RESUMO
Junctional epidermolysis bullosa (JEB) is a particularly devastating type of epidermolysis bullosa, especially in the newborn period. Data about the number of new cases of JEB in the United States were collected from the records of the Dystrophic Epidermolysis Bullosa Research Association of America (DebRA) nurse educator. Seventy-one children with JEB were reported to have been born in the 5 years between 2007 and 2011, reflecting an incidence of at least 3.59 per million per year, significantly higher than previously estimated (2.04 per million). There was a high prevalence of morbidity and infant mortality of at least 73%, as 52 of the 71 cases proved fatal by June 2012. These data emphasize the need for future research to develop treatment and ultimately a cure for this disorder.
Assuntos
Epidermólise Bolhosa Juncional/epidemiologia , Epidermólise Bolhosa Juncional/mortalidade , Feminino , Humanos , Incidência , Lactente , Mortalidade Infantil , Recém-Nascido , Masculino , Prevalência , Estudos Retrospectivos , Taxa de Sobrevida , Estados Unidos/epidemiologiaRESUMO
The classification system for vascular anomalies now used by experts worldwide comprises two distinct disease entities that differ in their biologic and pathologic features: vascular tumors and vascular malformations. Vascular tumors include infantile and congenital hemangiomas, tufted angiomas, and kaposiform hemangioendotheliomas. Infantile hemangiomas, the most common vascular anomaly, generally have a predetermined life cycle (proliferation and subsequent involution). GLUT-1, a glucose transporter, is a marker for these specific lesions during all phases of development. Vascular malformations are classified according to their vascular tissue of origin and include capillary, venous, arteriovenous, lymphatic, and mixed malformations. Complex lymphatic malformations and complex mixed malformations, which may have most vascular components, are the most difficult vascular malformations to successfully treat. These lesions are present at birth and often expand or grow in response to trauma, infection, or hormonal changes. Imaging advancements have enabled more accurate assessments and improved management of vascular anomalies. In addition, many lesions are now being managed with targeted pharmacologic therapy. Propranolol and steroids are used for complex or disfiguring tumors, and new anti-angiogenesis inhibitors such as sirolimus are selectively used to treat lymphatic and venous lymphatic malformations that are poorly responsive to sclerotherapy, embolization, and surgical excision. Multimodal therapies are often essential for complex lesions and require the combined expertise of an interdisciplinary team.
Assuntos
Antagonistas Adrenérgicos beta/uso terapêutico , Antineoplásicos/uso terapêutico , Diagnóstico por Imagem/métodos , Glucocorticoides/uso terapêutico , Malformações Vasculares , Procedimentos Cirúrgicos Vasculares/métodos , Humanos , Guias de Prática Clínica como Assunto , Prognóstico , Malformações Vasculares/classificação , Malformações Vasculares/diagnóstico , Malformações Vasculares/terapiaRESUMO
OBJECTIVE: To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB). STUDY DESIGN: We conducted a retrospective chart review of all patients with JEB who came to an interdisciplinary epidermolysis bullosa center at a tertiary care institution between 2004 and 2010. RESULTS: Twenty-five patients with JEB were identified, and 12 patients were seen in the otolaryngology clinic (age range, 2 months-15 years; 8 male, 4 female). Of the 12 patients, 8 underwent rigid laryngoscopy and bronchoscopy for upper respiratory tract symptoms; 7 of these patients displayed laryngeal pathology, and 5 of them underwent surgical intervention with successful resolution of symptoms. Furthermore, none of these patients had any short- or long-term complications from their surgery. A strict protocol and a precise problem-focused cold surgical technique were used in these cases to protect skin and mucus membranes. CONCLUSION: With appropriate precautions, endoscopic laryngeal surgery can be safe and effective in patients with JEB and larygnotracheal disease. Endoscopic laryngeal surgery is feasible when indicated for these patients.
Assuntos
Epidermólise Bolhosa Juncional/complicações , Doenças da Laringe/etiologia , Doenças da Traqueia/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Doenças da Laringe/terapia , Laringoscopia , Masculino , Estudos Retrospectivos , Doenças da Traqueia/terapiaRESUMO
OBJECTIVE: To determine whether pulmonary function decreases as a function of severity of pectus excavatum, and whether reduced function is restrictive or obstructive in nature in a large multicenter study. STUDY DESIGN: We evaluated preoperative spirometry data in 310 patients and lung volumes in 218 patients aged 6 to 21 years at 11 North American centers. We modeled the impact of the severity of deformity (based on the Haller index) on pulmonary function. RESULTS: The percentages of patients with abnormal forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), forced expiratory flow from 25% exhalation to 75% exhalation, and total lung capacity findings increased with increasing Haller index score. Less than 2% of patients demonstrated an obstructive pattern (FEV(1)/FVC <67%), and 14.5% demonstrated a restrictive pattern (FVC and FEV(1) <80% predicted; FEV(1)/FVC >80%). Patients with a Haller index of 7 are >4 times more likely to have an FVC of ≤80% than those with a Haller index of 4, and are also 4 times more likely to exhibit a restrictive pulmonary pattern. CONCLUSIONS: Among patients presenting for surgical repair of pectus excavatum, those with more severe deformities have a much higher likelihood of decreased pulmonary function with a restrictive pulmonary pattern.
Assuntos
Fluxo Expiratório Forçado/fisiologia , Tórax em Funil/diagnóstico , Insuficiência Respiratória/etiologia , Capacidade Vital/fisiologia , Adolescente , Criança , Progressão da Doença , Feminino , Seguimentos , Tórax em Funil/complicações , Tórax em Funil/fisiopatologia , Humanos , Masculino , Prognóstico , Estudos Prospectivos , Radiografia Torácica , Insuficiência Respiratória/diagnóstico , Insuficiência Respiratória/fisiopatologia , Índice de Gravidade de Doença , Espirometria , Tomografia Computadorizada por Raios X , Adulto JovemRESUMO
BACKGROUND: Vascular anomalies comprise a diverse group of diagnoses. While infantile hemangiomas are common, the majority of these conditions are quite rare and have not been widely studied. Some of these lesions, though benign, can impair vital structures, be deforming, or even become life-threatening. Vascular tumors such as kaposiform hemangioendotheliomas (KHE) and complicated vascular malformations have proven particularly difficult to treat. PROCEDURE: Here we retrospectively evaluate a series of six patients with complicated, life-threatening vascular anomalies who were treated with the mTOR inhibitor sirolimus for compassionate use at two centers after failing multiple other therapies. RESULTS: These patients showed significant improvement in clinical status with tolerable side effects. CONCLUSIONS: Sirolimus appears to be effective and safe in patients with life-threatening vascular anomalies and represents an important tool in treating these diseases. These findings are currently being further evaluated in a Phase II safety and efficacy trial.
Assuntos
Imunossupressores/uso terapêutico , Sirolimo/uso terapêutico , Malformações Vasculares/complicações , Malformações Vasculares/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Ensaios de Uso Compassivo , Feminino , Humanos , Imunossupressores/efeitos adversos , Lactente , Masculino , Estudos Retrospectivos , Sirolimo/efeitos adversos , Resultado do TratamentoRESUMO
Epidermolysis bullosa (EB) has become recognized as a multisystem disorder that poses a number of pre-, intra-, and postoperative challenges. While anesthesiologists have long appreciated the potential difficult intubation in patients with EB, other systems can be affected by this disorder. Hematologic, cardiac, skeletal, gastrointestinal, nutritional, and metabolic deficiencies are foci of preoperative medical care, in addition to the airway concerns. Therefore, multidisciplinary planning for operative care is imperative. A multinational, interdisciplinary panel of experts assembled in Santiagio, Chile to review the best practices for perioperative care of patients with EB. This paper presents guidelines that represent a synthesis of evidence-based approaches and the expert consensus of this panel and are intended to aid physicians new to caring for patients with EB when operative management is indicated. With proper medical optimization and attention to detail in the operating room, patients with EB can have an uneventful perioperative course.
Assuntos
Epidermólise Bolhosa/complicações , Assistência Perioperatória , Anestesia por Inalação , Epidermólise Bolhosa/patologia , Epidermólise Bolhosa/terapia , Humanos , Salas Cirúrgicas , Dor Pós-Operatória/terapia , Cuidados Pós-Operatórios , Sistema Respiratório/patologiaRESUMO
Dilated cardiomyopathy (DC) has been reported in severe epidermolysis bullosa (EB) subtypes. Poor nutritional status, low carnitine levels, selenium deficiency, chronic iron overload, drugs and viral etiology have been proposed as potential contributors. This was a retrospective, descriptive, multicenter study describing EB patients that developed DC, and determining potential pre-disposing risk factors. Fifteen patients were enrolled in the study; 11 of them were male subjects (73%). Eighty-seven per cent of the participants had dystrophic EB and 13% had junctional EB. Mean age at diagnosis of DC was 12.18 +/- 4.99 years. Chronic anemia was diagnosed in 13 of 15 patients (86.7%). Sixty per cent of patients had prior red blood cell transfusions. At diagnosis, selenium levels were low in 55% of the patients (n = 11) and total carnitine levels were low in 45% of the patients (n = 11). Systolic function was moderately impaired, with a mean shortening fraction of 19.38% (SD = 5.04, n = 8). After a mean follow-up period of 6.3 +/- 4.8 years, six patients were alive without being on any medications (40.0%), two were alive on medications (13.3%) and seven had died (46.7%). Limitations of the study was that it was a retrospective chart review with relatively small sample size. Retrospective chart review, relatively small sample size. This study substantiates the association between DC and EB. Currently, there is no single risk factor identified in EB patients that leads to DC. Further prospective studies are needed.
Assuntos
Cardiomiopatia Dilatada/diagnóstico , Cardiomiopatia Dilatada/etiologia , Epidermólise Bolhosa/complicações , Adolescente , Anemia/diagnóstico , Cardiomiopatia Dilatada/tratamento farmacológico , Carnitina/deficiência , Criança , Pré-Escolar , Doença Crônica , Epidermólise Bolhosa/tratamento farmacológico , Transfusão de Eritrócitos/efeitos adversos , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Fatores de Risco , Selênio/deficiência , Sístole/fisiologia , Adulto JovemRESUMO
Esophageal atresia/tracheoesophageal fistula (EA/TEF) repair using an open muscle-sparing thoracotomy has been the standard approach used in our institution. Whereas perioperative mortality is now very uncommon, short- and long-term morbidity is very common in these patients. However, the complexity of the esophageal anatomy and significant comorbidities appear to be important contributors to significant complications in these patients. At least 30% of the EA/TEF patients required esophageal dilatations for anastomotic stricture; this increased to 50% for patients with pure EA. Gastroesophageal reflux requiring an antireflux procedure was performed 23% of the time for EA/TEF and 30% for EA patients. In addition, there were a few complications, such as winging of the scapula and scoliosis, that were attributed in part to the utilization of a nonmuscle-sparing thoracotomy. The standard muscle-sparing thoracotomy remains a very versatile and useful approach to repairing esophageal atresia, and it is the standard for repairing more complex anatomical variants. The self-reported long-term quality of life in these patients is very good, except for a few individuals with protracted feeding disorders and severe dysphagia.
Assuntos
Atresia Esofágica/cirurgia , Complicações Pós-Operatórias/terapia , Toracotomia , Fístula Traqueoesofágica/cirurgia , Humanos , Lactente , Recém-Nascido , Complicações Pós-Operatórias/diagnóstico , Qualidade de Vida , Estudos RetrospectivosRESUMO
BACKGROUND: The utility of laparoscopic appendectomy (LA) in children remains controversial. The determination of the efficacy of LA in children is complicated by variable postoperative management, duration of antibiotics,and criteria for discharge. The aim of this study was to examine the results of a commitment to LA and the concurrent implementation of an evidence-based clinical pathway (CP) for management appendicitis in a children's hospital. METHODS: With institutional review board approval, all children presenting with appendicitis (n = 72; age =10.6 +/- 0.1 years) were offered LA and management directed by CP. Data were accrued prospectively for 12 consecutive months (May 2006 to April 2007) and analysis performed at 15 months. Data are reported as the mean +/- standard error of the mean. RESULTS: Children were stratified based on the operative findings: group one - acute 41; group two-suppurative=11; and group 3-gangrenous or perforated 20. Duration of hospital stay differed between the groups:group one= 26 +/- 0.3 hours; group 2 =48 +/- 3 hours; group 3= 127 +/- 6 hours (P <0.05). No patients in groups one or two suffered a complication or were readmitted following discharge. Two patients in group 3 (10%)were readmitted and treated with antibiotic therapy alone. Overall, 66% of the children with acute appendicitis(27/41) and 27% with suppurative appendicitis (3/11) were discharged within 24 hours of admission. Discharge by 24 hours in groups 1 and 2 was not influenced by age, gender, or time of operation (before or after 7 PM). CONCLUSIONS: The commitment to LA and use of CP resulted in discharge within 24 hours in 2 of 3 of children with acute appendicitis without readmission or complications being observed. Early discharge was not influenced by age, gender, or time of admission. For advanced appendicitis, length of hospital stay, determined by clinical parameters, resulted in a low rate of complication or readmission.
Assuntos
Apendicectomia/métodos , Apendicectomia/reabilitação , Procedimentos Clínicos , Laparoscopia , Tempo de Internação , Apendicite/cirurgia , Criança , Medicina Baseada em Evidências , Feminino , Humanos , Masculino , Alta do Paciente , SupuraçãoRESUMO
BACKGROUND: Given widespread adoption of the Nuss procedure, prospective multicenter study of management of pectus excavatum by both the open and Nuss procedures was thought desirable. Although surgical repair has been performed for more than 50 years, there are no prospective multicenter studies of its management. STUDY DESIGN: This observational study followed pectus excavatum patients treated surgically at 11 centers in North America, according to the method of choice of the patient and surgeon. Before operation, all underwent evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, hospital complications, and perioperative pain. One year after completion of treatment, patients will repeat the preoperative evaluations. This article addresses early results only. RESULTS: Of 416 patients screened, 327 were enrolled; 284 underwent the Nuss procedure and 43 had the open procedure. Median preoperative CT index was 4.4. Pulmonary function testing before operation showed mean forced vital capacity of 90% of predicted values; forced expiratory volume in 1 second (FEV(1)), 89% of predicted; and forced expiratory flow during the middle half of the forced vital capacity (FEF(25% to 75%)), 85% of predicted. Early postcorrection results showed that operations were performed without mortality and with minimal morbidity at 30 days postoperatively. Median hospital stay was 4 days. Postoperative pain was a median of 3 on a scale of 10 at time of discharge; the worst pain experienced was the same as was expected by the patients (median 8), and by 30 days after correction or operation, the median pain score was 1. Because of disproportionate enrollment and similar early complication rates, statistical comparison between operation types was limited. CONCLUSIONS: Anatomically severe pectus excavatum is associated with abnormal pulmonary function. Initial operative correction performed at a variety of centers can be completed safely. Perioperative pain is successfully managed by current techniques.
Assuntos
Tórax em Funil/cirurgia , Dor Pós-Operatória/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Tórax em Funil/fisiopatologia , Humanos , Internet , Masculino , Procedimentos Ortopédicos/efeitos adversos , Procedimentos Ortopédicos/métodos , Complicações Pós-Operatórias/epidemiologia , Estudos Prospectivos , Testes de Função RespiratóriaRESUMO
Vascular malformations are the second major category of vascular anomalies. In contrast to vascular tumors, they are present at birth and grow commensurately with the child. Although the molecular mechanisms underlying the formation of these lesions remain unclear, lesions are known to result from abnormal development and morphogenesis. Histologic examination of vascular malformations shows no evidence of cellular proliferation, but rather progressive dilation of abnormal channels. Vascular malformations are designated according to their predominant channel type; they may be capillary, venous, lymphatic, arterial, and combined malformations. Malformations with an arterial component are rheologically fast-flow, whereas capillary, lymphatic, and venous malformations are slow-flow in nature. The morbidity of vascular malformations varies greatly both within and among the clinical subgroups cited above. This article describes the clinical presentation, diagnosis, and management of vascular malformations. The more frequently encountered clinical presentations involving the head and neck are highlighted.
Assuntos
Capilares/anormalidades , Sistema Linfático/anormalidades , Veias/anormalidades , Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas/terapia , Capilares/cirurgia , Criança , Humanos , Sistema Linfático/cirurgia , Mancha Vinho do Porto/diagnóstico , Mancha Vinho do Porto/terapia , Escleroterapia , Síndrome de Sturge-Weber/diagnóstico , Síndrome de Sturge-Weber/terapia , Telangiectasia Hemorrágica Hereditária/diagnóstico , Telangiectasia Hemorrágica Hereditária/terapia , Veias/cirurgiaRESUMO
BACKGROUND AND OBJECTIVES: Complicated vascular anomalies have limited therapeutic options and cause significant morbidity and mortality. This Phase II trial enrolled patients with complicated vascular anomalies to determine the efficacy and safety of treatment with sirolimus for 12 courses; each course was defined as 28 days. METHODS: Treatment consisted of a continuous dosing schedule of oral sirolimus starting at 0.8 mg/m(2) per dose twice daily, with pharmacokinetic-guided target serum trough levels of 10 to 15 ng/mL. The primary outcomes were responsiveness to sirolimus by the end of course 6 (evaluated according to functional impairment score, quality of life, and radiologic assessment) and the incidence of toxicities and/or infection-related deaths. RESULTS: Sixty-one patients were enrolled; 57 patients were evaluable for efficacy at the end of course 6, and 53 were evaluable at the end of course 12. No patient had a complete response at the end of course 6 or 12 as anticipated. At the end of course 6, a total of 47 patients had a partial response, 3 patients had stable disease, and 7 patients had progressive disease. Two patients were taken off of study medicine secondary to persistent adverse effects. Grade 3 and higher toxicities attributable to sirolimus included blood/bone marrow toxicity in 27% of patients, gastrointestinal toxicity in 3%, and metabolic/laboratory toxicity in 3%. No toxicity-related deaths occurred. CONCLUSIONS: Sirolimus was efficacious and well tolerated in these study patients with complicated vascular anomalies. Clinical activity was reported in the majority of the disorders.
Assuntos
Imunossupressores/uso terapêutico , Sirolimo/uso terapêutico , Malformações Vasculares/tratamento farmacológico , Administração Oral , Adolescente , Adulto , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Imunossupressores/sangue , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Qualidade de Vida , Sirolimo/sangue , Adulto JovemRESUMO
BACKGROUND: In 1978, Drs. Fischer and Martin were among the first to preserve anorectal continence and create a pelvic reservoir in adult patients, in what has become the ileal pouch-anal anastomosis (IPAA). METHODS: Here we review our institutions' experience with 379 of these procedures from 1978 to present. To assess the specific health concerns of patients with ulcerative colitis (UC) and familial adenomatous polyposis (FAP) and determine the effects of IPAA on health-related quality of life, we evaluated patients with the SF-36, the Rating Form of Inflammatory Bowel Disease Patient Concerns (RFIPC), time trade-off questions, and a gamble question. RESULTS: IPAA patients did not differ from the general population in seven of eight general health categories assessed by the SF-36. When compared with the UC population as a whole using the RFIPC they had reduced concerns in almost all areas. In addition, time trade-off and gamble questions indicated that these patients, as a group, are willing to accept a significant risk of dying in order to achieve their results of the IPAA. CONCLUSIONS: This high level of satisfaction has led to the referral of patients who would not have otherwise considered a procedure requiring permanent ileostomy.
Assuntos
Canal Anal/cirurgia , Íleo/cirurgia , Proctocolectomia Restauradora/psicologia , Qualidade de Vida , Polipose Adenomatosa do Colo/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Anastomose Cirúrgica , Colite Ulcerativa/cirurgia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Avaliação de Resultados em Cuidados de Saúde , Proctocolectomia Restauradora/tendências , Inquéritos e QuestionáriosRESUMO
OBJECTIVE: Our aim was to present our experience with complications caused by placement of metallic stents in the pediatric airway. DESIGN AND SETTING: We conducted a retrospective study of the medical records of patients with complications resulting from metallic stent placement, managed by the senior authors between 1993 and 2002. RESULTS: Nine children had complications associated with the placement of metallic airway stents. Of these, 8 children required stent removal. Granulation tissue and tracheal stenosis were seen in all 7 children with long standing stent placement. There was 1 stent death in this series. CONCLUSIONS: Metallic airway stents can cause significant complications in the pediatric airway. These complications may supersede the airway compromise that necessitated their initial placement. As such, metallic stent placement should be approached with caution. The likelihood and severity of complications increase with time, as do the difficulties encountered upon removal. The proportion of patients in whom metallic stents may be placed "permanently" without complications is not known. Therefore we recommend that metallic airway stents be considered a temporizing measure of limited duration.
Assuntos
Stents/efeitos adversos , Traqueia , Doenças da Traqueia/terapia , Pré-Escolar , Feminino , Tecido de Granulação/patologia , Humanos , Lactente , Recém-Nascido , Masculino , Metais , Insuficiência Respiratória/terapia , Estudos Retrospectivos , Traqueia/patologia , Estenose Traqueal/etiologiaRESUMO
The most common hepatic vascular tumor in the pediatric population is the infantile hepatic hemangioma. Although these lesions have a spectrum of presentations, there are three main subtypes that have been described-focal, multifocal, and diffuse. An algorithm on the workup, treatment, and follow-up of these lesions can be based on this categorization. Recent shifts in the management of hemangiomas with beta-blockers (propranolol) have also influenced the treatment of hepatic hemangiomas. This article reviews the current understanding of hepatic hemangiomas and protocols in the management of these patients.
Assuntos
Hemangioma/patologia , Hemangioma/terapia , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/terapia , Humanos , LactenteRESUMO
BACKGROUND: Despite randomized controlled trials and meta-analyses, it remains unclear whether laparoscopic pyloromyotomy (LP) carries a higher risk of incomplete pyloromyotomy and mucosal perforation compared with open pyloromyotomy (OP). METHODS: Multicenter study of all pyloromyotomies (May 2007-December 2010) at nine high-volume institutions. The effect of laparoscopy on the procedure-related complications of incomplete pyloromyotomy and mucosal perforation was determined using binomial logistic regression adjusting for differences among centers. RESULTS: Data relating to 2830 pyloromyotomies (1802 [64%] LP) were analyzed. There were 24 cases of incomplete pyloromyotomy; 3 in the open group (0.29%) and 21 in the laparoscopic group (1.16%). There were 18 cases of mucosal perforation; 3 in the open group (0.29%) and 15 in the laparoscopic group (0.83%). The regression model demonstrated that LP was a marginally significant predictor of incomplete pyloromyotomy (adjusted difference 0.87% [95% CI 0.006-4.083]; P=0.046) but not of mucosal perforation (adjusted difference 0.56% [95% CI -0.096 to 3.365]; P=0.153). Trainees performed a similar proportion of each procedure (laparoscopic 82.6% vs. open 80.3%; P=0.2) and grade of primary operator did not affect the rate of either complication. CONCLUSIONS: This is one of the largest series of pyloromyotomy ever reported. Although laparoscopy is associated with a statistically significant increase in the risk of incomplete pyloromyotomy, the effect size is small and of questionable clinical relevance. Both OP and LP are associated with low rates of mucosal perforation and incomplete pyloromyotomy in specialist centers, whether trainee or consultant surgeons perform the procedure.
Assuntos
Mucosa Intestinal/lesões , Perfuração Intestinal/etiologia , Laparoscopia/efeitos adversos , Complicações Pós-Operatórias/etiologia , Estenose Pilórica/cirurgia , Piloro/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
BACKGROUND: A multicenter study of pectus excavatum was described previously. This report presents our final results. STUDY DESIGN: Patients treated surgically at 11 centers were followed prospectively. Each underwent a preoperative evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, complications, and perioperative pain. One year after treatment, patients underwent repeat chest CT scan, pulmonary function tests, and body image survey. A subset of 50 underwent exercise pulmonary function testing. RESULTS: Of 327 patients, 284 underwent Nuss procedure and 43 underwent open procedure without mortality. Of 182 patients with complete follow-up (56%), 18% had late complications, similarly distributed, including substernal bar displacement in 7% and wound infection in 2%. Mean initial CT scan index of 4.4 improved to 3.0 post operation (severe >3.2, normal = 2.5). Computed tomography index improved at the deepest point (xiphoid) and also upper and middle sternum. Pulmonary function tests improved (forced vital capacity from 88% to 93%, forced expiratory volume in 1 second from 87% to 90%, and total lung capacity from 94% to 100% of predicted (p < 0.001 for each). VO2 max during peak exercise increased by 10.1% (p = 0.015) and O2 pulse by 19% (p = 0.007) in 20 subjects who completed both pre- and postoperative exercise tests. CONCLUSIONS: There is significant improvement in lung function at rest and in VO2 max and O2 pulse after surgical correction of pectus excavatum, with CT index >3.2. Operative correction significantly reduces CT index and markedly improves the shape of the entire chest, and can be performed safely in a variety of centers.
Assuntos
Tórax em Funil/cirurgia , Procedimentos Ortopédicos , Adolescente , Imagem Corporal , Criança , Teste de Esforço , Feminino , Seguimentos , Tórax em Funil/diagnóstico por imagem , Tórax em Funil/fisiopatologia , Tórax em Funil/psicologia , Humanos , Masculino , Complicações Pós-Operatórias/epidemiologia , Estudos Prospectivos , Testes Psicológicos , Testes de Função Respiratória , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
The Nuss procedure is a surgical repair technique for pectus excavatum with fewer delayed complications reported in the current literature. We report the case of a 23-year-old male who presented after a syncopal episode during activity. Further evaluation demonstrated a fibrous band causing severe right ventricle outflow obstruction from a prior Nuss procedure.
Assuntos
Tórax em Funil/cirurgia , Hipertrofia Ventricular Direita/etiologia , Procedimentos Cirúrgicos Minimamente Invasivos , Procedimentos Ortopédicos , Complicações Pós-Operatórias , Síncope/etiologia , Humanos , Hipersensibilidade/complicações , Hipersensibilidade/etiologia , Hipertrofia Ventricular Direita/diagnóstico , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos/instrumentação , Níquel/efeitos adversos , Procedimentos Ortopédicos/instrumentação , Complicações Pós-Operatórias/diagnóstico , Próteses e Implantes/efeitos adversos , Adulto JovemRESUMO
Epidermolysis bullosa (EB) is a spectrum of rare, inherited, blistering skin disorders, primarily affecting the skin and pharyngoesophageal mucosa. EB affects approximately 2 to 4 per 100,000 children each year. Blistering and scarring occur in response to even the most minor trauma. In this article, the authors outline the potential management options for patients with EB complicated by feeding difficulties secondary to esophageal strictures as well as those with nutritional deficiencies requiring a gastrostomy tube for supplemental feeding.