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2.
HNO ; 72(1): 57-68, 2024 Jan.
Artigo em Alemão | MEDLINE | ID: mdl-38047932

RESUMO

Congenital malformations of the pinna and aural atresia can result in major aesthetic and functional deficits. Knowledge about embryologic developments and established classification systems is an essential requirement when dealing with affected patients. Early detection of deficiencies and introduction of appropriate diagnostic measures is vital to initiate adequate therapies and prevent long-term disabilities. Treatment for malformations of the pinna-if requested-is mostly surgical, infrequently an epithesis is applied. As in other surgical fields, tissue engineering will likely play a crucial role in the future. Treatment of aural stenosis and atresia aims at improvement of hearing levels and prevention of secondary complications like cholesteatoma and chronic otorrhea. Auditory rehabilitation comprises a spectrum from conventional hearing aids to invasive hearing implants, the latter being favored in recent years.


Assuntos
Anormalidades Congênitas , Microtia Congênita , Otopatias , Humanos , Anormalidades Congênitas/terapia , Anormalidades Congênitas/cirurgia , Microtia Congênita/diagnóstico , Microtia Congênita/terapia , Microtia Congênita/complicações , Otopatias/diagnóstico , Otopatias/terapia , Orelha Externa , Audição , Testes Auditivos
3.
J Pediatr Surg ; 57(6): 1067-1071, 2022 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-35264304

RESUMO

BACKGROUND: There is a paucity of data on the frequency of transfusion during pediatric surgery index cases and guidelines for pretransfusion testing, defined as type and screen and crossmatch testing, prior to operation are not standardized. This study aimed to determine the incidence of perioperative blood transfusions during index neonatal operations and identify risk factors associated with perioperative blood transfusion to determine which patients benefit from pretransfusion testing. METHODS: A retrospective review of infants who underwent index neonatal cases between 2013 and 2019 was performed. Data were collected for patients who underwent operations for Hirschsprung's disease, esophageal atresia/tracheoesophageal fistula (EA/TEF), biliary atresia, anorectal malformation, omphalocele, gastroschisis, duodenal atresia, congenital diaphragmatic hernia (non-ECMO) or pulmonary lobectomy. Infants under 6 months were included except in the case of lobectomy where infants up to 12 months were included. RESULTS: Analysis was performed on 420 patients. Twenty-five (6.0%) patients received perioperative blood transfusion. Patients who received perioperative transfusion most commonly underwent EA/TEF repair. Patients who received perioperative transfusion had higher rates of structural heart disease (52.0% vs 17.7%, p<0.001), preoperative transfusion (48.0% vs 8.9%, p<0.001), and prematurity (52.0% vs 25.6%, p = 0.005). Presence of all three risk factors resulted in a 48% probability of requiring perioperative transfusion. CONCLUSIONS: Blood transfusion during the perioperative period of neonatal index operations is rare. Factors associated with increased risk of perioperative transfusion include prematurity, structural heart disease, and history of previous blood transfusion. LEVEL OF EVIDENCE: III.


Assuntos
Transfusão de Sangue , Anormalidades Congênitas , Criança , Anormalidades Congênitas/cirurgia , Anormalidades Congênitas/terapia , Atresia Esofágica/complicações , Atresia Esofágica/cirurgia , Cardiopatias/congênito , Cardiopatias/cirurgia , Humanos , Incidência , Lactente , Recém-Nascido , Período Perioperatório , Estudos Retrospectivos , Fatores de Risco , Fístula Traqueoesofágica/complicações , Fístula Traqueoesofágica/epidemiologia , Fístula Traqueoesofágica/cirurgia
4.
Urol Int ; 106(2): 195-198, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-33957634

RESUMO

INTRODUCTION: The urachus is an embryologic remnant which is formed from the obliteration of the allantois. Urachal abnormalities are caused when defective obliteration of the urachus happens. They are an infrequent condition. Incidence is estimated to be between 5,000 and 8,000 live births. Its diagnosis and management remain a challenge due to the lack of an specific clinical picture and the controversy about the management. OBJECTIVE: The objective of this study is to assess the clinical presentation, diagnosis, therapeutic management, and outcomes of urachal anomalies in our health area. MATERIALS AND METHODS: We performed a retrospective review of all cases of urachal anomalies recorded Tenerife (southern health area), La Gomera, and El Hierro Islands during a 5 year period. RESULTS: Twenty-three cases of urachal pathology were included. The mean age of presentation was 32 years old. 73.9% were male. 65% were diagnosed in adults. In 30.3% of the cases, it was a casual finding. Symptoms included fever, umbilical exudate, hematuria, abdominal pain, and umbilical granuloma. The main diagnostic tests were ultrasound and computed tomography scan. Treatments were selected conservative management (43.5%), selective resection, partial cystectomy, and radical cystectomy. All patients had a good evolution. However, 2 cases where benign tumors were suspected, clinically, had a final histology of cancer in the specimen. CONCLUSIONS: Due to the lack of a specific clinical picture and undefined findings in image tools, diagnosis is difficult and it may be inaccurate. Despite more data are needed, our results suggest that the systematic excision of urachal lesions could result in safer outcomes since cases where a benign lesion is clinically suspected might result in malignant tumors.


Assuntos
Úraco/anormalidades , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/terapia , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
5.
Rev. chil. obstet. ginecol. (En línea) ; 86(6): 591-622, dic. 2021. ilus, tab
Artigo em Espanhol | LILACS | ID: biblio-1388701

RESUMO

Resumen La Sociedad Chilena de Infectología, a través de su Comité de Infecciones Neonatales, en conjunto con la Sociedad Chilena de Obstetricia y Ginecología, proponen un documento de diagnóstico y manejo de la infección por citomegalovirus (CMV) en la mujer embarazada y el recién nacido. Esta guía aborda el manejo de la infección en el binomio, su enfrentamiento diagnóstico y terapéutico, orientado al equipo de salud que atiende a mujeres embarazadas y recién nacidos con infección por CMV en Chile. Considera la situación epidemiológica global y latinoamericana, con recomendaciones para la evaluación clínica y de laboratorio; establece criterios de diagnóstico, propone enfoques terapéuticos de acuerdo a la situación clínica, analiza las medidas de prevención y establece una propuesta nacional para el seguimiento de esta enfermedad. Se ha puesto especial énfasis en entregar, de forma práctica, y con la mayor evidencia posible, las recomendaciones para el manejo del binomio con infección por CMV.


Abstract The Chilean Society of Infectology, through its Neonatal Infections Committee in conjunction with the Chilean Society of Obstetrics and Gynecology, propose a document for the Diagnosis and Management of Cytomegalovirus Infection in Pregnancy and Newborn. This guideline suggests the management of mother and child infection, its diagnostic and therapeutic options. Considers the global and Latin American epidemiology, with recommendations for clinical and laboratory evaluation, diagnostic criteria, therapeutic approaches according to the clinical situation, analyzes prevention measures and establishes a national proposal for monitoring this disease.


Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Complicações Infecciosas na Gravidez/diagnóstico , Complicações Infecciosas na Gravidez/terapia , Infecções por Citomegalovirus/diagnóstico , Infecções por Citomegalovirus/terapia , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/terapia , Diagnóstico Pré-Natal , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/terapia , Infecções por Citomegalovirus/congênito
6.
Pediatr Rev ; 42(4): 180-188, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33795464

RESUMO

Congenital ear abnormalities present an aesthetic and psychosocial concern for pediatric patients and their parents. Diagnosis of external ear deformities is based on clinical examination and is facilitated by an understanding of normal ear anatomy. Ear anomalies can be categorized as malformations or deformations. Malformations are characterized by absent anatomical structures of the ear (or absence of the ear itself), as exemplified by microtia and anotia. Ear deformations are characterized by ear anatomical landmarks that are present but are distorted or abnormal, with Stahl ear, constricted ear, and prominent ear being common presentations. Ear malformations will not improve with growth of the patient and uniformly require surgical intervention to recreate an anatomically typical ear. Although a small percentage of ear deformations can self-resolve, most patients with ear deformations will require nonsurgical or surgical reconstruction to achieve a normal or more aesthetic ear. In recent decades the use of nonsurgical ear splinting or molding has been recognized as a highly effective method in correcting a variety of congenital ear deformations when treatment is initiated in the first 8 weeks of life. The urgency in initiating nonsurgical treatment of ear deformations at an early age makes prompt recognition of these ear deformations essential because surgical correction remains the only viable reconstructive option in older infants and children.


Assuntos
Anormalidades Congênitas , Procedimentos de Cirurgia Plástica , Idoso , Criança , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/terapia , Orelha Externa/cirurgia , Face , Humanos , Lactente , Pais
7.
Rev. medica electron ; 43(2)mar.-abr. 2021.
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1251950

RESUMO

El tratamiento del pie zambo congénito ha evolucionado a lo largo de la historia. Desde la Antigüedad hasta finales de la Edad Media se utilizaron las manipulaciones e inmovilizaciones seriadas. Del Renacimiento al siglo XVII se crearon las primeras ortesis. En el siglo XVIII comenzó el uso de moldes de yeso y se desarrollaron ortesis y calzados complejos. El período del siglo XIX hasta la tercera década del XX, se caracterizó por la práctica de las tenotomías, siendo la cirugía el principal enfoque terapéutico. En el siglo XX, Joseph Kite e Ignacio Ponseti describieron su eficaz método no quirúrgico, lo que produjo el regreso a las manipulaciones e inmovilizaciones seriadas frente a la cirugía agresiva. Cuando se revisa la historia del tratamiento del pie zambo, sorprende ver que los médicos tratantes cometían los mismos errores una y otra vez, porque ignoraban constantemente lo que ya habían aprendido de sus antecesores y, en su lugar, a menudo se veían confundidos por las nuevas informaciones o tendencias. En el siglo XXI, los avances en biología celular, genética molecular, diagnóstico por la imagen, biomecánica y biomateriales hacen prever que se puedan diseñar tratamientos personalizados para los pacientes con pie zambo(AU)


Congenital clubfoot treatment has evolved throughout history. Serial manipulations and immobilizations were used from antiquity to the end of the Middle Ages. From the Renaissance to the 17th century the first orthotics were created. In the 18th century, the use of plaster casts began and complex orthotics and footwear developed. The period from 19th century until the third decade of the 20th century was characterized by the practice of tenotomies, with surgery being the main therapeutic approach. In the 20th century, Joseph Kite and Ignacio Ponseti described their effective non-surgical method, which led to the return to serial manipulations and immobilizations in the face of aggressive surgery. When reviewing the history of clubfoot treatment, it is surprising to see that the treating doctors made the same mistakes over and over again because they constantly ignored what they had already learned from their predecessors and, instead, were often confused by the new ones information or trends. In the 21st century, advances in cell biology, molecular genetics, diagnostic imaging, biomechanics and biomaterials suggest that personalized treatments can be designed for patients with clubfoot(AU)


Assuntos
Humanos , Masculino , Feminino , Procedimentos Ortopédicos/história , Pé Torto/congênito , História da Medicina , Terapêutica/história , Terapêutica/métodos , Anormalidades Congênitas/história , Anormalidades Congênitas/terapia , Pé Torto/história
8.
Otolaryngol Head Neck Surg ; 164(3): 489-500, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-32807006

RESUMO

OBJECTIVE: Endoscopic surgical management or injection laryngoplasty of type 1 laryngeal clefts in pediatric patients is used in those who do not respond to conservative treatment. This study compares conservative treatment, endoscopic surgical repair, and injection laryngoplasty for the management of type 1 laryngeal clefts. DATA SOURCES: PubMed, Web of Science, and Embase. REVIEW METHODS: This systematic review included studies of patients with type 1 laryngeal clefts who were managed with conservative treatment, injection laryngoplasty, or endoscopic repair, and all studies reported postintervention outcomes. Two independent investigators assessed study eligibility, rated the quality, and extracted data for analysis. A random effects model was used for meta-analysis of pooled data. RESULTS: Of the 1209 studies identified, 27 met inclusion criteria. There were 543 patients with type 1 laryngeal clefts represented in the studies, with outcomes reported for 537. Conservative therapy had a 52% (95% CI, 37%-66%; I2 = 63%) success rate at improving symptoms, while endoscopic repair had a significantly higher percentage resolution of symptoms (70%; 95% CI, 59%-79%; I2 = 62%, P < .001) as compared with conservative treatment (51%; 95% CI, 36%-65%; I2 = 62%) or injection laryngoplasty (36%; 95% CI, 20%-57%; I2 = 70%). The quality scores of the studies ranged from 7 to 12 out of 16. CONCLUSION: Our systematic review demonstrated significant improvement and resolution of symptoms for patients with type 1 laryngeal clefts treated with endoscopic repair as compared with other modalities. More prospective and controlled studies comparing treatment strategies with validated instruments to measure outcomes are necessary to determine their efficacy in the management of type 1 laryngeal clefts.


Assuntos
Anormalidades Congênitas/terapia , Laringe/anormalidades , Anormalidades Congênitas/classificação , Tratamento Conservador , Humanos , Laringoplastia/métodos , Laringoscopia
9.
J Plast Reconstr Aesthet Surg ; 74(2): 377-381, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33071167

RESUMO

INTRODUCTION: Cryptotia is characterized by an absence of the upper part of the temporoauricular sulcus with the superior third of the auricle buried under the temporal skin. The principle of correction of cryptotia is to achieve both a functional and aesthetic ear. METHODOLOGY: A 4-year prospective study was carried out in a single centre on infants with cryptotia. We introduce a two-staged approach for non-surgical correction of cryptotia and a treatment algorithm based on the age of the patient. In the first stage, we unbury the auricle over 2 weeks. The second stage involves 2 additional weeks of helical moulding to correct the underlying or residual deformation. In our algorithm, patients more than 6 weeks of age undergo only the first stage of cryptotia correction, whereas those less than 6 weeks of age progress to the second stage of treatment. RESULTS: A total of 5 patients (7 ears) were treated with ages ranging from 1 day to 7 months. Two patients underwent 2-stage treatment and 3 underwent 1-stage treatment. At 1 year, all buried helices remained retracted. Four out of 7 ears treated had "Excellent" outcomes and 3 out of 7 ears had "Good" outcomes. All caregivers were extremely satisfied with the outcomes. CONCLUSION: This simple and efficacious two-stage technique and algorithm is effective in the correction of cryptotia, even if the treatment is initiated beyond the traditional moulding window in the first six weeks of life. Using this approach, patients with cryptotia benefit by avoiding or simplifying surgical reconstruction in the future.


Assuntos
Anormalidades Congênitas/terapia , Cartilagem da Orelha/anormalidades , Tração/métodos , Algoritmos , Tomada de Decisão Clínica/métodos , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Tração/instrumentação , Resultado do Tratamento
10.
Urology ; 149: 199-205, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33189736

RESUMO

OBJECTIVE: To describe 5 cases with complete urinary bladder duplication, their associated conditions, and their respective treatment. Urinary bladder duplication is an extremely rare congenital anomaly of the urinary system. So far about 70 cases have been published in the English literature, most of them as case reports and a few case series. METHODS AND RESULTS: All consecutive patients with bladder duplication treated at our institution between 2000 and 2015 were included. Patient records were retrospectively analyzed, and 5 patients with urinary bladder duplication were identified (see Summary Figure). Two patients were male. All duplications were recognized by health care providers. In 1 case recognition was prenatal (MRI in utero at 22 weeks of gestation), the latest recognition was at 12 months of age. A voiding cystourethrography was performed in 4 patients to confirm the diagnosis. In 4 patients the bladder duplication could be classified according to Abrahamson with 3 complete reduplications and one complete sagittal septum. All patients suffered from associated congenital diseases, but only one patient had urinary tract infections. Surgical treatment was only performed in one patient. Median follow-up was 34 months. DISCUSSION: Urinary bladder duplications reflect extremely seldom disorders that are almost always associated with other congenital anomalies. Treatment depends on patients' symptoms and associated conditions and hence needs to be individualized to each patient.


Assuntos
Bexiga Urinária/anormalidades , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/terapia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
11.
Surgery ; 169(2): 311-317, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33097243

RESUMO

BACKGROUND: The true incidence of congenital anomalies in sub-Saharan Africa is unknown. Owing to complex challenges associated with congenital anomalies, many affected babies may never present to a health facility, resulting in an underestimation of disease burden. METHODS: Interviews were conducted with Ugandans between September 2018 and May 2019. Responses from community members versus families of children with congenital anomalies were compared. RESULTS: A total of 198 Ugandans were interviewed (91 family members, 80 community members). All participants (N = 198) believed that seeking surgical care would lead to poverty, 43% (n = 84) assumed fathers would abandon the child, and 26% (n = 45) thought a child with a congenital anomaly in their community had been left to die. Causes of anomalies were believed to be contraceptive methods (48%, n = 95), witchcraft (17%, n = 34), or drugs (10%, n = 19). Of family members, 25 (28%) were advised to allow the child to die. Families with affected children were more likely to have a lower income (P < .001), believe anomalies could be treated (P = .007), but thought that allowing the child to die was best for the family (32% vs 9%; P < .0001). Monthly household income <50,000 Uganda shillings ($13 United States dollars) was a significant predictor of the father leaving the family (P = .024), being advised to not pursue medical care (P = .046), and believing that God should decide the child's fate (P = .047). CONCLUSION: Families face significant financial and social pressures when deciding to seek surgical care for a child with a congenital anomaly. Many children with anomalies may die and never reach a health facility to be counted, thus contributing to a hidden mortality.


Assuntos
Mortalidade da Criança , Anormalidades Congênitas/mortalidade , Aceitação pelo Paciente de Cuidados de Saúde/psicologia , Pobreza/economia , Estigma Social , Adulto , Criança , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/economia , Anormalidades Congênitas/terapia , Efeitos Psicossociais da Doença , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Incidência , Masculino , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Pobreza/psicologia , Inquéritos e Questionários/estatística & dados numéricos , Uganda/epidemiologia , Adulto Jovem
12.
Artigo em Chinês | MEDLINE | ID: mdl-33040500

RESUMO

Objective:To report the nonsurgical correction of congenital auricular deformities in children older than 3 months, analysis the effect and the recurrence and the influencing factors. Method:Patients with auricular deformities who came to our department from July 2017 to August 2019 were collected. EarWell correction was performed for non-invasive correction. Follow-up was performed for at least 3 months after treatment. Data was collected to analysis the effect and the recurrence and the influencing factors. Result:At the end of follow-up, 76 cases of 88 ears were collected, at the end of treatment in this group, the efficiency was 87.5%, and the recurrence rate was 19.48%, 3 months after the end of treatment. There was a statistically significant difference in the distribution of auricle deformities(P=0.018) and the age of first treatment(P=0.028) between children in the effective group and those in the ineffective group. Of all the auricle deformities, the treatment of cryptotia was the most effective, and the effectiveness of prominent ears was the lowest. The family history(P=0.314), gender(P=0.421), and feeding method(P=0.557) of the effective and ineffective groups. There was no significant difference in the gestational weeks at birth(P=0.641), the mode of production(P=0.849), and birth weight(P=0.08). There was no significant difference in age between the relapsed group and the non-relapsed group at the age of first treatment(P=0.833).There was significant difference in the distribution of auricle deformities between the relapsed group and the non-relapsed group(P=0.013). There was no statistically significant difference between the effective group and the ineffective group at the age of first diagnosis and treatment time if we exclude cryptotia. Conclusion:For children who are treated beyond the treatment time window, the main factor affecting the treatment effect is the type of deformity. Nonsurgical correction can still be tried for older than 3 months with auricular deformities, especially for cryptotia, ear wheel deformities, and auricular cavity deformities. We do not recommend to try nonsurgical correction for children older than 3 months with prominent ears and cup ears.


Assuntos
Anormalidades Congênitas , Pavilhão Auricular , Orelha Externa , Criança , Anormalidades Congênitas/terapia , Pavilhão Auricular/anormalidades , Orelha Externa/anormalidades , Humanos , Lactente , Recém-Nascido , Anamnese , Recidiva
13.
Semin Perinatol ; 44(7): 151282, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-32819725

RESUMO

As we confront COVID-19, the global public health emergency of our times, new knowledge is emerging that, combined with information from prior epidemics, can provide insights on how to manage this threat in specific patient populations. Severe Acute Respiratory Syndrome (SARS) and Middle East Respiratory Syndrome (MERS), both caused by coronaviruses, caused serious respiratory illness in pregnant women that resulted in adverse perinatal outcomes. Thus far, COVID-19 appears to follow a mild course in the vast majority of pregnant women. A significant proportion of pregnant women appear to be asymptomatic carriers of SARS-CoV-2. However, there is limited information on how COVID-19 impacts the fetus and whether vertical transmission occurs. While these knowledge gaps are addressed, it is important to recognize the highly efficient transmission characteristics of SARS-C0V-2 and its potential for causing serious disease in vulnerable individuals, including health care workers. This review provides perspectives from a single center in New York City, the epicenter of the pandemic within the United States. It offers an overview of the preparations required for deliveries of newborns of mothers with COVID-19 and the management of neonates with particular emphasis on those born with complex issues.


Assuntos
COVID-19 , Anormalidades Congênitas/terapia , Terapia Intensiva Neonatal/métodos , Complicações Infecciosas na Gravidez , Prática Avançada de Enfermagem , Teste para COVID-19 , Atresia Esofágica/terapia , Oxigenação por Membrana Extracorpórea , Feminino , Cardiopatias Congênitas/terapia , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Recém-Nascido , Controle de Infecções , Transmissão Vertical de Doenças Infecciosas , Terapia Intensiva Neonatal/organização & administração , Neonatologistas , Enfermeiros Neonatologistas , Planejamento de Assistência ao Paciente , Equipe de Assistência ao Paciente/organização & administração , Isolamento de Pacientes , Isoladores de Pacientes , Gravidez , Procedimentos de Cirurgia Plástica , Ressuscitação/métodos , SARS-CoV-2 , Fatores de Tempo , Fístula Traqueoesofágica/terapia
15.
World J Surg ; 44(11): 3616-3619, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32642795

RESUMO

BACKGROUND: Congenital anomalies are one component of the overwhelming surgical disease burden in low- and middle-income countries (LMICs). Lymphatic malformations (LMs) are a common congenital deformity of the head and neck in which the utilization of sclerotherapy may avoid surgery and yield superior outcomes. To be useful in LMICs, sclerosing agents must be widely available, inexpensive, and effective. METHODS: A retrospective review of 10 pediatric patients with macrocystic or mixed LMs who were treated with self-compounded doxycycline sclerotherapy at Rwanda's Central University Teaching Hospital of Kigali was performed. Doxycycline oral tablets were crushed by hand, mixed with normal saline at a concentration of doxycycline 10 mg/mL, and injected directly into LMs of the head and neck. RESULTS: Ten pediatric patients underwent 21 sclerotherapy sessions with a mean of 2.1 sessions per patient (SD 1.3, range 1-5). Of the 8 patients that were seen in follow-up, all achieved at least 80% resolution, 6 of 8 achieved 100% resolution, and none required surgery. One patient developed an infection at the injection site which resolved with antibiotics. CONCLUSIONS: Self-compounded doxycycline sclerotherapy is a safe, effective, and widely available treatment option for sclerotherapy of LMs in LMICs.


Assuntos
Anormalidades Congênitas/terapia , Doxiciclina , Escleroterapia , Criança , Doxiciclina/uso terapêutico , Cabeça/anormalidades , Humanos , Pescoço/anormalidades , Estudos Retrospectivos , Ruanda , Resultado do Tratamento
16.
Gynecol Endocrinol ; 36(8): 746-748, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31994956

RESUMO

Vaginal dilation, currently considered as the first-line therapy for vaginal aplasia in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, is a safe and effective treatment that aims to create a functional neovagina. However, rigid vaginal dilators classically described in the literature usually cause physical discomfort and side effects that can lead to vaginal necrosis. Here, we present two cases of MRKH syndrome patients with vaginal agenesis whose main complaint was the inability to have sexual intercourse with their partners. Considering unavailability of acrylic dilators and previous studies reporting good responses with the use of silicone dilators in women with post-radiotherapy vaginal stenosis, the medical team and patients opted for creation of a neovagina through the daily use of silicone vaginal dilators. Patient 1 developed an 8-cm vagina after 6 months of treatment and had a satisfactory sex life with her partner. Patient 2 developed a 7-cm vagina and reported significant symptom improvement. None of the patients developed side effects after the treatment. The use of inexpensive and easily accessible silicone vaginal dilators may be an effective and noninvasive alternative with few side effects for women with vaginal agenesis, particularly in the developing countries.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/terapia , Anormalidades Congênitas/terapia , Dilatação , Ductos Paramesonéfricos/anormalidades , Vagina/anormalidades , Vagina/patologia , Transtornos 46, XX do Desenvolvimento Sexual/patologia , Adulto , Coito/fisiologia , Anormalidades Congênitas/patologia , Tratamento Conservador , Dilatação/instrumentação , Dilatação/métodos , Feminino , Procedimentos Cirúrgicos em Ginecologia/efeitos adversos , Procedimentos Cirúrgicos em Ginecologia/instrumentação , Procedimentos Cirúrgicos em Ginecologia/métodos , Humanos , Ductos Paramesonéfricos/patologia , Resultado do Tratamento , Adulto Jovem
17.
J Minim Invasive Gynecol ; 27(1): 74-79, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31129299

RESUMO

STUDY OBJECTIVE: To evaluate the treatment of patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with a combination of oocyte retrieval and surgical vaginoplasty in a single laparoscopic procedure. DESIGN: A case series. SETTING: The study was conducted at 2 tertiary referral facilities for MRKH syndrome in Milan, Italy, between July 2017 and September 2018. PATIENTS: Eleven patients presented with MRKH and required surgical vaginoplasty while expressing a desire for future fertility. INTERVENTIONS: Two experienced surgeons and an expert in assisted reproductive technology performed concomitant vaginoplasty according to the modified technique of Davydov and laparoscopic oocyte retrieval for gamete cryopreservation. MEASUREMENTS AND MAIN RESULTS: Before the procedure, patients underwent extensive counseling and gave written consent. At the start of surgery, 10.4 ± 4.4 (mean ± standard deviation [SD]) oocytes were retrieved laparoscopically, and 8.8 ± 3.1 (SD) mean mature oocytes were cryopreserved. After oocyte retrieval, the steps of the modified Davydov technique were followed. The total operative time was 116 ± 16 minutes (mean ± SD), and no intraoperative/postoperative complications were observed. CONCLUSION: This is the first report of combined oocyte retrieval and vaginoplasty for patients with MRKH syndrome. The approach was found to be feasible in patients with a desire for future fertility. It is our belief that physicians treating patients with MRKH should refer patients to centers with expertise in both vaginoplasty and assisted reproductive technology.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/terapia , Anormalidades Congênitas/terapia , Preservação da Fertilidade/métodos , Procedimentos Cirúrgicos em Ginecologia/métodos , Laparoscopia/métodos , Ductos Paramesonéfricos/anormalidades , Recuperação de Oócitos/métodos , Procedimentos de Cirurgia Plástica/métodos , Vagina/cirurgia , Transtornos 46, XX do Desenvolvimento Sexual/cirurgia , Adolescente , Adulto , Terapia Combinada , Anormalidades Congênitas/cirurgia , Criopreservação , Estudos de Viabilidade , Feminino , Seguimentos , Humanos , Complicações Intraoperatórias/etiologia , Itália , Ductos Paramesonéfricos/cirurgia , Duração da Cirurgia , Indução da Ovulação/métodos , Complicações Pós-Operatórias/etiologia , Adulto Jovem
18.
J Pediatr Surg ; 55(8): 1507-1510, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31443920

RESUMO

PURPOSE: Perineal groove is a rare congenital anomaly of the perineum, and only a few papers describing a small number of cases have been reported in the medical literature. This study aimed to evaluate the clinical characteristics and proper management of perineal groove. METHODS: We performed a retrospective review of 26 pediatric patients who were diagnosed with perineal groove between January 2012 and October 2018 at our institution. RESULTS: Perineal groove was extremely prevalent among the females: 25 of the 26 patients were girls, and only one patient was a boy. All the patients presented with an unusual lesion at the anus or perineum, but no symptoms related to this anomaly. The median age at the first visit to our clinic was 1.5 month (range, 0.3-11.4 month). Two types, complete and partial, were used to categorize the appearance of perineal groove. In a sample group, 55% (12/22) of the patients had complete perineal groove while 45% (10/22) had partial perineal groove. One patient underwent an anoplasty at another hospital following the diagnosis of an imperforate anus. One male and 13 female patients were followed beyond the age of two, and 10 patients (71%) showed a natural healing process. CONCLUSION: Perineal groove manifested as two types of appearance and showed excellent results with conservative treatment in our study. A natural healing process can be expected in the long-term follow-up. Perineal groove must be differentiated from other defects to avoid unnecessary surgical treatment. LEVELS OF EVIDENCE: Therapeutic Study, Level IV.


Assuntos
Anormalidades Congênitas/terapia , Tratamento Conservador , Períneo/anormalidades , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos
19.
J Obstet Gynaecol Res ; 46(3): 542-546, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31854484

RESUMO

Vaginal creation is the standard treatment for Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Although non-surgical method is recommended as a first-line treatment in the American College of Obstetricians and Gynecologists guidelines for gynecological practice, it is not commonly performed in Japan. At our hospital, vaginal dilation using uterine cervical dilators (Hegar's dilator) is performed for patients with MRKH syndrome. We report four cases successfully treated with vaginal dilation. After the examination, patients were instructed to practice daily self-dilation at home. The initiation size was No. 13 with 10.5-mm diameter. After the vaginal cavity was dilated to a depth of 6 cm, the size of dilators was gradually increased until No. 30 with 25-mm diameter in a tip and 28-mm diameter in a trunk. The duration required to achieve the outcome was 5-22 months. All cases were successfully treated without any severe complication.


Assuntos
Transtornos 46, XX do Desenvolvimento Sexual/terapia , Anormalidades Congênitas/terapia , Ductos Paramesonéfricos/anormalidades , Vagina/diagnóstico por imagem , Transtornos 46, XX do Desenvolvimento Sexual/diagnóstico por imagem , Adolescente , Adulto , Anormalidades Congênitas/diagnóstico por imagem , Dilatação/métodos , Feminino , Humanos , Imageamento por Ressonância Magnética , Ductos Paramesonéfricos/diagnóstico por imagem , Resultado do Tratamento
20.
Artigo em Inglês, Português | LILACS, SES-SP | ID: biblio-1136726

RESUMO

ABSTRACT Objective: To describe the reports of parents of newborns (NB) with congenital malformations hospitalized in a Neonatal Intensive Care Unit (NICU) who received bad news, in order to identify the issues related to the perception of bad news given adequately or inadequately. Methods: A cross-sectional study was conducted from January to October 2018, in which parents of newborns with congenital malformations hospitalized in NICUs were interviewed at visiting hours, according to inclusion criteria. The questionnaire had semi-structured questions related to reception of bad news. Analysis of the data was descriptive. Results: 28 mothers and two fathers were interviewed and 16 (53.3%) reported having had at least one bad news in the NICU. Of those, 10 (62.5%) considered appropriate the way in which the news was given. The justifications were: sincerity of the professional, delicacy to give the news, giving hope to the family, use of appropriate words and demonstration of caring about the newborn. Six participants (37.5%) considered inadequate the way of breaking bad news. The reasons were: unpreparedness and lack of knowledge about the child's case, use of difficult language, haste or anxiety and discouragement of family hope. Most of the news was given by a professional alone, often by a medical resident. Conclusions: The communication of bad news was considered adequate by the parents, although this perception was not unanimous. This study, therefore, indicates that it is necessary to improve the communication of bad news in this NICU. Training professionals can assist in this process.


RESUMO Objetivo: Descrever os relatos dos pais de recém-nascidos (RNs) com malformações congênitas internados em uma Unidade de Terapia Intensiva Neonatal (UTIN) a respeito de como receberam as más notícias, buscando identificar as questões relacionadas à percepção de uma má notícia fornecida de forma adequada ou inadequada. Métodos: Realizou-se estudo transversal de janeiro a outubro de 2018, no qual se entrevistaram pais de RNs portadores de malformações congênitas internados em UTIN selecionados segundo critérios de inclusão e presentes em horário de visita. O questionário utilizado tinha questões semiestruturadas pertinentes ao recebimento de más notícias. A análise foi descritiva. Resultados: Entrevistaram-se 28 mães e dois pais, dos quais 16 (53,3%) apontaram ter tido pelo menos uma má notícia na UTIN. Destes, 10 (62,5%) consideraram adequada a maneira de dar essa notícia. As justificativas foram: sinceridade do profissional, delicadeza para dar a notícia, dar esperança à família, uso de palavras adequadas e cuidado demonstrado com o RN. Seis participantes (37,5%) avaliaram como inadequada a comunicação de más notícias. Motivos foram despreparo e falta de conhecimento, uso de linguagem difícil, pressa ou ansiedade e desencorajamento de esperanças da família. A maior parte das notícias foi dada por um profissional sozinho, muitas vezes por um médico residente. Conclusões: A percepção da comunicação de más notícias foi considerada adequada por parte dos pais, embora não tenha sido unânime. Este estudo aponta ser necessário melhorar a comunicação dessas notícias na UTIN analisada. O treinamento dos profissionais, nesse sentido, pode auxiliar nesse processo.


Assuntos
Humanos , Masculino , Feminino , Recém-Nascido , Adolescente , Adulto , Adulto Jovem , Pais/psicologia , Relações Profissional-Família , Revelação da Verdade , Anormalidades Congênitas/diagnóstico , Anormalidades Congênitas/terapia , Terapia Intensiva Neonatal , Percepção , Atitude Frente a Saúde , Unidades de Terapia Intensiva Neonatal , Estudos Transversais , Estudos Prospectivos
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