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1.
Plast Reconstr Surg ; 146(4): 400e-408e, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32969994

RESUMO

BACKGROUND: Young women with congenital breast asymmetry have impaired psychological well-being and self-esteem. However, little is known regarding the effects of surgical intervention in this population. This cohort study aims to assess postoperative changes in health-related quality of life following surgical treatment of breast asymmetry in young women using a prospective, longitudinal study design. METHODS: From 2008 to 2018, 45 young women undergoing surgical correction of breast asymmetry of benign cause and 101 unaffected, female controls completed the following surveys: Short-Form 36v2, Rosenberg Self-Esteem Scale, and Eating-Attitudes Test-26. Surveys were administered at baseline and at up to 9-year follow-up. RESULTS: Participants with breast asymmetry scored significantly worse than controls at baseline on the Rosenberg Self-Esteem Scale and in two Short-Form 36v2 domains: Social-Functioning and Role-Emotional. Asymmetry participants experienced significant postoperative improvements on the Rosenberg Self-Esteem Scale, and in three Short-Form 36v2 domains: Role-Physical, Social Functioning, and Mental Health. These improvements were sustained for a minimum of 5 years. Postoperatively, asymmetry participants' quality of life was comparable to controls and did not vary by age at the time of surgery, asymmetry severity, or diagnosis. CONCLUSIONS: Surgical treatment of breast asymmetry in young women yields significant and sustained improvements in psychosocial quality of life. Postoperatively, patients returned to a level of functioning commensurate with their peers. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.


Assuntos
Mama/anormalidades , Mama/cirurgia , Mamoplastia , Qualidade de Vida , Adolescente , Estudos de Coortes , Anormalidades Congênitas/psicologia , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Estudos Longitudinais , Estudos Prospectivos , Autorrelato , Resultado do Tratamento , Adulto Jovem
4.
Adv Clin Exp Med ; 29(4): 505-511, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32348039

RESUMO

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also known as Müllerian agenesis or aplasia, is a congenital disease manifested by the aplasia of the uterus and the upper 2/3 of the vagina; its incidence is 1 in 4,000-5,000 female live births. We can distinguish 2 types of the MRKH syndrome: type I, which is characterized by an isolated absence of 2/3 of the vagina and uterus; and type II or MURCS (Müllerian duct aplasia, unilateral renal agenesis and cervicothoracic somite anomalies), which is also associated with other symptoms. The treatment of the MRKH syndrome patients aims at creating a neovagina and enabling sexual intercourse. Non-surgical techniques are the first-choice methods, and more than 90% of patients notice an anatomical and functional improvement if they are well-prepared emotionally. If non-surgical treatment does not bring about the expected results, a surgical procedure remains an option. The surgical method is mainly determined by the surgeon's experience. There are a few types of operations, though none of them seems superior to others. The next challenge is to provide these patients with a chance to become parents. Nowadays, a uterine transplant, a surrogate or adoption are the available solutions. An interdisciplinary approach is required, and the treatment should consist of medical and psychological support. This review presents the current knowledge about the MRKH syndrome with regard to the current methods of non-surgical and surgical treatment as well as a summary of the associated psychological problems.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual , Anormalidades Congênitas , Ductos Paramesonéfricos/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico , Transtornos 46, XX do Desenvolvimento Sexual/genética , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/genética , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Útero/anormalidades , Vagina/anormalidades
5.
World Neurosurg ; 139: e496-e500, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32311554

RESUMO

OBJECTIVE: Symptomatic Atlanto-axial (C1-2) osteoarthritis (AAOA) is a common phenomenon in elderly patients; however, there is a paucity of data on the effectiveness of posterior atlanto-axial fusion (PAAF) for this condition. To this end, here we assess changes in patient-reported outcomes and neck-related disability in adult patients undergoing PAAF for symptomatic C1-2 AAOA. METHODS: In this retrospective study, the clinical records of consecutive patients with symptomatic AAOA who underwent PAAF between 2004 and 2017 were reviewed. Patient demographics, comorbidities, intraoperative and postoperative variables, and complication rates were collected. Neck Disability Index (NDI) scores were recorded at baseline and 6 weeks, 6 months, 1 year, and 2 years postoperatively. RESULTS: Forty-two patients (average age, 72.04 ± 8.56 years; 26.19% males) met the study's inclusion criteria. In this cohort, 19.04% had previous subaxial cervical spine surgery, 35.71% had a history of smoking (all had stopped smoking before surgery), and 11.90% had type II diabetes. At baseline, the majority of patients had a normal neurologic exam. The average preoperative NDI score was 26.88 ± 24.85, which improved to 10.59 ± 14.88 at the 1-year follow-up and 13.20 ± 14.96 at the 2-year follow-up (P = 0.004). At baseline, 18% of the patients reported severe disability based on NDI score; this percentage decreased to 2% at 1 year and 0 at 2 years (P = 0.01). Importantly, a high percentage (11.90%) of patients had undergone previous subaxial cervical fusion for their pain due to a mistaken diagnosis for this condition, without symptom relief. CONCLUSIONS: In appropriately selected patients, PAAF may decrease neck pain and improve functional disability in patients with AAOA. Future prospective longitudinal studies are needed to corroborate these findings.


Assuntos
Articulação Atlantoaxial/anormalidades , Anormalidades Congênitas/cirurgia , Cervicalgia/cirurgia , Osteoartrite/cirurgia , Fusão Vertebral/métodos , Idoso , Idoso de 80 Anos ou mais , Articulação Atlantoaxial/cirurgia , Comorbidade , Diabetes Mellitus Tipo 2/complicações , Avaliação da Deficiência , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Autorrelato , Fumar/efeitos adversos , Resultado do Tratamento
6.
Fertil Steril ; 113(4): 723-732, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32228875

RESUMO

The prevalence of congenital cervical agenesis or dysgenesis ranges from 1/80,000 to 1/100,000, and in about 50% of these cases it coexists with congenital vaginal agenesis. This narrative review summarizes the contemporary knowledge in the field of conservative surgical restoration of the reproductive tract. The management of congenital cervical malformations aims to [1] provide relief from the obstructive symptoms, [2] establish normal sexual function, and [3] preserve the uterus for future fertility. In cases of cervical agenesis and vaginal aplasia, the surgical approach involves the creation of neovagina, the creation of neocervix, and then subsequent restoration of the continuity of the genital tract. In cases where vagina is not congenitally absent, the surgical approach involves either a direct uterovaginal anastomosis or initial creation of neocervix and then subsequent restoration of the continuity of the genital tract. The neocervix can be surgically created with small intestinal submucosa, split-thickness skin graft, full-thickness skin graft, peritoneal flap, or vaginal mucosa lined with a polytetrafluoroethylene graft. Most of the published cases report long-term menstruation and sporadic pregnancies. Conservative surgery of cervical congenital malformations could serve as a first-line treatment. Sexual function and menstruation are established in the majority of patients. Extirpatory surgery may be preserved for surgical failures after initial restoration of the continuity of uterus-cervix-vagina or in cases with more complex anatomy.


Assuntos
Colo do Útero/anormalidades , Colo do Útero/cirurgia , Gerenciamento Clínico , Procedimentos Cirúrgicos Reconstrutivos/métodos , Anormalidades Urogenitais/cirurgia , Útero/anormalidades , Colo do Útero/diagnóstico por imagem , Anormalidades Congênitas/diagnóstico por imagem , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Laparoscopia/métodos , Gravidez , Retalhos Cirúrgicos , Anormalidades Urogenitais/diagnóstico por imagem , Útero/diagnóstico por imagem , Útero/cirurgia , Vagina/anormalidades , Vagina/diagnóstico por imagem , Vagina/cirurgia
7.
J Pediatr Adolesc Gynecol ; 33(4): 410-414, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32224249

RESUMO

STUDY OBJECTIVE: This study aimed to evaluate the reproductive potential of patients with Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) who were candidates for uterus transplantation (UTx) before inclusion in the experimental trial, and to summarize the existing experience with posttransplantation embryo transfers in functionally successful cases. DESIGN AND SETTING: A prospective study at a tertiary medical center. PARTICIPANTS: Ten pre-UTx women with MRKHS and 7 successful UTx cases. INTERVENTIONS AND MAIN OUTCOME MEASURES: Ovarian stimulations, frozen embryo collection, embryo transfers, and incidence of pregnancy in MRKHS women in the pre- and posttransplantation periods. RESULTS: The average number of ovarian stimulations to collect the required frozen embryos was 1.9 (1-3). On average, the number of aspirated oocytes was 16.4 (7-38), 12.6 (5-26) oocytes were fertilized, and 7.0 (1-18) embryos were cryopreserved per cycle. To date, the average number of embryo transfers per recipient was 4.9 (3-8), and the embryo transfer/pregnancy rate was 8.8% (3 of 34). Three pregnancies have been achieved to date, including a missed abortion in the 8th week, a birth in the 35th week, and an ongoing pregnancy in the 30th week. CONCLUSIONS: Despite the small number of UTx cases, our data indicated that women with MRKHS showed a good response to ovarian stimulation and blastocyst formation. Although the procedures for fertilization, cryopreservation, and transfer of the embryos were standardized, the success rate per embryo transfer achieved in the study group has been low to date.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Ductos Paramesonéfricos/anormalidades , Taxa de Gravidez , Útero/transplante , Adulto , República Tcheca , Transferência Embrionária/estatística & dados numéricos , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Recuperação de Oócitos/estatística & dados numéricos , Indução da Ovulação/estatística & dados numéricos , Gravidez , Estudos Prospectivos
8.
Fertil Steril ; 113(3): 681-682, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32192600

RESUMO

OBJECTIVE: To introduce an innovation that combines single port laparoscopic and Wharton-Sheares-George cervicovaginal reconstruction using a small intestinal submucosa (SIS) graft in a patient with congenital cervical atresia, absence of vagina, and incomplete uterine septum (U2aC4V4). DESIGN: Video article introducing a new surgical technique. SETTING: University hospital. PATIENT(S): A 15-year-old patient with congenital cervical atresia, absence of vagina, and incomplete uterine septum had primary amenorrhea and cyclic lower abdominal pain. The magnetic resonance imaging did not show hematometra and the endometrium was 6 mm when she had lower abdominal pain. INTERVENTION(S): A neovagina (depth, 7 cm; width, 2.5 cm) was created using the Wharton-Sheares-George neovaginoplasty. By single laparoscopy, the bladder was separated from the anterior surface of the uterus. With the mold in the neovagina created by the assistant, the apex of neovagina was opened. Then the lower uterine segment was exposed and incised. A T-shaped intrauterine device was connected to an 8-cm-long catheter scissored from a 14-F Foley catheter and was inserted into the uterus to prevent cervical or vaginal stenosis. The upper end of the graft was applied onto the lower uterine segment with delayed absorbable sutures. The lower end was sutured to the high vaginal or vestibular mucosa. MAIN OUTCOME MEASURE(S): The feasibility and effect of combination single port laparoscopic with vaginal cervicovaginal reconstruction in the congenital atresia of cervix. RESULT(S): The operation was successful. The operating time was 90 minutes. Hospitalization was 3 days. There were no intraoperative and postoperative complications. The patient had resumption of menses at three cycles postoperatively, and she had no dysmenorrhea. No cervical or vaginal stenosis occurred because of the Foley catheter. CONCLUSION(S): Single port laparoscopic combined with vaginal cervicovaginal reconstruction provided a minimally invasive, safe, and effective surgical option for the young patient with congenital atresia of cervix. It was successful and without complications or cervical or vaginal stenosis.


Assuntos
Colo do Útero/anormalidades , Colo do Útero/cirurgia , Anormalidades Congênitas/cirurgia , Laparoscopia/métodos , Procedimentos Cirúrgicos Reconstrutivos/métodos , Útero/cirurgia , Vagina/anormalidades , Adolescente , Amenorreia/etiologia , Amenorreia/cirurgia , Colo do Útero/patologia , Anormalidades Congênitas/patologia , Feminino , Procedimentos Cirúrgicos em Ginecologia/métodos , Humanos , Mucosa Intestinal/patologia , Mucosa Intestinal/transplante , Intestino Delgado/patologia , Intestino Delgado/transplante , Dor Pélvica/etiologia , Dor Pélvica/cirurgia , Estruturas Criadas Cirurgicamente , Anormalidades Urogenitais/complicações , Anormalidades Urogenitais/cirurgia , Vagina/patologia , Vagina/cirurgia
9.
J Pediatr Surg ; 55(5): 811-820, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32061363

RESUMO

PURPOSE: This study aims to compare the prevalence and outcomes of surgically correctable congenital anomalies between sexes. METHODS: Upon registration on PROSPERO (CRD42019120165), a librarian aided in conducting a systematic review using PRISMA guidelines. The five largest relevant studies were included for each anomaly. Cumulative prevalence differences and confidence intervals were calculated, and the Cochran-Mantel-Haenszel test was performed. RESULTS: Of 42,722 identified studies, 68 were included in our analysis. All included anomalies had greater than 1000 patients except duodenal atresia (n = 787) and intestinal duplication (n = 148). Males had a significantly higher prevalence than females in 10/14 anomalies (Hirschsprung's disease, omphalomesenteric duct, congenital diaphragmatic hernia, anorectal malformation, malrotation, esophageal atresia, congenital pulmonary airway malformation, intestinal atresia, omphalocele, and gastroschisis; p < 0.001). There was no difference in the prevalence of duodenal atresia or intestinal duplication between sexes (p = 0.88 and 0.65, respectively). Females had a significantly higher prevalence of biliary anomalies (atresia and choledochal cyst). CONCLUSION: Our study indicates that males have higher prevalence rates of most congenital anomalies. Further investigations are required to illuminate the embryology underlying this sex distribution and whether sex influences outcomes. TYPE OF STUDY: Systematic review and meta-analysis. LEVEL OF EVIDENCE: Prognostic study, level II.


Assuntos
Anormalidades Congênitas , Anormalidades Congênitas/epidemiologia , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Fatores Sexuais
10.
Int J Pediatr Otorhinolaryngol ; 131: 109886, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31968273

RESUMO

INTRODUCTION: Injection laryngoplasty (IL) is a brief, minimally invasive procedure involving injection of agents to augment the interarytenoid space. It was initially described as a diagnostic and temporizing measure for management of type one laryngeal clefts (LC1) and associated swallowing dysfunction (SwD), but more gradually it is being proposed as a definitive treatment modality. However, the morbidity of this treatment for LC1 remains under-investigated. This study sought to determine the morbidities associated with IL as a treatment modality for LC1 and associated SwD. METHOD: Single centre retrospective review of a prospective surgical database of one Pediatric Otolaryngologist at a tertiary care center. Participants included pediatric patients with an endoscopic diagnosis of LC1, treated with IL between 2000 and 2018-excluding those with concurrent upper airway anomalies. Patient charts were reviewed for demographic information, immediate post-op complications (within the first 14 days following IL), and subsequent management. RESULTS: Out of 85 consecutive, eligible patients, 12 were excluded (5 subglottic stenosis, 6 laryngomalacia, and one tracheomalacia). Of the 73 included patients, 42 were male and 31 females. The median age at IL was 29 months (range 1-132, interquartile range of 38 months). All IL procedures in this study utilized hyaluronic acid derivatives. From this series, 13 patients experienced post-operative complications. The complications encountered were respiratory distress (N = 5), croup-like cough (N = 6), and stridor (N = 6). These complications were either self-limiting (N=9), managed by systemic or inhaled steroids (N = 4), or admitted to hospital for monitoring (N=3). One case (augmented with dextranomer and hyaluronic acid) required intubation, repeat endoscopy, and drainage of seroma. CONCLUSION: IL was followed by respiratory morbidity in nearly two in ten of this series of consecutive patients. All the morbidities occurred in association with one injection product. Parents should be counselled appropriately about potential morbidities associated with this procedure.


Assuntos
Anormalidades Congênitas/cirurgia , Dextranos/administração & dosagem , Dextranos/efeitos adversos , Ácido Hialurônico/administração & dosagem , Ácido Hialurônico/efeitos adversos , Laringoplastia/efeitos adversos , Laringe/anormalidades , Complicações Pós-Operatórias/epidemiologia , Criança , Pré-Escolar , Endoscopia , Feminino , Humanos , Lactente , Injeções , Laringoplastia/métodos , Laringe/cirurgia , Masculino , Estudos Retrospectivos
11.
Int J Pediatr Otorhinolaryngol ; 131: 109844, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31901483

RESUMO

OBJECTIVE: Identify incidence and factors associated with respiratory complications after type 1 cleft repair. METHODS: Retrospective chart review of patients who underwent cleft repair over a 5-year period performed by a single surgeon. Primary endpoint was respiratory complications (oxygen desaturation <90%). Fisher's exact test was used to identify differences between repair types (endoscopic carbon dioxide laser-assisted repair and injection laryngoplasty). Logistic regression was used to identify predictors of respiratory events. RESULTS: Fifty-five patients were included. Thirty-four (62%) patients underwent endoscopic carbon dioxide laser-assisted repair and 21 (38%) underwent injection laryngoplasty. Average hospital stay for each group was 1.6 days (SD = 3.1) and 0.6 days (SD = 0.9), respectively. Desaturations occurred in three patients (9%) in the laser-assisted repair group and one patient (4%) in the injection group. All occurred within 3 h after surgery and resolved with supplemental oxygen, oral airway placement, and/or mask ventilation. Two affected patients had comorbid diagnosis of asthma (one had poor medication compliance), and one had a history of developmental delay and hypotonia. In the injection group, desaturations occurred in one patient with a history of tracheal stenosis and double aortic arch. No correlation existed between repair type and desaturation (p = 0.57). No variables were significant predictors of events. CONCLUSIONS: In this cohort, respiratory events after type 1 laryngeal cleft repair occurred early in the postoperative period, in children with cardiac and pulmonary comorbidities. This suggests postoperative admission may only be necessary for a select group of patients undergoing type 1 cleft repair. However, further research is needed to determine criteria for same-day discharge.


Assuntos
Anormalidades Congênitas/cirurgia , Laringoplastia/efeitos adversos , Laringoplastia/métodos , Laringe/anormalidades , Oxigênio/sangue , Complicações Pós-Operatórias/etiologia , Asma/complicações , Criança , Pré-Escolar , Estudos de Coortes , Deficiências do Desenvolvimento/complicações , Feminino , Hospitalização , Humanos , Lactente , Recém-Nascido , Injeções , Laringe/cirurgia , Lasers de Gás/uso terapêutico , Tempo de Internação , Masculino , Hipotonia Muscular/complicações , Complicações Pós-Operatórias/sangue , Período Pós-Operatório , Estudos Retrospectivos , Estenose Traqueal/complicações , Anel Vascular/complicações
12.
Orthopade ; 49(3): 255-259, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31628500

RESUMO

BACKGROUND: Sprengel's deformity is a rare congenital anomaly that can present in children, leading to cosmetic and functional impairment. This study investigated clinical results of this deformity among cases managed using a modified Green procedure. METHODS: From February 2008 to September 2015 a total of 34 patients with Sprengel's deformity were treated with a modified Green procedure. The mean age of patients at the time of surgery was 4.8 years (range 2-12 years) and the average follow-up time was 6.1 years (range 4-10 years). The shoulder abduction, Cavendish classification, Rigault classification, scapular elevation, and postoperative complications were evaluated. RESULTS: The mean shoulder abduction was 102.3° (range 70-140°) preoperatively and 142.6° (range 120-170°) postoperatively. The scapular elevation was 4.2 cm (range 2-6.5 cm) preoperatively and 1.35 cm (range 0-2 cm) postoperatively. Improvement by at least one Cavendish and/or Rigault grade was attained in all cases postoperatively. The differences in preoperative and postoperative shoulder abduction, scapular elevation, Cavendish grade and Rigault grade were statistically significant (p < 0.001). CONCLUSION: The modified Green procedure is a relatively safe and reliable method in the treatment of severe Sprengel's deformity, which ensures successful shoulder function as well as a good cosmetic appearance.


Assuntos
Anormalidades Congênitas/cirurgia , Escápula/anormalidades , Articulação do Ombro/anormalidades , Criança , Pré-Escolar , Humanos , Osteotomia , Amplitude de Movimento Articular , Escápula/cirurgia , Articulação do Ombro/cirurgia , Resultado do Tratamento
13.
Clin Imaging ; 59(2): 172-178, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31821975

RESUMO

AIM: To review the experience of a single tertiary center with obstructed hemivagina and ipsilateral renal agenesis (OHVIRA syndrome) and evaluate if MRI findings correlate with outcome. METHODS: Patients (n = 32) diagnosed with OHVIRA syndrome between 2001 and 2019 were analyzed. Presenting symptoms, age of menarche, age at operation, MRI findings, management, and outcome were reviewed. In sagittal MRI planes, distance from hematocolpos to perineum was measured. Measurements were compared among patients who underwent single-stage vaginoplasty and hemihysterectomy. RESULTS: Mean age at diagnosis and mean age of menarche was 16.8 ± 6.4 (10-33) and 12.8 ± 1.0 (10-15). Main presenting complaints were abdominal pain/dysmenorrhea. Eighteen anomalies (56.3%) were on right side. Twenty-eight had ipsilateral renal agenesis, 3 patients had normal renal anatomy and one had unilateral multicystic dysplastic kidney. In MRI, 21 patients had hematocolpos, 11 patients had both hematocolpos and hematometra. 28 patients underwent single-stage vaginoplasty and vaginal septum resection. One had hemihysterectomy due to sepsis at presentation. Three patients had hemihysterectomy due to proximal vaginal septum and impossibility of vaginoplasty. During follow-up, 7 cases (21.9%) had married and 5 of these (71.4%) were pregnant or had delivered. Five patients had reoperation during follow-up. MRI images of 19 patients indicated mean distances from hematocolpos to perineum whom underwent vaginoplasty or hemihysterectomy were 33.9 ± 18.1 mm (10-79 mm) and 87.3 ± 11.0 mm (80-100), respectively (p = .009). CONCLUSIONS: Gold standard treatment of OHVIRA syndrome is single-stage vaginoplasty. Distance from hematocolpos to perineum in MRI may correlate with surgical outcome. Hemihysterectomy may be an alternative for extreme proximal vaginal septum or infectious complications.


Assuntos
Anormalidades Múltiplas/diagnóstico por imagem , Anormalidades Múltiplas/cirurgia , Anormalidades Congênitas/diagnóstico por imagem , Anormalidades Congênitas/cirurgia , Nefropatias/congênito , Rim/anormalidades , Imagem por Ressonância Magnética/métodos , Vagina/anormalidades , Vagina/diagnóstico por imagem , Adolescente , Adulto , Criança , Feminino , Humanos , Histerectomia/métodos , Rim/diagnóstico por imagem , Rim/cirurgia , Nefropatias/diagnóstico por imagem , Nefropatias/cirurgia , Síndrome , Resultado do Tratamento , Vagina/cirurgia , Adulto Jovem
14.
BJOG ; 127(2): 230-238, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31397072

RESUMO

Women with congenital absolute uterine factor infertility (AUFI) often need vaginal restoration to optimise sexual function. Given their lack of procreative ability, little consideration has previously been given to the resultant vaginal microbiome (VM). Uterine transplantation (UTx) now offers the opportunity to restore these women's reproductive potential. The structure of the VM is associated with clinical and reproductive implications that are intricately intertwined with the process of UTx. Consideration of how vaginal restoration methods impact VM is now warranted and assessment of the VM in future UTx procedures is essential to understand the interrelation of the VM and clinical and reproductive outcomes. TWEETABLE ABSTRACT: The vaginal microbiome has numerous implications for clinical and reproductive outcomes in the context of uterine transplantation.


Assuntos
Anormalidades Congênitas/cirurgia , Infertilidade Feminina/cirurgia , Microbiota/fisiologia , Transplante de Órgãos , Útero/transplante , Vagina/microbiologia , Feminino , Humanos , RNA Ribossômico 16S/fisiologia , Técnicas de Reprodução Assistida , Útero/anormalidades , Útero/microbiologia , Vagina/fisiopatologia
15.
Am J Case Rep ; 20: 1915-1919, 2019 Dec 21.
Artigo em Inglês | MEDLINE | ID: mdl-31862868

RESUMO

BACKGROUND Thoracoschisis is a very rare congenital birth defect defined by the herniation of intra-abdominal organs through a defect in the thoracic wall. Though often associated with other birth defects as a part of the "limb-body wall complex" deformities, thoracoschisis has very rarely been reported as an isolated finding. CASE REPORT Here we present the case of a 30-day-old term male infant with an isolated left thoracoschisis managed successfully by primary closure. The patient was monitored postnatally in the Neonatal Intensive Care Unit (NICU) of Maputo Central Hospital because of the presence of a herniated mass through a left chest wall defect below the left nipple. Computed tomography (CT) scans suggested the presence of a left diaphragmatic hernia, left rib agenesis, and herniation of an unidentifiable intra-abdominal organ through the anterior left chest wall. On day of life (DOL) 30, when global health outreach pediatric surgeons arrived at the hospital, the decision was made to operate on the child. The mass was found to be of liver origin, the exposed tissue was excised, and primary closure of the chest wall was accomplished. The patient's postoperative course involved a wound infection that resolved favorably with treatment, allowing for discharged home on postoperative day (POD) 17 in stable condition. CONCLUSIONS Our case report highlights the importance of recognizing this rare condition and directing appropriate surgical care.


Assuntos
Anormalidades Congênitas/cirurgia , Hérnias Diafragmáticas Congênitas/cirurgia , Hepatopatias/congênito , Hepatopatias/cirurgia , Parede Torácica/anormalidades , Parede Torácica/cirurgia , Anormalidades Congênitas/diagnóstico por imagem , Hérnias Diafragmáticas Congênitas/diagnóstico por imagem , Humanos , Recém-Nascido , Hepatopatias/diagnóstico por imagem , Masculino , Doenças Raras , Costelas/anormalidades , Parede Torácica/diagnóstico por imagem , Tomografia Computadorizada por Raios X
16.
BMC Womens Health ; 19(1): 144, 2019 11 26.
Artigo em Inglês | MEDLINE | ID: mdl-31771581

RESUMO

BACKGROUND: To evaluate the feasibility and clinical outcomes of vaginoplasties using a neovaginal polylactic acid prosthesis made with 3-dimensional (3D) printing technology as an intraneovaginal mould. METHODS: This was an interventionist, prospective, and multicentre clinical pilot investigation of a sanitary product (PACIENA prosthesis®) aiming to recruit and operate on 8 patients over 6 months with a follow-up period of 6 months. Only six patients with Rokitansky syndrome and one patient with Morris syndrome (7 patients in total) were operated on in two university hospitals: "La Fe", Valencia (H1) and "Arrixaca", Murcia (H2). INTERVENTIONS: Extensive surgical dissection of a defined space between the urethra and bladder in the front and of the rectum in the back as well as insertion of the PACIENA prosthesis® covered with Interceed® were performed. After 12 days, the prosthesis was changed to the silicone-covered version for daily application. RESULTS: In the 6 patients with Rokitansky syndrome (86%), the primary endpoint (satisfactory vaginal outcome in terms of appearance, function, and sensation without relevant additional morbidity) was achieved, although only 2 patients (28%) were sexually active at the end of 6 months of follow-up. The patient with Morris syndrome withdrew from the study after 1 month. Patients without bacterial colonization showed positive Schiller tests at 1 month, and subsequent biopsies showed adequate keratinization and epidermization. Epithelization and iodopositivity were delayed in the patients who developed inflammatory granulomas. CONCLUSIONS: Good anatomical and functional results can be achieved with the PACIENA prosthesis® for vaginoplasties without skin grafts. However, adequate patient selection and education, good surgical techniques and haemostasis, postoperative support, and prevention of bacterial colonization are important. TRIAL REGISTRATION: This clinical study was approved by the Ethical Clinical Investigation Committee of San Juan University Hospital on September 27, 2016, to be conducted in the participating centres; it was authorized by the Spanish Agency of Medicines and Health Products (AEMPS) on April 24, 2017 (exp. no. 585/16/EC), to be carried out in that hospitals.


Assuntos
Anormalidades Congênitas/cirurgia , Próteses e Implantes , Implantação de Prótese/métodos , Procedimentos Cirúrgicos Reconstrutivos/métodos , Vagina/anormalidades , Vagina/cirurgia , Adulto , Celulose Oxidada , Estudos de Viabilidade , Feminino , Humanos , Projetos Piloto , Estudos Prospectivos , Desenho de Prótese , Resultado do Tratamento , Adulto Jovem
17.
Arch Gynecol Obstet ; 300(6): 1633-1636, 2019 12.
Artigo em Inglês | MEDLINE | ID: mdl-31667605

RESUMO

OBJECTIVE: To compare using the acellular porcine small intestinal submucosa (SIS) graft or the Interceed in patients with MRKH syndrome undergoing creation of a neovagina. METHODS: In this retrospective study, patients with MRKH syndrome undergoing creation of a neovagina from 2016 to 2018 were retrospectively investigated. Wharton-Sheares-George neovaginoplasty was performed using the acellular porcine SIS graft or the Interceed. RESULTS: Overall, 67 patients were included for analysis. The operating time, the estimated blood loss and return of bowel activity were similar between the two groups. However, the total cost in the SIS group was significantly higher than that in the Interceed group due to the cost of the SIS graft. The mean length and width of the neovagina were similar between the two groups. However, the incidence of granulation in vaginal apex was higher in the SIS graft group than that in the Interceed group. There was no statistically significant difference in the total FSFI scores between the two groups who became sexually active postoperatively. CONCLUSIONS: Our results demonstrated that Wharton-Sheares-George method provided the patients to have satisfactory sexual intercourse. The Interceed played a role in the reconstruction of neovagina no less than the SIS graft.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Mucosa Intestinal/transplante , Ductos Paramesonéfricos/anormalidades , Procedimentos Cirúrgicos Reconstrutivos/métodos , Vagina/cirurgia , Adulto , Animais , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Estudos Retrospectivos , Estruturas Criadas Cirurgicamente , Suínos
18.
Semin Pediatr Surg ; 28(5): 150843, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31668290

RESUMO

Adult and pediatric urologists, gynecologists, and surgeons are often faced with primary reconstruction of complex congenital defects of the vagina as seen in cloacal anomalies, disorders of sexual development (DSD), and vaginal agenesis as well as with the secondary repair of post-surgical or radiation-induced vaginal stenosis or foreshortening. Many options have been reported and the choice of a particular method is influenced by surgeon experience, regional trends, availability of multidisciplinary teams and many other social and age-related factors. No one option has become the solution for all patients of all ages and anatomical challenges. Here we discuss a newer approach that can be used as an option for bridging the gap in young patients with a long distance from the perineum to the vaginal structures, for total vaginoplasty in some cases of primary vaginal agenesis in adolescents or young adults, and for correction of stricture in patients who have undergone previous reconstructions. Our initial experience using autologous buccal mucosa for reconstructions on eight patients with varying diagnoses is retrospectively reviewed.


Assuntos
Anormalidades Congênitas/cirurgia , Procedimentos Cirúrgicos em Ginecologia/métodos , Mucosa Bucal/transplante , Vagina/anormalidades , Vagina/cirurgia , Adolescente , Adulto , Tomada de Decisão Compartilhada , Feminino , Humanos , Estudos Retrospectivos , Transplante Autólogo , Adulto Jovem
19.
J Ayub Med Coll Abbottabad ; 31(3): 466-468, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31535531

RESUMO

Gastrointestinal duplication cyst is a rare congenital anomaly with a reported incidence of 1 in 4500 live births. Any part of gastrointestinal tract from mouth to anus can be affected with this anomaly. Among gastrointestinal tract duplications, gastric duplication cyst is extremely rare (2- 9%). We are presenting a case of the stomach duplication in a four (04) day old male child who presented in our Emergency Department with complaints of non-bilious, non-projectile vomiting and visible bulge in upper abdomen since birth. Workup showed enteric duplication cyst which was excised. Complete surgical resection is the treatment of choice in gastric duplication cyst.


Assuntos
Obstrução da Saída Gástrica/etiologia , Estômago/anormalidades , Anormalidades Congênitas/cirurgia , Humanos , Recém-Nascido , Masculino , Estômago/cirurgia
20.
Orv Hetil ; 160(38): 1510-1513, 2019 Sep.
Artigo em Húngaro | MEDLINE | ID: mdl-31537097

RESUMO

Gallbladder agenesis is a rare congenital malformation due to an embryological defect of the biliary system. In most cases it is asymptomatic, but it can also mimic biliary colic. We report the case of a 72-year-old Caucasian woman with a medical history of cardiovascular disease and hypercholesterolemia, under cholesterol-lowering and hypotensive treatment, who presented symptoms suggesting biliary colic. She underwent laparoscopic surgery that confirmed the final diagnosis. We present our approach in this rare case as well as a brief review of medical literature. The surgeon should decide intraoperatively whether to continue and search for a possible ectopic gallbladder or investigate further with imaging studies. Gallbladder agenesis is a rare condition that the surgeon must be aware of. In the cases of inconclusive or indirect signs of cholelithiasis, the best approach is complementary imaging investigations such as magnetic resonance cholangiopancreatography in order to avoid surgery. Orv Hetil. 2019; 160(38): 1510-1513.


Assuntos
Anormalidades Congênitas/cirurgia , Vesícula Biliar/anormalidades , Laparoscopia , Adulto , Idoso , Colecistectomia Laparoscópica , Colelitíase/diagnóstico , Diagnóstico Diferencial , Feminino , Vesícula Biliar/cirurgia , Humanos
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