = .04). CONCLUSIONS: Overall, patients tolerated alveolar bone grafting well regardless of harvest technique or pain management approach. While indwelling PCs did not significantly decrease IV morphine usage, these patients had significantly shorter lengths of stays.


Assuntos
Enxerto de Osso Alveolar , Fissura Palatina/cirurgia , Manejo da Dor , Dor Pós-Operatória/tratamento farmacológico , Transplante Ósseo/métodos , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Morfina/uso terapêutico , Manejo da Dor/métodos , Estudos Retrospectivos , Resultado do Tratamento
18.
J Craniofac Surg ; 31(1): 85-90, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31609948

RESUMO

BACKGROUND: Lengthening temporalis myoplasty (LTM) and cross-face nerve graft with free gracilis muscle transfer (CFNG-FGMT) are the 2 most common procedures used to restore dynamic facial animation and improve facial symmetry. There has not been direct comparison or consensus. Here, the authors compare our experience with respect to muscle activity, symmetry, and excursion. METHODS: A retrospective review was performed of patients with facial palsy who had CFNG-FGMT or LTM from 2008 to 2016 at a single institution. Postoperative surface EMG was recorded at maximum open smile. Normal and paralyzed sides of the face were analyzed with Facial Assessment by Computer Evaluation software. Commissure excursion and symmetry was assessed. RESULTS: Six patients with LTM and 10 with CFNG-FGMT met inclusion criteria. Muscle activity was 1st identified in LTM patients after 3 months (47.42 mV, P < 0.001) and CFNG-FGMT patients after 3 months (28.30 mV, P < 0.001) compared to immediate postoperative period. Relative to preoperative excursion, there was significant increase of 3.33 mm in commissure excursion seen at the 0 to 3 month period for LTM patients (P = 0.04). Commisure excursion for CFNG-FGMT was seen later, in the 3- to 6-month postoperative period (4.01, P = 0.024). During smile, CFNG-FGMT patients had better symmetry than unilateral LTM patients. In bilateral LTM patients, there was no significant change in symmetry. CONCLUSION: Dynamic facial animation improved in both surgical groups. The LTM demonstrates a faster rate of muscle recruitment compared to CFNG-FGMT. After 3 months, both LTM and CFNG-FGMT groups had comparable excursions. A decision-making algorithm is presented.


Assuntos
Músculo Grácil/cirurgia , Procedimentos de Cirurgia Plástica , Músculo Temporal/cirurgia , Adolescente , Criança , Paralisia Facial/etiologia , Feminino , Humanos , Masculino , Transferência de Nervo , Estudos Retrospectivos , Sorriso , Adulto Jovem
19.
Cleft Palate Craniofac J ; 57(1): 127-131, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31327233

RESUMO

OBJECTIVE: Deficiencies of the upper lip vermilion occur with some frequency following repair of unilateral and bilateral clefts of the lip and can compromise the aesthetic outcome. The presence of dense scar tissue within the lip at the cleft site as well as intrinsic vermillion deficiencies can make long-lasting correction of this deformity challenging. We describe a technique to address vermillion deficiencies after cleft lip repair. DESIGN: A novel lip augmentation technique for correction of residual vermilion deficiencies after unilateral and bilateral cleft lip repair is presented. This technique combines precise placement of a contoured dermal fat graft with local tissue (V-Y) rearrangement. CONCLUSION: In our experience, this method of lip augmentation following either unilateral or bilateral cleft lip repair can restore upper lip vermillion symmetry and provide predictable and durable results in patients with mild to severe vermillion deficiency.


Assuntos
Fenda Labial/cirurgia , Procedimentos de Cirurgia Plástica , Tecido Adiposo/transplante , Estética Dentária , Humanos , Lábio/cirurgia , Resultado do Tratamento
20.
Cleft Palate Craniofac J ; 57(9): 1069-1077, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32618203

RESUMO

OBJECTIVE: To identify demographic factors that influence choosing nasoalveolar molding (NAM) in the treatment of cleft lip with or without cleft palate (CL±P), and NAM treatment compliance. DESIGN: This work is a retrospective cohort study. SETTING: Tertiary pediatric hospital. PATIENTS, PARTICIPANTS: One hundred forty-nine patients with a diagnosis of unilateral complete CL±P receiving treatment when NAM was offered (January 1, 2008-July 26, 2016). MAIN OUTCOME MEASURE(S): Demographic variables collected included race, ethnicity, ZIP code, number of caregivers, caregiver employment status, and health insurance status. Medical variables collected included diagnosis, treatment pursued, compliance with NAM, completion of NAM, and the treating cleft surgeon and orthodontist. Data were analyzed via Fisher exact tests, χ2 tests, and multivariate logistic regression to identify factors that influence the decision to pursue NAM and treatment compliance. RESULTS: Univariate analyses identified the following significant factors predicting the pursuit of NAM: race and insurance type (both P < .001), surgeon (P = .005), income level (P = .009), comorbidities (P = .015), and syndromic diagnosis (P = .033). Driving distance trended toward significance (P = .078). Multivariate regression analyses indicated that Asian race (P = .047), insurance type (P = .046), driving distance (P = .019), and surgeon (P = .017) were significant predictors of pursuit of NAM. CONCLUSIONS: There are disparities in patient choice of NAM at our center for children with complete cleft lip. African American patient families were less likely to pursue this intervention. A stronger understanding of the barriers that lower income and minority patients face is needed in order to better characterize disparities in cleft care.


Assuntos
Fenda Labial , Fissura Palatina , Processo Alveolar , Criança , Fenda Labial/terapia , Fissura Palatina/terapia , Hospitais Pediátricos , Humanos , Moldagem Nasoalveolar , Nariz , Estudos Retrospectivos
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