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OBJECTIVE: Disparities in insurance and socioeconomic status (SES) may impact surgical management and subsequent postoperative outcomes for patients with craniosynostosis. This systematic review summarizes the evidence on possible differences in surgical care, including procedure type, age at surgery, and differences in surgical outcomes such as complications, length of hospital stay, and child development based on SES. DESIGN: The databases Scopus, PubMed, and CINAHL were searched between May and July 2022. Following PICO criteria, studies included focused on patients diagnosed with craniosynostosis; corrective surgery for craniosynostosis; comparison of insurance, income, or zip code; and surgical management of postoperative outcomes. RESULTS: The initial search yielded 724 articles. After three stages of screening, 13 studies were included. Assessed outcomes included: type of procedure (6 articles), age at time of surgery (3 articles), post-operative complications (3 articles), referral delay (2 articles), length of stay (2 articles), hospital costs (2 articles), and child development (1 article). Of the studies with significant results, insurance type was the main SES variable of comparison. While some findings were mixed, these studies indicated that patients with public medical insurance were more likely to experience a delay in referral, undergo an open rather than minimally-invasive procedure, and have more complications, longer hospitalization, and higher medical charges. CONCLUSIONS: This study demonstrated that SES may be associated with several differences in the management of patients with craniosynostosis. Further investigation into the impact of SES on the management of patients with craniosynostosis is warranted to identify possible interventions that may improve overall care.
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OBJECTIVE: Investigate an association between upper airway obstruction (UAO) management in Robin Sequence (RS) and need for bilateral myringotomy and tubes (BMT). METHODS: Retrospective chart review of RS patients treated at a tertiary free-standing pediatric hospital from 1995 to 2020 was performed. Patients were grouped based on airway management: conservative, tracheostomy, tongue-lip adhesion (TLA), and mandibular distraction osteogenesis (MDO). Demographic data, cleft palate (CP) association, numbers of BMT and ear infections, and audiogram data including tympanograms were collected. One-way ANOVA and Chi-square/Fisher's exact tests were used to compare continuous and categorical data, respectively. Multivariable regression analysis was used to compare BMT rates between treatment groups. RESULTS: One hundred forty-eight patients were included, 70.3 % of which had CP. Most patients (67.6 %) had at least one BMT; 29.1 % required two or more BMT. The rate of BMT was higher in patients with CP compared to those with intact palates (p = 0.003; 95 % CI 1.30-3.57) and those treated with tracheostomy (p = 0.043; 95 % CI 1.01, 2.27). Surgically managed patients were more likely to have hearing loss (67.5 % vs. 35.3 %, p = 0.017) and ear infections (42.1 % vs. 20.0 %, p = 0.014) pre-compared to post-procedure for airway management. CONCLUSION: Most RS patients require at least 1 set of BMT. Those with CP and/or treated with tracheostomy had a higher likelihood of needing BMT. Rate of hearing loss and ear infection was higher in surgically managed RS patients. Patients with RS and overt CP require a statistically higher number of BMTs compared to those with either submucous cleft palate or intact palate.
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Obstrução das Vias Respiratórias , Ventilação da Orelha Média , Síndrome de Pierre Robin , Humanos , Síndrome de Pierre Robin/complicações , Síndrome de Pierre Robin/cirurgia , Masculino , Estudos Retrospectivos , Obstrução das Vias Respiratórias/cirurgia , Obstrução das Vias Respiratórias/etiologia , Feminino , Ventilação da Orelha Média/métodos , Lactente , Traqueostomia , Pré-Escolar , Manuseio das Vias Aéreas/métodos , Osteogênese por Distração/métodos , CriançaRESUMO
Background: Interventions for type B postaxial polydactyly include suture ligation and surgical excision, yet there is a paucity of literature comparing the outcomes of these procedures. This study sought to compare patient-reported long-term outcomes of postaxial digit excision. Methods: A six-question survey was distributed from January 2021 to March 2022 to patients who underwent treatment for type B postaxial polydactyly at a single pediatric institution from 2010 to 2016. Patients were queried about the incidence of pain sensitivity, keloid healing, and/or persistent presence of bump ("nubbin") at the treatment site. Results: A total of 158 responses accounting for 258 digits were attained for a 53% response rate. The majority of digits (67.4%, n = 174) were surgically excised. Median age at procedure was 49 days: 13.0 days for ligation, 63.0 days for surgical excision. Median age at survey was 8 [IQR 5.4-10.2] years. Short-term (<30 days after procedure) complications rate was 1.6%. The rate of a raised or sensitive scar was 39.5% (ligation 51.5% versus surgery 35.4%, P < 0.05). The likelihood of postoperative sensitivity (P = 0.80) was similar among groups. However, the odds of a residual bump or raised scar at the surgical site was significantly higher in the ligation group (P = 0.001). These findings remained significant in the adjusted analysis. Conclusion: This study suggests that suture ligation can be used in select cases without increasing the prevalence of long-term pain or sensitivity, albeit with greater risk of a bump or raised scar at the excision site compared with surgical excision.
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OBJECTIVE: Evaluate infants with Robin Sequence (RS) who were successfully treated with conservative airway measures alone vs. those who failed and eventually underwent surgical airway intervention after a protracted course of conservative management. DESIGN: Retrospective review of prospectively gathered database. SETTING: Large tertiary care institution. PATIENTS: Infants diagnosed with RS (n = 122) who underwent primary airway management at a single institution from 1994-2020. MAIN OUTCOME MEASURE: Patient demographics, nutritional and respiratory status, laboratory values, and polysomnographic results were compared between patients who were discharged after successful conservative airway management (Group 1, n = 61) and patients that underwent surgical airway intervention after failing a prolonged course of conservative management (Group 2, n = 61). Receiver operating characteristic (ROC) curve analysis was done to assess continuous variables that may predict failure of conservative airway management. RESULTS: 122 infants with RS were investigated. While several variables were significantly different between groups, the following polysomnographic EARN factors, with cut points, were identified as most predictive of failed conservative airway management: ETCO2 (max) > 49 mmHg, AHI > 16.9 events/hour, OAHI REM >25.9 events/hour, OAHI Non-REM > 23.6 events/hour. CONCLUSIONS: We identified factors in infants with RS that were associated with severe UAO that failed to improve despite weeks of conservative airway management. Our results may expedite earlier definitive treatment of these critical patients and reduce risks for known complications of prolonged UAO.
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Dermatofibrosarcoma protuberans (DFSP) is a rare malignant fibroblastic tumor. DFSP has an insidious onset, slow growth, and heterogeneous presentation that can create a delay in diagnosis and increase morbidity. In this case report, we present a child with DFSP that presented as a large, slow-growing mass over the dorsum of the left foot. She underwent successful surgical excision with no functional sequelae.
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OBJECTIVE: Robin sequence (RS) consists of micrognathia and glossoptosis that result in upper airway obstruction (UAO). In RS patients who undergo tracheostomy, long-term goals include natural decannulation (ND) without further surgical airway intervention. The objective of this study was to identify long-term trends in the rate and length of time to ND. METHODS: Retrospective chart review on 144 patients with RS treated from 1995 to 2020 at a pediatric tertiary care center. Patients were grouped by year of tracheostomy. Demographic data, UAO management, postoperative care, complications, and time to decannulation were recorded. RESULTS: Thirty-six patients met the inclusion criteria. Tracheostomy was performed at a median age of 45.5 days. 19 (53%) patients experienced ND at a median time of 66.1 months. ND rate was higher in non-syndromic patients (93% non-syndromic vs 27% syndromic; P < .0001) and during the first study period (1995-2006: 78%, 2007-2020: 28%; P = .003). Cox proportional-hazard regression demonstrated that white race [aHR 0.15 (0.03-0.8); P = .023] and higher birthweight [aHR 0.9 (0.8-0.98); P = .018] were associated with a higher likelihood of ND while a syndromic diagnosis had a negative association with ND [aHR 12.5 (3.3-50.0); P < .001]. CONCLUSIONS: Our study documented that ND in patients with RS who underwent tracheostomy was significantly associated with ethnicity, birthweight, and syndromic status. The negative impact on successful ND was most observed in patients with syndromic associations. Additionally, ND rates are lower in the 2007 to 2020 subgroup. We suspect this is because alternative management techniques such as tongue lip adhesion and mandibular distraction osteogenesis became primary surgical treatment in severe RS upper airway obstruction at our institution starting in 2007.
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Obstrução das Vias Respiratórias , Síndrome de Pierre Robin , Humanos , Criança , Lactente , Estudos Retrospectivos , Resultado do Tratamento , Síndrome de Pierre Robin/complicações , Síndrome de Pierre Robin/cirurgia , Peso ao Nascer , Obstrução das Vias Respiratórias/etiologia , Obstrução das Vias Respiratórias/cirurgia , Traqueostomia/efeitos adversosRESUMO
OBJECTIVE: Cleft lip repair (CLR) can be complicated by hypertrophic scar or keloid. Botulinum toxin type A (BTA) may improve postoperative scarring by reducing muscle tension and cytokine activity at the scar site. This systematic review analyzes the available evidence regarding the effect of BTA on scar quality after CLR. DESIGN: The search was conducted in 6 different databases in accordance with PRISMA guidelines (PubMed, Scielo, Embase, Scopus, Web of Science, and Cochrane) using "botulinum toxin" and "cleft lip" as keywords. SETTING: Academic hospital. PATIENTS: Exclusive to patients who underwent CLR and BTA injection. OUTCOME MEASURES: Mean visual analog scores (VAS), mean Vancouver scar scale (VSS), scar width, and BTA or CLR-related complications. RESULTS: Five studies for a total of 216 patients met inclusion criteria. Four studies reported on primary CLR during infancy while 1 study recruited older patients seeking revision. All patients had BTA (range: 1-2â units/kg) injected in the orbicularis oris muscle. One study documented BTA injections in additional perioral muscles. All 4 studies that measured scar width and had a saline control arm found a significant decrease in width with BTA injection. Improvement of VAS and VSS with BTA was reported in 3 of 5 studies and 2 of 5 studies, respectively. There were no reports of complications associated with BTA or CLR. CONCLUSION: The existing studies support the use of BTA injection to improve scar quality following CLR with low concern for complication. Further investigations with a greater number of patients are necessary.
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OBJECTIVE: Several studies have compared perioperative parameters and early postoperative morphology between endoscope-assisted strip craniectomy with orthotic therapy (endoscopic repair) and cranial vault remodeling (open repair). To extend these results, the authors evaluated school-age anthropometric outcomes after these techniques across three institutions. METHODS: School-aged children (age range 4-18 years) with previously corrected isolated sagittal craniosynostosis were enrolled. Upon inclusion, 3D photographs and patient-reported outcomes were obtained, and the cephalic index and head circumference z-scores were calculated. Analyses of covariance models controlling for baseline differences and a priori covariates were performed. RESULTS: Eighty-one participants (median [range] age 7 [4-15] years) were included. The mean (95% CI) school-age cephalic index was significantly higher in the endoscopic cohort, though within the normal range for both groups (endoscopic 78% [77%-79%] vs open 76% [74%-77%], p = 0.027). The mean change in the cephalic index from preoperation to school age was significantly greater in the endoscopic group (9% [7%-11%] vs open 3% [1%-5%], p < 0.001). Compared to preoperative measurements, mean school-age head circumference z-scores decreased significantly more in the open cohort (-1.6 [-2.2 to -1.0] vs endoscopic -0.3 [-0.8 to -0.2], p = 0.002). Patient-reported levels of stigma were within the normal limits for both groups. CONCLUSIONS: Endoscopic and open repair techniques effectively normalize school-age anthropometric outcomes. However, endoscopic repair produces a clinically meaningful and significantly greater improvement in the school-age cephalic index, with maintenance of head growth. These findings demonstrate the importance of early referral by pediatricians and inform treatment decisions.
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Craniossinostoses , Criança , Humanos , Lactente , Pré-Escolar , Adolescente , Resultado do Tratamento , Estudos Retrospectivos , Craniossinostoses/cirurgia , Crânio/cirurgia , Craniotomia/métodos , Medidas de Resultados Relatados pelo PacienteRESUMO
Despite rules set forth by the National Resident Matching Program and American Association of Medical Colleges (AAMC), prohibited questions during the residency interview process are well documented. This study describes the prevalence of these encounters by surveying residency applicants to integrated plastic and reconstructive surgery (PRS) programs for the 2022 match cycle. Methods: An anonymous 16-question REDCap survey was distributed to 2022 cycle applicants of a single PRS program. The applicants were queried about demographic information, interview experience, and questions deemed illegal by the AAMC/NRMP guidelines. Results: One hundred survey responses were attained for a 33.1% response rate. The majority of respondents were aged 26-30 (76%), women (53%), and white (53%); 33% received 15+ interviews for the application cycle. Seventy-eight percent of respondents reported being asked a prohibited question during at least one interview, with the most common "illegal" question categories being number/ranking of interviews (42%), marital status (33%), career balance (25%), and race/ethnicity (22%). Only 25.6% of applicants considered the subject matter inappropriate, whereas 42.3% were unsure. Although no applicant took action to report the potentially illegal scenarios, 30% said that their experiences influenced their rank list. Conclusions: Our survey study revealed that prohibited interview questions in PRS residency interviews are common. Permissible lines of questioning and discussion between programs and applicants during residency interviews have been defined by AAMC. Institutions should provide guidance and training to all participants. Applicants should be made aware of and empowered to utilize available anonymous reporting tools.
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The pharyngeal arches form the cornerstone of the complex anatomy of the face and neck. These embryologic structures are the foundation of face and neck development, and anomalous growth can result in craniofacial abnormalities. Surgeons who manage head and neck pathology and pathoanatomy will invariably encounter conditions associated with aberrant pharyngeal arch anatomy, and a thorough understanding of the normal and pathological development of these important structures is paramount to accurate diagnosis and treatment. This manuscript is the first of a three-part educational series that addressed the pharyngeal/branchial arch embryology, development, nomenclature, and normal anatomy (Part I), pathologic anomalies of ear and neck derived from abnormal development of the arches (Part II), and different types of orofacial clefts, including Tessier clefts (Part III).
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Região Branquial , Fenda Labial , Fissura Palatina , Humanos , Região Branquial/anormalidades , Cabeça , PescoçoRESUMO
Calcinosis cutis is the term used to describe the deposition of calcium compounds within the skin and subcutaneous tissue, which can occur after the administration of intravenous calcium compounds. Its etiology is broad, and the clinical presentation is variable, creating a diagnostic challenge. Although iatrogenic calcinosis cutis is extremely uncommon, awareness and early diagnosis of this entity can reduce the risks of severe complications, including soft tissue damage, restricted joint mobility, and even nerve compression. Clinical suspicion should prompt a thorough review of the medical history and appropriate radiographic studies. Evidence of extensive soft tissue calcification must be present on radiographic imaging to confirm the diagnosis. Iatrogenic calcinosis cutis is managed conservatively, and resolution of symptoms is expected within 2 months of symptom onset. Herein we report the case of an infant with DiGeorge syndrome who developed iatrogenic calcinosis cutis after receiving an intraoperative infusion of calcium gluconate. Our patient presented with right lower extremity swelling, erythema, and warmth over a broad area of the leg centered on the entry point of the venipuncture. This was initially mistaken and managed as cellulitis, but once an accurate diagnosis was made, the symptoms gradually resolved with conservative care and no functional sequelae. We also present the literature on iatrogenic and idiopathic calcinosis cutis in the pediatric population.
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SUMMARY: Prenatal diagnosis of cleft palate (CP) is challenging. The current study's objective was to investigate whether prenatal alveolar cleft width is associated with the likelihood of a cleft of the secondary palate in unilateral cleft lip (CL). The authors reviewed two-dimensional ultrasound (US) images in fetuses with unilateral CL from January of 2012 to February of 2016. Images of the fetal face were obtained with a linear and/or curved probe in the axial and coronal planes. Measurements of the alveolar ridge gap were taken by the senior radiologist. Postnatal phenotype findings were compared with prenatal findings. Thirty patients with unilateral CL met inclusion criteria; average gestational age was 26.67 ± 5.11 weeks (range, 20.71 to 36.57 weeks). Ten fetuses were found to have an intact alveolar ridge by prenatal US; postnatal examination confirmed intact secondary palate in all. Small alveolar defects (<4 mm) were noted in three fetuses; postnatal examination documented CP in a single patient. CP was confirmed in 15 of the remaining 17 fetuses who had alveolar cleft width greater than 4 mm. An alveolar defect of greater than or equal to 4 mm on prenatal US was associated with greater likelihood of a cleft of the secondary palate [c 2 (2, n = 30) = 20.23; P < 0.001]. In the setting of unilateral CL, prenatal US documentation of alveolar defects greater than or equal to 4 mm are highly predictive of the presence of a cleft of the secondary palate. Conversely, an intact alveolar ridge is associated with an intact secondary palate. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.
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Fenda Labial , Fissura Palatina , Feminino , Humanos , Gravidez , Lactente , Fissura Palatina/diagnóstico por imagem , Fenda Labial/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodos , Processo Alveolar/diagnóstico por imagemRESUMO
Digital extensor hypoplasia (DEH) is a rare malformation that presents with loss of active finger extension at the metacarpophalangeal (MCP) joints. Descriptions of optimal treatment and outcomes in this population are sparse. We describe successful operative treatment of a child with DEH involving the extensor digitorum communis, extensor digiti minimi, and the extensor indicis proprius tendons. The 5-year-old male patient was referred for severe limitation on bilateral finger extension since birth. He had been previously diagnosed with arthrogryposis and managed conservatively. Due to lack of improvement, magnetic resonance imaging was done evidencing hypoplasia/aplasia of the extensor tendons. The patient underwent successful tendon transfers using extensor carpi radialis longus to the common extensor tendons, and one hand required an additional tenolysis procedure. 2 years postoperatively, his MCP position and finger extension are markedly improved, and he is able to grip objects without limitation or difficulty. The patient returned to full activity without restriction.
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OBJECTIVE: Collect data from craniofacial surgeons to analyze mandibular distraction osteogenesis (MDO) protocols, and facial nerve dysfunction (FND) to characterize this common, but poorly documented complication after MDO in infants with Robin Sequence (RS). DESIGN, SETTING, AND PARTICIPANTS: A 16-question anonymous survey designed through REDCap was digitally distributed to members of the American Cleft Palate-Craniofacial Association and International Society of Craniofacial Surgery (ISCFS). MAIN OUTCOME MEASURE(S): Demographic information, MDO perioperative variables, surgeon experience with FND after MDO for patients with RS, and the timing and duration of FND were analyzed. RESULTS: Eighty-four responses were collected, with 80 included for analysis. Almost two-thirds of respondent surgeons reported FND as a complication of MDO in patients with RS (51, 63.8%); 58.8% (n = 47) transient FND and 5% (n = 4) with permanent facial nerve palsy only. Both transient and permanent FND was documented by 13 (16.3%) respondents. Among respondents, FND was observed immediately following initial device placement/osteotomies in 45.1%, during distraction in 45.1%, during consolidation in 19.6%, and following device removal in 43.1%. Twenty-five of these respondent surgeons reported resolution of FND between 1 and 3 months (53.2%, n = 25). CONCLUSIONS: FND after MDO in patients with RS was noted by most respondents in this survey study. While most surgeons noted temporary FND, one-fifth reported long-term dysfunction. FND was documented most commonly following device placement/osteotomies or during active distraction. Further research should seek to establish risk factors associated with FND and identify surgical and perioperative prevention strategies.
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Obstrução das Vias Respiratórias , Osteogênese por Distração , Síndrome de Pierre Robin , Lactente , Humanos , Estudos Retrospectivos , Síndrome de Pierre Robin/cirurgia , Síndrome de Pierre Robin/complicações , Osteogênese por Distração/métodos , Nervo Facial , Resultado do Tratamento , Mandíbula/cirurgia , Obstrução das Vias Respiratórias/cirurgiaRESUMO
BACKGROUND: Supermicrosurgery is a technique that allows microsurgeons to accomplish dissections and anastomoses of vessels and nerve fascicles with diameters of ≤0.8 mm. Considering the potential benefits of this technique and limited literature synthesizing the outcomes of supermicrosurgery, the aim of this study was to summarize the available evidence of reconstructive supermicrosurgery and to estimate the success rate. METHODS: We conducted a comprehensive search across PubMed, Scopus, Embase, and Web of Science. We included patient-based studies reporting on procedures for soft-tissue reconstruction with free flaps specifying the use of supermicrosurgery. We excluded studies reporting on lymphatic surgery, solely peripheral nerve surgery, and tissue replantation. Our primary endpoint was to calculate the flap success rate. Pooled estimates were calculated using a random-effects meta-analytic model. RESULTS: Forty-seven studies reporting outcomes of 698 flaps were included for qualitative synthesis. Overall, 15.75% of flaps were used for head and neck, 4.4% for breast and trunk, 9.3% for upper limb, and 69.2% for lower limb reconstruction. The most used flap was the superficial circumflex iliac artery perforator flap (41.5%). The overall flap success rate was 96.6% (95%CI 95.2%-98.1%). The cumulative rate of partial flap loss was 3.84% (95%CI 1.8%-5.9%). The overall vascular complication rate resulting in complete or partial flap loss was 5.93% (95%CI 3.5%-8.3%). CONCLUSIONS: Supermicrosurgery displays a high success rate. Further studies are necessary to explore the true potential of supermicrosurgery. This technique reformulates the boundaries of reconstructive surgery due to its extensive application.
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Retalhos de Tecido Biológico , Retalho Perfurante , Procedimentos de Cirurgia Plástica , Humanos , Retalhos de Tecido Biológico/irrigação sanguínea , Retalho Perfurante/irrigação sanguínea , Extremidade Inferior , CabeçaRESUMO
INTRODUCTION: Robin sequence (RS) is a leading cause of obstructive sleep apnea (OSA) in newborns. Most studies have focused on understanding anatomic factors leading to OSA and changes in apnea-hypopnea index (AHI) on polysomnography (PSG) beyond the neonatal period. This study aims to define age-related OSA features between patients with RS, without RS and healthy controls using PSG-based analyses of respiratory arousal responses and gas-exchange parameters. DESIGN: Retrospective comparison of PSG features in a total of 48 children encompassing three groups: (a) infants with RS (n = 24, <1-year old), (b) non-RS older children (1-2 years old) with severe OSA (obstructive AHI (OAHI) of ≥10 events; n = 12), and (c) control infants and children (0-2 years old) without sleep apnea (OAHI ≤1.5/h, n = 12). We examined OSA sleep-stage specific and position-specific indexes, and the relationship between OSA severity and respiratory arousal indexes (OAHI/respiratory arousal indexes). RESULTS: OSA sleep-stage specific indexes (rapid eye movement [REM] vs non-REM[NREM]) as well as position-specific indexes (supine vs nonsupine) were similar in individuals with and without RS. Relative to the non-RS groups, infants with RS have more sustained hypoxemia (time with SpO2 < 90%) and reduced arousal responses to OSA demonstrated by higher OAHI/respiratory arousal indexes. OAHI/respiratory arousal indexes significantly correlated with the severity of hypoxemia in infants with RS. CONCLUSION: Infants with RS and OSA show reduced arousal responses to apneic events, which correlates with higher hypoxemia severity. OAHI/respiratory arousal indexes in RS may identify high-risk individuals with upper airway obstruction and reduced arousal protective responses.
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Síndrome de Pierre Robin , Apneia Obstrutiva do Sono , Criança , Lactente , Humanos , Recém-Nascido , Adolescente , Pré-Escolar , Estudos Retrospectivos , Síndrome de Pierre Robin/complicações , Apneia Obstrutiva do Sono/etiologia , Hipóxia/complicações , Nível de AlertaRESUMO
OBJECTIVE: Robin Sequence (RS), characterized by micrognathia, glossoptosis, and upper airway obstruction, is an increasingly recognized diagnosis. An effective surgical intervention is mandibular distraction osteogenesis (MDO). This study analyzes published evidence regarding facial nerve dysfunction (FND) associated with MDO. DESIGN AND SETTING: According to PRISMA guidelines, a systematic review was carried out with databases queried in June 2019 using MESH terms, or equivalent terms, as follows: "distraction osteogenesis" and "Robin Sequence". A review of original Spanish and English articles, were included. Outcome measures included the prevalence of FND; the affected branches; the rate of permanent vs. transient FND; the use of an internal vs. external device; the daily distraction rate; and finally, the overall distraction length. Subsequently, a meta-analysis was conducted to collate results regarding the prevalence of FND and the factors associated with it. RESULTS: Of 239 unique studies identified, 19 studies with 729 patients met inclusion criteria; 52 patients developed FND after MDO. A random-effects meta-analysis yielded a pooled prevalence of FND of 6.40%, with moderately heterogeneous studies (I2 = 41%, τ2 = 0.006). Marginal mandibular nerve involvement was most commonly noted. Nine studies reported transient FND, six permanent, one both, and two unspecified. Internal distractors were used in 8 studies and external in 3 and both in 2. Distraction rate was 1.00 to 2.00â mm/day and total distraction length ranged from 13.00 to 22.3â mm. Sample size was the only parameter inversely associated with rate of FND (p = 0.04). CONCLUSION: This analysis of FND associated with MDO for patients with RS demonstrates a lack of consistent documentation. MDO-associated FND does not appear to be uncommon, and permanent dysfunction can occur. This review underscores the importance of thorough documentation to elucidate the mechanism of FND.
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Nervo Facial , Síndrome de Pierre Robin , Humanos , Osteogênese , Estudos Retrospectivos , Resultado do Tratamento , Mandíbula/cirurgia , Síndrome de Pierre Robin/cirurgia , Síndrome de Pierre Robin/complicaçõesRESUMO
Sickle cell disease (SCD) leads to the formation of an atypical hemoglobin tetramer with reduced capacity to carry oxygen. Although correlation between SCD and craniosynostosis (CS) has been mentioned, these are mostly small series or case reports. This article aimed to study any correlation between these entities in a large pediatric population. Methods: We retrospectively reviewed head CT scans of SCD patients from 0 to 8 years of age who required a CT for issues unrelated to their head shape between 2012 and 2020. We excluded patients with known history of CS or any CS-related syndrome, hydrocephalus, shunt placement, history of cranial surgery, or any reported cerebral or cranial shape abnormality. Results: Ninety-four CT scans were analyzed. The mean age at imaging was 4.48 ± 2.30 years. CS prevalence in this cohort was 19.1%. Analysis between independent variables and patients with +CS showed that SCD-associated vasculopathy, first-degree relatives with SCD, and the use of folic acid had a statistically significant association with CS development. Conclusions: Approximately 20% of pediatric patients with SCD developed CS. This association was higher in those patients who had a family history of SCD, used folic acid, and had SCD-associated vasculopathy. While the clinical impact of these findings needs more extensive study, centers that manage patients with SCD should be aware of the relatively high concordance of these diagnoses, vigilantly monitor head shape and growth parameters, and understand the potential risks associated with unidentified or untreated CS.
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BACKGROUND: The considerable increase in labiaplasty requires plastic surgeons to be acquainted with the surgical techniques, their respective complications, and satisfaction rates. The authors conducted a systematic review of the available evidence on labia minoraplasty, looking at surgical techniques, clinical outcomes, and patient satisfaction. METHODS: A comprehensive search across PubMed, Web of Science, SCOPUS, and CochraneCENTRAL was performed through October of 2020. A random-effects model meta-analysis was performed to assess satisfaction and complication rates. RESULTS: Forty-six studies including 3804 patients fulfilled the inclusion criteria. The pooled satisfaction rate after labia minoraplasty was 99 percent (95 percent CI, 97 to 99 percent). Substantial heterogeneity was present across studies ( I2 = 63.09 percent; p < 0.001). The highest pooled incidence of dehiscence was reported for laser-assisted labiaplasty (5 percent; 95 percent CI, 2 to 8 percent) and wedge resection (3 percent; 95 percent CI, 1 to 5 perecent). The highest pooled incidence of hematoma formation (8 percent; 95 percent CI, <1 to 23 percent) and postoperative bleeding (2 percent; 95 percent CI, <1 to 15 percent) was reported for W-shape resection. The highest pooled incidence of transient pain or discomfort was reported for de-epithelialization (2 percent; 95 percent CI, <1 to 23 percent) and W-shape resection (2 percent; 95 percent CI, <1 to 15 percent). Three cases of flap necrosis were reported; two occurred using wedge resection labia minoraplasty and one was not specified. The most common causes for revision surgery ( n = 169) were dehiscence and aesthetic concerns. CONCLUSIONS: Labia minoraplasty is a safe procedure, but serious complications requiring surgical management have been reported. Surgical experience, knowledge of female genital anatomy, and thorough technique selection in accordance with the patient characteristics are mandatory.