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1.
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
2.
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
3.
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
4.
Cir. plást. ibero-latinoam ; 45(3): 327-336, jul.-sept. 2019. ilus, tab
Artigo em Espanhol | IBECS | ID: ibc-184410

RESUMO

La tercera campaña de ayuda humanitaria llevada a cabo por la Sociedad Española de Cirugía Plástica, Reparadora y Estética (SECPRE) en Monrovia (Liberia) se desarrolló durante el mes de noviembre de 2018 y supone la continuación de este tipo de ayudas en el continente africano promovidas por la ONG Juan Ciudad. Como en otras misiones humanitarias protagonizadas por cirujanos plásticos, el objetivo es mejorar la calidad de vida de la población más vulnerable y colaborar en su reincorporación a la sociedad. Las campañas humanitarias quirúrgicas tienen una fecha de ida y otra de vuelta, con un proyecto de actividad quirúrgica que desarrollar en pocos días y en unas condiciones de recursos limitados. Una buena planificación, apoyándonos en los datos obtenidos de las experiencias anteriores, facilitó la consecución de nuestros objetivos. Con la presentación de este artículo queremos transmitir nuestra experiencia personal en este proyecto


The third humanitarian aid campaign by the Spanish Society of Plastic, Reconstructive and Aesthetic Surgery (SECPRE) in Monrovia (Liberia) was carried out in November 2018 and reflects the continuation of such aid programmes in the African continent promoted by the NGO Juan Ciudad. As in other humanitarian missions involving plastic surgeons, the objective is to improve the quality of life of the most vulnerable population and to collaborate in their reintegration into society. The surgical humanitarian campaigns have a round trip date, with a surgical activity project to be developed in a few days and under limited resource conditions. Good planning, based on the data obtained from previous experiences contributed to the achievement of our objectives. Our aim presenting this article is sharing our personal experience in this project


Assuntos
Humanos , Socorro em Desastres , Sociedades Médicas/organização & administração , Cirurgia Plástica/tendências , Queimaduras/cirurgia , Anormalidades Congênitas/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/história , Procedimentos Cirúrgicos Reconstrutivos/tendências , África
5.
Rev. bras. cir. plást ; 34(3): 410-413, jul.-sep. 2019. ilus
Artigo em Inglês, Português | LILACS | ID: biblio-1047167

RESUMO

Criptotia é uma deformidade auricular congênita comum em orientais e rara em ocidentais, sendo a grande maioria dos estudos de técnicas cirúrgicas orientais e aplicados em crianças. Nesta patologia, a cartilagem do polo superior da orelha encontra-se alojada embaixo da pele na região temporal, o que impossibilita o uso de óculos, devido à falta de apoio e torna o polo superior sem definição estética. O presente estudo tem por objetivo relatar o caso de um paciente adulto com criptotia, submetido ao tratamento cirúrgico com retalho de pedículo subcutâneo mastóideo, revisando as principais técnicas descritas para o tratamento deste acometimento. O retalho de pedículo subcutâneo descrito por Yoshimura, mostrou-se adequado para a correção da criptotia em paciente ocidental e adulto.


Cryptotia is a congenital ear deformity common in Easterners and rare in Westerners, with most studies addressing Eastern surgical techniques applied to children. In this pathology, the cartilage of the upper pole of the ear is lodged subcutaneously in the temporal region, which prevents individuals from using glasses due to lack of support and prevents esthetic definition of the upper pole. The present study aimed to report the case of an adult patient with cryptotia undergoing surgical treatment using a mastoid subcutaneous pedicle flap and review the main techniques described for the treatment of this involvement. The subcutaneous pedicle flap described by Yoshimura proved to be adequate for correcting cryptotia in a Western adult patient.


Assuntos
Anormalidades Congênitas , Adulto , Procedimentos Cirúrgicos Reconstrutivos , Cartilagem da Orelha , Deformidades Adquiridas da Orelha , Orelha Externa , Estética , Equipamentos para Estética , Anormalidades Congênitas/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Procedimentos Cirúrgicos Reconstrutivos/reabilitação , Cartilagem da Orelha/anormalidades , Cartilagem da Orelha/cirurgia , Deformidades Adquiridas da Orelha/cirurgia , Deformidades Adquiridas da Orelha/genética , Orelha Externa/anormalidades , Orelha Externa/cirurgia
6.
J Bone Joint Surg Am ; 101(15): 1357-1365, 2019 Aug 07.
Artigo em Inglês | MEDLINE | ID: mdl-31393426

RESUMO

BACKGROUND: There have been many reports on the treatment of congenital kyphoscoliosis. However, congenital deformities in the cervicothoracic spine (C7-T1) have not been well described because of the rarity of these conditions. METHODS: The medical records and imaging studies of 25 children who were treated with 360° osteotomy for congenital deformities in the cervicothoracic spine (C7-T1) at a mean age of 11.4 years were reviewed. RESULTS: All 25 children presented with torticollis; 4 presented with neck pain; 10, with facial asymmetry; and 3, with preoperative neurological deficits. Twenty-three patients had congenital deformities in other regions of the spine. Six patients had a total of 8 intraspinal deformities. On average, the cervicothoracic curve was corrected from 53° preoperatively to 14° at the latest follow-up, the segmental kyphosis was corrected from 25° to 12°, and the head tilt improved from 25° to 5°. Nineteen patients had a total of 28 complications, including 1 transient cord injury together with a permanent C8 nerve root injury, 11 transient nerve root injuries, 1 transient Horner syndrome, 9 cases of decompensation of a compensatory curve, 2 implant failures, 2 cases of hemothorax, 1 dural tear, and 1 case of delayed wound-healing. CONCLUSIONS: Most congenital cervicothoracic deformities are fixed, and early surgical intervention may be needed. A 360° osteotomy is indicated for this type of rigid deformity and may provide satisfactory correction. However, 360° osteotomy in the cervicothoracic spine (C7-T1) is technically demanding with a higher risk of nerve root injuries, although most injuries tend to be transient. If the compensatory thoracic curve is severe and rigid, 1-stage or staged surgery in this region may be required. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.


Assuntos
Cifose/cirurgia , Osteotomia/métodos , Amplitude de Movimento Articular/fisiologia , Escoliose/cirurgia , Tomografia Computadorizada por Raios X/métodos , Adolescente , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Criança , Estudos de Coortes , Anormalidades Congênitas/diagnóstico por imagem , Anormalidades Congênitas/cirurgia , Bases de Dados Factuais , Feminino , Humanos , Cifose/congênito , Cifose/diagnóstico por imagem , Masculino , Duração da Cirurgia , Osteotomia/efeitos adversos , Posicionamento do Paciente , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/fisiopatologia , Cuidados Pré-Operatórios/métodos , Prognóstico , Recuperação de Função Fisiológica , Estudos Retrospectivos , Medição de Risco , Escoliose/congênito , Escoliose/diagnóstico por imagem , Vértebras Torácicas/diagnóstico por imagem , Vértebras Torácicas/cirurgia , Torcicolo/diagnóstico , Torcicolo/etiologia , Torcicolo/cirurgia
7.
BJOG ; 126(11): 1328-1335, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31338951

RESUMO

Congenital uterovaginal aplasia commonly occurs in Mayer-Rokitansky-Küster-Hauser syndrome. Various methods of neovagina creation exist, including nonsurgical self-dilation, surgical dilation, and surgical procedures involving skin or intestinal transplants. Subsequent uterus transplantation is necessary to enable pregnancy. We review the main characteristics, advantages, and disadvantages of established neovagina creation methods and discuss their suitability regarding subsequent uterus transplantation. Suitability criteria include sufficient vaginal length, absence of previous major intra-abdominal surgery, a natural vaginal axis, and a natural vaginal epithelium. In conclusion, Vecchietti-based laparoscopically assisted neovagina creation provides ideal functional conditions for uterus transplantation. Nonsurgical self-dilation and Wharton-Sheares-George vaginoplasty may also be suitable. TWEETABLE ABSTRACT: This review discusses the main advantages and disadvantages of neovagina creation methods with regard to subsequent uterus transplantation.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Procedimentos Cirúrgicos em Ginecologia/métodos , Ductos Paramesonéfricos/anormalidades , Cuidados Pré-Operatórios/métodos , Transplantes , Útero/transplante , Transtornos 46, XX do Desenvolvimento Sexual/fisiopatologia , Anormalidades Congênitas/fisiopatologia , Feminino , Humanos , Ductos Paramesonéfricos/fisiopatologia , Ductos Paramesonéfricos/cirurgia , Seleção de Pacientes , Estruturas Criadas Cirurgicamente , Resultado do Tratamento , Vagina/cirurgia
8.
Pediatr Surg Int ; 35(11): 1279-1289, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31324976

RESUMO

INTRODUCTION/PURPOSE: The burden of pediatric surgical disease is largely unknown in low- and middle-income countries such as Uganda where access to care is limited. METHODS: Implementation of a locally led database in January 2012 at a Ugandan tertiary referral hospital, and review of 3465 prospectively collected pediatric surgical admissions from January 2012 to August 2016. RESULTS: 2090 children (60.3%) underwent surgery during admission. 59% were male and 41% female. 28.6% of admissions were in neonates and 50.4% were in children less than 1 year old. Congenital anomalies including Hirschsprung's, anorectal malformations, intestinal atresias, omphalocele, and gastroschisis were the most common diagnoses (38.6%) followed by infections (15.0%) and tumors (8.6%). Mortality rates were substantially higher than those of high-income countries; for example, gastroschisis and intussusception had mortality rates of 90.1% and 19.7%, respectively. Post-operative mortality was highest in the congenital anomalies group (15.0%). CONCLUSION: There is a high burden of infant congenital anomalies with higher mortality rates compared to high-income countries. The unit performs primarily specialized procedures appropriate for a tertiary center. We hope that these data will facilitate evaluation of ongoing quality improvement and capacity-building initiatives.


Assuntos
Anormalidades Congênitas/epidemiologia , Neoplasias/epidemiologia , Ferimentos e Lesões/epidemiologia , Pré-Escolar , Anormalidades Congênitas/cirurgia , Feminino , Mortalidade Hospitalar , Hospitalização , Humanos , Lactente , Recém-Nascido , Masculino , Neoplasias/cirurgia , Estudos Prospectivos , Centros de Atenção Terciária , Uganda/epidemiologia , Ferimentos e Lesões/cirurgia
9.
Artigo em Chinês | MEDLINE | ID: mdl-31315353

RESUMO

Objective: To analyze the clinical characteristics and appropriate surgical procedures, and discuss the classification of congenital middle ear malformation. Methods: All cases were from the Center of Otorhinolaryngology, the Sixth Medical Center of Department of PLA General Hospital. All of these cases, including 26 male patients (ears) , 10 female patients (11 ears) , aged from 7 to 57 years old, had normal external auditory canal, tympanic membrane, conductive hearing loss, type A tympanogram and negative Gelle's test. Tympanoplasty was performed in all cases. The deformity was classified to three types,i.e., Type I (stapes foot plate mobility): Ⅰa, ossicular chain deformity with normal stapes suprastructure; Ⅰb, ossicular chain deformity with abnormal stapes suprastructure; Type Ⅱ (stapes foot plate fixation): Ⅱ a,normal ossicular chain, Ⅱ b, ossicular chain malformation; and Type Ⅲ: vestibular window osseous atresia or undeveloped, or with round window atresia. The malformation of type Ⅱ and Ⅲ may be accompanied with abnormal facial nerve. In addition, the papers on middle ear malformation published from 1982 to 2017 were analyzed retrospectively. The clinical data of 451 ears malformation were summarized. Results: According to the revisional classification criteria in 37 ear samples from our hospital, 20 ears were type I. 6 type Ⅰa cases were used PORP (partial ossicular replacement prosthesis) to reconstruct the ossicular chain; 14 type Ⅰb cases were used TORP (total ossicular replacement prosthesis) to reconstruct the ossicular chain. For the 5 ears of type Ⅱ, 2 of which were type Ⅱ a and 3 were type Ⅱ b. 4 ear samples of type Ⅱ were implanted with Piston ossicular prosthesis, 1 was implanted with TORP in which the ossificated foot plate was removed with periosteum preserved. 12 ear samples were type Ⅲ, with vestibular window osseous atresia, facial nerve malformation, and stapes suprastructure malformation. The pistons ossicular prosthesis were implanted in vestibular window in 3 ears with facial nerve covering vestibular window partially. The surgery had to be given up in 5 ears, and TORP was implanted in 4 ears at the opening with preserved periosteum at the beginning of the tympanic scala because of facial nerve covering vestibular window totally. 30 ears with complete follow-up data had no sensorineural hearing loss and the average air-bone conduction decreased 23.3±10.7 dB (P<0.05).There were 234 ears of type Ⅰ in 451 ears of congenital middle ear malformation reported in the literature. 113 of which were type Ⅰa, the basic surgery was ossicular chain shaking and artificial or autogenous PORP implantation. Type Ⅰb was 121 ears, with autogenous or artificial TORP and PORP. Type Ⅱ was125 ears, including type Ⅱa 22 ears, Ⅱb 60 ears, and no subclassification for 43 ears. The surgery of type Ⅱ was the same as otosclerosis. The vestibular window atresia of type Ⅲ was 92 ears, the surgery of 17 ears had to be abandoned, the other ears underwent vestibular window, promontory or semicircular canal opening to reconstruct hearing with Piston, autogenous or artificial TORP. Conclusion: Referring to the classification of congenital middle ear malformation combining with appropriate surgical materials and methods, otologists can better understand and choose appropriate surgical method to the middle ear malformation.


Assuntos
Anormalidades Congênitas/classificação , Anormalidades Congênitas/cirurgia , Ossículos da Orelha/cirurgia , Orelha Média/anormalidades , Adolescente , Adulto , Criança , Ossículos da Orelha/anormalidades , Orelha Média/cirurgia , Feminino , Perda Auditiva Condutiva/congênito , Perda Auditiva Condutiva/cirurgia , Humanos , Masculino , Pessoa de Meia-Idade , Prótese Ossicular , Estudos Retrospectivos , Membrana Timpânica/anormalidades , Membrana Timpânica/cirurgia , Timpanoplastia , Adulto Jovem
10.
Urology ; 131: 240-244, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31150693

RESUMO

OBJECTIVE: To evaluate the effect of a scar-free surgical approach, the modified McIndoe technique, in cases of vaginal agenesis. MATERIALS AND METHODS: Seven patients with Mayer-Rokitansky-Kuster-Hauser syndrome underwent vaginoplasty with the modified McIndoe technique. Mucosa grafts harvested from the vulva were minced into small particles and transplanted to the neovagina. RESULTS: Epithelization in the neovagina is approximately 20 times in size than that in the mucosa harvested from the donor site. An adequate vaginal length was obtained in all cases, with a minimal change in genital appearance and invisible scars. All 4 sex-active patients reported satisfactory sexual experiences, with spontaneous lubrication during intercourse. CONCLUSION: With the modified McIndoe technique, using an autologous micromucosa graft harvested from the vulva and the buccal cavity, we can physiologically reconstruct a new mucosa-lined vagina with minimal sacrifice.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Procedimentos Cirúrgicos em Ginecologia/métodos , Membrana Mucosa/transplante , Ductos Paramesonéfricos/anormalidades , Vagina/anormalidades , Vagina/cirurgia , Adulto , Autoenxertos , Cicatriz/prevenção & controle , Feminino , Humanos , Ductos Paramesonéfricos/cirurgia , Complicações Pós-Operatórias/prevenção & controle , Vulva/cirurgia , Adulto Jovem
11.
Int J Surg ; 68: 11-19, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31185313

RESUMO

BACKGROUND: Inguinal hernia repair is one of the most frequent operations in pediatric surgery and is increasingly performed laparoscopically. The latter introduced new momentum in the debate on the necessity of contralateral exploration, as the rates of contralateral patent processus vaginales and metachronous inguinal hernias determine whether a routine closure would be overtreatment or useful prevention. MATERIALS AND METHODS: We searched MEDLINE via PubMed, Web of Science and Scopus at the 6th of September 2017; reference lists and CrossRef were snowballed for reports citing identified studies. Eligibility criteria were age <18 years, preoperative diagnosis of unilateral hernia, laparoscopic evaluation, and publication since January 2012. Studies using hernioscopy (transinguinal laparoscopy) were excluded. We reported our systematic review following PRISMA criteria. RESULTS: We included 32 reports consisting of 19,188 pediatric patients diagnosed with unilateral inguinal hernia. Of these, 38.5% (95% confidence interval: 34%-43.1%) had a contralateral open processus vaginalis concomitantly found during laparoscopic inguinal hernia repair. A secondary analysis using nine studies that compared open and laparoscopic approaches found that prophylactic closure of contralateral patent processus vaginales resulted in a risk difference of 5.7% (95% confidence interval: 3.6%-7.7%; P < 0.001) following 2691 (42.8%) procedures (nine studies: Ten of 6282 patients operated laparoscopically had a metachronous hernia, versus 286 of 5764 with open hernia repair). CONCLUSIONS: Prophylactic closure of a contralateral patent processus vaginalis reduces the number of metachronous inguinal hernias, but about 18 procedures must be performed to prevent one metachronous inguinal hernia, indicating that the indication should be based on personal circumstances of the patient.


Assuntos
Anormalidades Congênitas/diagnóstico , Hérnia Inguinal/cirurgia , Herniorrafia/métodos , Canal Inguinal/anormalidades , Laparoscopia/métodos , Criança , Pré-Escolar , Anormalidades Congênitas/cirurgia , Feminino , Hérnia Inguinal/congênito , Hérnia Inguinal/prevenção & controle , Humanos , Lactente , Canal Inguinal/cirurgia , Masculino , Procedimentos Cirúrgicos Reconstrutivos/métodos
12.
BMJ Case Rep ; 12(6)2019 Jun 26.
Artigo em Inglês | MEDLINE | ID: mdl-31248898

RESUMO

This case report describes an extremely rare association between gallbladder agenesis and choledochal cyst (CC). A 9-year-old girl presented with recurrent abdominal pain in the right upper quadrant. Radiological studies revealed a CC type IVa and an agenesis of gallbladder and cystic duct. Due to the possibility of biliary neoplasm, the patient underwent cyst resection and hepaticoduodenostomy. Histopathological findings showed inflamed fibrous tissue covered by biliary epithelium with no evidence of malignancy.


Assuntos
Cisto do Colédoco/complicações , Cisto do Colédoco/diagnóstico por imagem , Anormalidades Congênitas/diagnóstico por imagem , Vesícula Biliar/anormalidades , Procedimentos Cirúrgicos do Sistema Biliar , Criança , Cisto do Colédoco/cirurgia , Anormalidades Congênitas/cirurgia , Feminino , Vesícula Biliar/diagnóstico por imagem , Vesícula Biliar/cirurgia , Humanos , Imagem por Ressonância Magnética
13.
Surgery ; 165(6): 1234-1242, 2019 06.
Artigo em Inglês | MEDLINE | ID: mdl-31056199

RESUMO

BACKGROUND: Congenital anomalies are the leading cause of infant death and pediatric hospitalization, but existing estimates of the associated costs of health care are either cross-sectional surveys or economic projections. We sought to determine the percent of total hospital health care expenditures attributable to major anomalies requiring surgery within the first year of life. METHODS: Utilizing comprehensive California statewide data from 2008 to 2012, cohorts of infants undergoing major surgery, with birth defects and with surgical anomalies, were constructed alongside a referent group of newborns with no anomalies or operations. Cost-to-charge and physician fee ratios were used to estimate hospital and professional costs, respectively. For each cohort, costs were broken down according to admission, birth episode, and first year of life, with additional stratifications by birth weight, gestational age, and organ system. RESULTS: In total, 68,126 of 2,205,070 infants (3.1%) underwent major surgery (n = 32,614) or had a diagnosis of a severe congenital anomaly (n = 57,793). These accounted for $7.7 billion of the $18.9 billion (40.7%) of the total health care costs/expenditures of the first-year-of-life hospitalizations, $7.0 billion (48.6%) of the costs for infants with comparatively long birth episodes, and $5.2 billion (54.7%) of the total neonatal intensive care unit admission costs. Infants with surgical anomalies (n = 21,264) totaled $4.1 billion (21.7%) at $80,872 per infant. Cardiovascular and gastrointestinal diseases accounted for most admission costs secondary to major surgery or congenital anomalies. CONCLUSION: In a population-based cohort of infant births compared with other critically ill neonates, surgical congenital anomalies are disproportionately costly within the United States health care system. The care of these infants, half of whom are covered by Medi-Cal or Medicaid, stands as a particular focus in an age of reform of health care payments.


Assuntos
Anormalidades Congênitas/economia , Anormalidades Congênitas/cirurgia , Utilização de Instalações e Serviços/economia , Gastos em Saúde/estatística & dados numéricos , Custos Hospitalares/estatística & dados numéricos , California , Utilização de Instalações e Serviços/estatística & dados numéricos , Feminino , Disparidades em Assistência à Saúde/economia , Disparidades em Assistência à Saúde/estatística & dados numéricos , Hospitalização/economia , Hospitalização/estatística & dados numéricos , Humanos , Lactente , Recém-Nascido , Armazenamento e Recuperação da Informação , Unidades de Terapia Intensiva Neonatal/economia , Unidades de Terapia Intensiva Neonatal/estatística & dados numéricos , Terapia Intensiva Neonatal/economia , Terapia Intensiva Neonatal/estatística & dados numéricos , Masculino
14.
Int Braz J Urol ; 45(5): 1076-1077, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31136107

RESUMO

INTRODUCTION: OHVIRA syndrome is a rare entity characterized by renal and Mullerian anomalies. The objective of the video is, through a clinical case, to discuss the importance of diagnosis, management and treatment, to avoid the complications that this syndrome entails, and to improve the long-term prognosis. MATERIALS AND METHODS: We report the case of a 10-year-old girl who consulted for abdominal pain, being diagnosed with OHVIRA syndrome. We describe the diagnosis and the surgical technique. In addition, we perform a systematic review in PubMed to report the published literature of this topic and we show the optimal management of this pathology.


Assuntos
Anormalidades Congênitas/cirurgia , Nefropatias/congênito , Rim/anormalidades , Laparoscopia/métodos , Nefroureterectomia/métodos , Vagina/anormalidades , Vagina/cirurgia , Anormalidades Múltiplas , Criança , Feminino , Humanos , Rim/cirurgia , Nefropatias/cirurgia , Reprodutibilidade dos Testes , Síndrome , Resultado do Tratamento , Útero/anormalidades , Útero/cirurgia
15.
Fertil Steril ; 112(1): 174-176, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-31103284

RESUMO

OBJECTIVE: To describe a McIndoe procedure technique for surgical management of Mayer-Rokitansky-Kuster-Hauser syndrome with the use of Nile tilapia skin as a scaffold for the proliferation of new vaginal epithelium. DESIGN: Surgical video article. Local Institutional Review Board approval and written permission from the patient were obtained. There were no conflicts of interest. SETTING: University hospital. PATIENT(S): A 17-year-old woman who presented at our gynecology department with the complaint of primary amenorrhea. At physical examination, she had a phenotypically normal vulva with no vaginal canal. Magnetic resonance imaging of the abdomen and pelvis revealed normal ovaries and absence of uterus and vaginal canal. No other congenital malformations were found. Karyotype was 46,XX. INTERVENTION(S): The McIndoe procedure involved only a vaginal approach. Labia minora were separated, and a transverse midline incision of 3 cm was made. The vesicorectal space was progressively dissected. Blunt dissection was performed initially with digital separation of tissues. The neovagina was then inspected with the introduction of a vaginal speculum, allowing for review of hemostasis. Blunt dissection was continued with the aid of the speculum, to reach the appropriate vaginal dimensions. Subsequently, a vaginal acrylic mold covered with two pieces of processed and sterilized tilapia fish skin was inserted and accommodated into the newly created cavity. The external side of the tilapia skin, which maintained its grayish coloration after the removal of the scales, stayed in contact with the acrylic mold, while the white internal side of the tilapia skin, which was previously attached to the fish's muscle, stayed in contact with the walls of the neocavity. The mold was held in position by four multifilament polyglactin 1.0 sutures in the labia majora, thus preventing expulsion. MAIN OUTCOME MEASURE(S): Anatomic data, such as measurement of the final canal length, and histomorphologic analysis, 180 days after surgery. RESULT(S): The patient remained on bed rest for 9 days, after which the tilapia fish skin had been partially reabsorbed. After this time, the acrylic mold was removed. A larger plastic mold was then inserted and the patient was advised to wear it day and night for the first postoperative month. The vaginal mold had to be worn each night until normal sexual intercourse was possible. The final canal length 180 days after surgery was between 8 and 9 cm. For the histopathologic analysis, fragments of the lateral vaginal wall were removed 180 days after surgery and showed the presence of stratified squamous epithelium with five cell layers, ectasic blood vessels, and occasional desquamated epithelial cells. CONCLUSION(S): The procedure described offered this patient an anatomic and functional neovagina by means of a simple, safe, easy, effective, quick, and minimally invasive procedure. Limitations include the experimental nature of this study, based on a single case report with no long-term outcome results. The tilapia fish skin is a low-cost and widely available biomaterial.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Anormalidades Congênitas/cirurgia , Procedimentos Cirúrgicos em Ginecologia/métodos , Ductos Paramesonéfricos/anormalidades , Procedimentos Cirúrgicos Reconstrutivos/métodos , Transplante de Pele/métodos , Estruturas Criadas Cirurgicamente , Tilápia , Vagina/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico por imagem , Adolescente , Animais , Anormalidades Congênitas/diagnóstico por imagem , Feminino , Sobrevivência de Enxerto , Humanos , Ductos Paramesonéfricos/diagnóstico por imagem , Ductos Paramesonéfricos/cirurgia , Transplante Heterólogo , Resultado do Tratamento , Vagina/anormalidades , Vagina/diagnóstico por imagem
16.
BMJ Case Rep ; 12(4)2019 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-31005874

RESUMO

Vaginal agenesis is one of the major congenital anomalies affecting women. Postoperative prosthetic vaginal dilators are indicated in patients treated surgically for vaginal agenesis. Although different dental materials such as acrylics and silicone-coated materials are used, addition silicones alone have never been used for the fabrication of prosthetic vaginal dilators. Addition silicone materials have advantages, such as high elastic recovery, high tear and tensile strengths, which aid in the definitive use of vaginal dilators. Vaginal dilators fabricated using addition silicone materials act as an efficient prosthesis in terms of function and ease of use in surgically treated vaginal agenesis patients.


Assuntos
Anormalidades Congênitas/cirurgia , Dilatação/instrumentação , Próteses e Implantes , Vagina/anormalidades , Feminino , Humanos , Procedimentos Cirúrgicos Reconstrutivos , Silicones , Vagina/cirurgia
17.
PLoS One ; 14(4): e0214967, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30958858

RESUMO

OBJECTIVE: Numerous studies established a link between socioeconomic status (SES) and several dimensions of general health. This study examines the association between maternal education as a widely used indicator of SES and outcome in newborns requiring surgical correction of congenital anomalies. METHODS: Ambispective data analysis of newborns with esophageal atresia (EA), intestinal atresia (IA), congenital diaphragmatic hernia (CDH), omphalocele (OC), gastroschisis (GS) undergoing surgery between 01/2008-11/2017 accessing the clinical databases Neodat and Viewpoint. Maternal education was determined according to the validated education classification CASMIN and stratified into "low" SES and "high" SES group. Endpoints were incidence of postoperative complications, length of mechanical ventilation, and readmission to NICU. RESULTS: Inclusion of 169 patients with EA (n = 32), IA (n = 24), CDH (n = 47), OC (n = 19), GS (n = 47). Women of low SES (n = 67, 40%) attended fewer prenatal screenings (total, 4.6 vs. 7.9, P<0.0001; EA, 3.7 vs. 7.1, P = 0.0002; IA, 3.5 vs. 9.4, P = 0.0006; OC, 2.5 vs. 8.8, P = 0.009; GS, 4.1 vs. 7.0, P = 0.002). Low SES was associated with higher incidence of patients born small for gestational age (37% vs. 20%, P = 0.019), with additional congenital malformations (37% vs. 15%, P = 0.001), being born in a peripheral center (7% vs. 0%, P = 0.008), and with higher incidence of 5´APGAR scores <7 (23% vs. 7%, P = 0.004). Moreover, low SES was associated with higher incidence of postoperative complications (total 70% vs. 32%, P<0.0001; EA, 60% vs. 23%, P = 0.04; IA, 67% vs. 11%, P = 0.008; CDH, 83% vs. 46%, P = 0.009; GS, 74% vs. 25%, P = 0.001), and higher readmission rate to NICU (IA, 33% vs. 0%, P = 0.043; GS, 32% vs. 4%, P = 0.007). CONCLUSIONS: Low maternal education is associated with a reduced uptake of prenatal screenings, adverse neonatal outcomes, and higher incidence of postoperative complications in newborns with congenital anomalies. Primary prevention and specific support should be provided prenatally for families with low SES to avoid adverse outcomes.


Assuntos
Anormalidades Congênitas , Bases de Dados Factuais , Complicações Pós-Operatórias , Fatores Socioeconômicos , Adulto , Anormalidades Congênitas/epidemiologia , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Incidência , Recém-Nascido , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/prevenção & controle , Estudos Retrospectivos
18.
J Pediatr Surg ; 54(8): 1590-1594, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31027906

RESUMO

INTRODUCTION: Cloacal malformations, a confluence of the urinary tract, vagina and rectum into a single common channel, has a broad and complex anatomic spectrum requiring an imaging tool for visualization, measurement, and surgical planning for the reconstruction of these structures. We evaluated the role of 3-D fluoroscopy for this purpose, as it offers a combination of spatial correlation with precise anatomic measurements. METHODS: We examined our imaging protocol for patients with a cloacal malformation and report our experience with rotational fluoroscopy and 3-D reconstruction in 16 consecutive patients referred for cloacal reconstruction. The length of the common channel (CC), the length of the urethra from the bladder neck to the common channel, and the height (and existence or absence) of a vagina or vaginas were determinants of the surgical procedures used for the repair. RESULTS: We performed 16 consecutive 3-D cloacagrams (age range 4 months to 9 years) using a new protocol (Figure 1) that provided the following data which helped with surgical planning: Gynecologic: 3 cases with a single vagina, 5 cases with a duplicated Mullerian system (3 of which were asymmetric) and 2 cases with high vaginas requiring vaginal replacement. Colorectal: Four had a high rectum requiring an abdominal approach, and 6 had a rectum reachable via a posterior sagittal approach. Urologic: Two ectopic ureters requiring reimplantation, 3 patients had vesicoureteral reflux (1 bilateral, 2 unilateral), 1 patient had no bladder, and 7 had a normal sized bladder. Common channel length and urethral length were demonstrated in all cases and used to decide between a total urogenital mobilization or a separation of vagina(s) from the common channel, urogenital separation. CONCLUSION: The 3-D cloacagram can help predict the surgical plan for urologic, gynecologic, and colorectal components of the cloacal repair. It can predict the CC length as well as the length of the urethra. It helps with predicting the need for vaginal replacement and whether an abdominal approach is needed for the rectum. Its effectiveness is based on the ability to adequately distend structures and see their distal most extent, an advantage over other modalities such as MRI. Added benefits (particularly from the 3D view) include a better spatial understanding of the defect and the diagnosis of concomitant urological abnormalities such as vesicoureteral reflux and ectopic ureters. Disadvantages to this procedure include the need for general anesthesia and a higher exposure to radiation. LEVEL OF EVIDENCE: 3.


Assuntos
Anormalidades Congênitas , Fluoroscopia , Imagem Tridimensional , Reto , Sistema Urinário , Vagina , Criança , Pré-Escolar , Anormalidades Congênitas/diagnóstico por imagem , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Lactente , Reto/anormalidades , Reto/diagnóstico por imagem , Sistema Urinário/anormalidades , Sistema Urinário/diagnóstico por imagem , Vagina/anormalidades , Vagina/diagnóstico por imagem
19.
Biomed Res Int ; 2019: 2360185, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30931323

RESUMO

Hypothesis/Aims of Study: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea. The ESHRE/ESGE categorizes this disorder within the class 5 uterine malformation of the female genital tract anomalies. It is characterized by congenital absence of the uterus, cervix, and upper part of the vagina in otherwise phenotypically normal 46XX females. These patients have normal ovaries, biphasic ovarian cycle, and female psychosexual identification. Laparoscopic Vecchietti's operation-surgical method in which the vagina increases in size by gradually applying traction to the vaginal vault-is one of the methods used to treat MRKH. The aim of this study was to establish the urogynecological and sexual functions after Vecchietti's operation. Study Design Materials and Methods: Fifteen patients with MRKHS who underwent laparoscopic Vecchietti's operation were included. A control group of 15 age-matched, childless, sexually active women were examined during the same period. All patients underwent the basic evaluation of anatomical outcomes. Sexual outcomes were established by the Polish validated Female Sexual Function Index (FSFI) questionnaire. Continence status was assessed by Polish validated Urinary Distress Inventory (UDI-6) and the Incontinence Impact Questionnaire (IIQ-7). Results: Mean age of MRKH group was 22.06±5.13 yrs. Mean follow-up after surgery was 8.02±3.43 yrs. Mean age of women from control group was 22.4±4.35. Mean FSFI scores show good quality of sexual life in both groups. UDI-6 scores showed that patients after Vecchietti surgery have urogynecological problems significantly more often than healthy women do. Based on the IIQ-7, it is evident that one patient from the MRKH group (6,6%) suffers from stress urinary incontinence and the rest (20%) have rather irritative problems with the functioning of the lower urinary tract. Conclusion: Quality of sexual life after the Vecchietti's operation in long-term follow-up does not differ from that of healthy women, but these patients suffer more frequent from urogynecological complaints. The trial is registered with NCT03809819.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Amenorreia/fisiopatologia , Anormalidades Congênitas/fisiopatologia , Laparoscopia/efeitos adversos , Ductos Paramesonéfricos/anormalidades , Vagina/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/fisiopatologia , Adolescente , Adulto , Amenorreia/etiologia , Colo do Útero/fisiopatologia , Colo do Útero/cirurgia , Anormalidades Congênitas/etiologia , Anormalidades Congênitas/cirurgia , Feminino , Humanos , Ductos Paramesonéfricos/fisiopatologia , Ductos Paramesonéfricos/cirurgia , Polônia , Comportamento Sexual , Saúde Sexual , Inquéritos e Questionários , Útero/fisiopatologia , Útero/cirurgia , Vagina/fisiopatologia , Saúde da Mulher , Adulto Jovem
20.
J Obstet Gynaecol Res ; 45(7): 1398-1403, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30916459

RESUMO

Mayer-Rokitansky-Küster-Hauser syndrome is characterized by aplasia of the uterus and the upper ⅔ of the vagina, with normal female genotype (46,XX) and the development of secondary sexual characters. One of the most popular surgical treatment is the McIndoe procedure, which major drawbacks are the scar of split thickness skin graft (STSG)harvesting, and risk of vaginal stenosis for graft retraction. We report the case of a patient, operated modifying the McIndoe technique, by harvesting a full thickness skin grafts (FTSG) from patient's breasts, undergone simultaneous reduction mammoplasty. The FTSG provided a complete lining of the reconstructed neovagina. At 6 months, we observed complete mucinous metaplasia of the vaginal walls and 1 year after surgery no sign of stenosis, without the use of the mold. The advantages of this technique are reduced donor site morbidity, when compared with traditional McIndoe technique, and reduced risk of neovagina retraction ensured by FTSG, compared with STSG. If future patients' series confirm the good results achieved, we could consider it as a first-line option in patients undergoing neovagina reconstruction, seeking breasts or body contouring surgery.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Doenças Mamárias/cirurgia , Anormalidades Congênitas/cirurgia , Ductos Paramesonéfricos/anormalidades , Procedimentos Cirúrgicos Reconstrutivos/métodos , Transplante de Pele/métodos , Vagina/anormalidades , Transtornos 46, XX do Desenvolvimento Sexual/complicações , Transtornos 46, XX do Desenvolvimento Sexual/patologia , Doenças Mamárias/congênito , Doenças Mamárias/patologia , Terapia Combinada , Anormalidades Congênitas/genética , Anormalidades Congênitas/patologia , Estética , Feminino , Humanos , Hipertrofia , Ductos Paramesonéfricos/patologia , Ductos Paramesonéfricos/cirurgia , Vagina/cirurgia , Adulto Jovem
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