A atresia colônica é uma malformação intestinal congênita que resulta em um segmento não latente do cólon e caracterizada por obstrução intestinal inferior que se manifesta com distensão abdominal e falha na passagem de mecônio em recém-nascidos.
Introdução
O que você precisa saber de cara
A atresia colônica é uma malformação intestinal congênita que resulta em um segmento não latente do cólon e caracterizada por obstrução intestinal inferior que se manifesta com distensão abdominal e falha na passagem de mecônio em recém-nascidos.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 8 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Atresia do cólon
Centros de Referência SUS
24 centros habilitados pelo SUS para Atresia do cólon
Centros para Atresia do cólon
Detalhes dos centros
Hospital Universitário Prof. Edgard Santos (HUPES)
R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808
Serviço de Referência
Hospital Infantil Albert Sabin
R. Tertuliano Sales, 544 - Vila União, Fortaleza - CE, 60410-794 · CNES 2407876
Serviço de Referência
Hospital de Apoio de Brasília (HAB)
AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456
Serviço de Referência
Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)
Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207
Serviço de Referência
Hospital das Clínicas da UFG
Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424
Serviço de Referência
Hospital Universitário da UFJF
R. Catulo Breviglieri, Bairro - s/n - Santa Catarina, Juiz de Fora - MG, 36036-110 · CNES 2297442
Atenção Especializada
Hospital das Clínicas da UFMG
Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167
Serviço de Referência
Hospital Universitário Julio Müller (HUJM)
R. Luis Philippe Pereira Leite, s/n - Alvorada, Cuiabá - MT, 78048-902 · CNES 2726092
Atenção Especializada
Hospital Universitário João de Barros Barreto
R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878
Serviço de Referência
Hospital Universitário Lauro Wanderley (HULW)
R. Tabeliao Estanislau Eloy, 585 - Castelo Branco, João Pessoa - PB, 58050-585 · CNES 0002470
Atenção Especializada
Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)
R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647
Serviço de Referência
Hospital Pequeno Príncipe
R. Des. Motta, 1070 - Água Verde, Curitiba - PR, 80250-060 · CNES 3143805
Serviço de Referência
Hospital Universitário Regional de Maringá (HUM)
Av. Mandacaru, 1590 - Parque das Laranjeiras, Maringá - PR, 87083-240 · CNES 2216108
Atenção Especializada
Hospital de Clínicas da UFPR
R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980
Serviço de Referência
Hospital Universitário Pedro Ernesto (HUPE-UERJ)
Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221
Serviço de Referência
Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)
Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988
Serviço de Referência
Hospital São Lucas da PUCRS
Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928
Serviço de Referência
Hospital de Clínicas de Porto Alegre (HCPA)
Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601
Serviço de Referência
Hospital Universitário da UFSC (HU-UFSC)
R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356
Serviço de Referência
Hospital das Clínicas da FMUSP
R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485
Serviço de Referência
Hospital de Base de São José do Rio Preto
Av. Brg. Faria Lima, 5544 - Vila Sao Jose, São José do Rio Preto - SP, 15090-000 · CNES 2079798
Atenção Especializada
Hospital de Clínicas da UNICAMP
R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223
Serviço de Referência
Hospital de Clínicas de Ribeirão Preto (HCRP-USP)
R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187
Serviço de Referência
UNIFESP / Hospital São Paulo
R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689
Serviço de Referência
Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Publicações mais relevantes
Successful Surgery for Type III Colonic Atresia Using the Side-to-Side Santulli Procedure: Rescue Treatment During the Practice of Global Pediatric Surgery.
Colonic atresia (CA) is a rare congenital intestinal condition that requires surgical intervention. The surgical approaches for its treatment may vary from primary anastomosis to stoma creation. We report a case of CA treated with side-to-side anastomosis and Santulli-type colostomy. A three-year-old girl with a history of CA who had undergone ascending colostomy in the neonatal period visited the surgical mission of the World Surgical Foundation for definitive surgical management. The details of the operative findings in the neonatal period are unknown. Preoperative contrast enema revealed an unused rectosigmoid segment, suggesting that primary end-to-end anastomosis would be challenging. Exploratory laparotomy revealed type III CA in the ascending colon. The distal colon, from the transverse to the descending colon, was absent. Due to the 5-fold caliber difference between the ascending and sigmoid colon, a side-to-side Santulli procedure with an end colostomy was performed to restore colonic continuity. The procedure effectively restored intestinal continuity and addressed a significant caliber discrepancy. Postoperative recovery was uneventful, with consistent colostomy output and normal rectal stool defecation. Two months postoperatively, the patient remained in a good general condition and had a favorable bowel function. In conclusion, the side-to-side Santulli procedure offers a safe approach for CA, providing an additional safeguard as a pressure-release valve for cases with significant caliber differences between the proximal and distal colon, ensuring smooth stool passage.
Intestinal Atresia in Finland: Maternal Risk Factors, Prevalence, Associated Anomalies and Survival.
We aimed to investigate prevalence, associated anomalies and survival of congenital intestinal atresia and to examine maternal risk factors for jejunoileal atresia (JIA). All children born with, or pregnancies terminated because of, JIA or colonic atresia (CA) in Finland during 1987-2019 were identified from the Finnish Register of Congenital Malformations. Clinical information was obtained from national health registers. Maternal risk factors were assessed using all JIA cases from 2004 to 2017 (n = 101). For each case, five appropriately matched live-born controls were selected. We identified 175 JIA and 48 CA cases. About half were isolated anomalies. Gastrointestinal anomalies were the most common associated defects (26% in JIA, 35% in CA), followed by cardiac anomalies in JIA (13%) and urinary tract anomalies in CA (19%). Survival was 88% in JIA and 94% in CA. Only two of 224 patients died directly due to intestinal atresia. Maternal insulin use (adjusted odds ratio [aOR] 8.4, 95% CI 1.4-51.0) and propionic acid derivatives (aOR 4.6, 95% CI 1.5-14.8) were associated with increased JIA risk. Although associated anomalies were frequent, mortality in intestinal atresia remained low. Maternal insulin and propionic acid derivative use may meaningfully contribute to JIA risk. IV.
Colonic web in infants and toddlers: a cause of chronic bowel obstruction.
Colonic web, a rare subtype of colonic atresia, typically presents in the neonatal period. However, delayed presentation, as seen in our cases, poses significant diagnostic and therapeutic challenges. We report two cases-an infant and a toddler-who presented with chronic constipation, abdominal distension and failure to thrive. Both patients were stabilised and underwent exploratory laparotomy, which confirmed the diagnosis of colonic web. A surgery involving web excision and diversion colostomy was performed successfully. Postoperative recovery was uneventful, and both patients demonstrated significant improvement during follow-up. These cases highlight the importance of maintaining a broad differential diagnosis in older infants and toddlers with obstructive bowel symptoms, the utility of imaging and intraoperative findings for diagnosis and the need for careful follow-up to rule out coexisting conditions like Hirschsprung Disease.
Balloon dilatation therapy for postoperative bowel stricture following primary anastomosis of colonic atresia: A case report.
Prevalence, risk factors and mortality associated with colonic atresia: a population-based case-control study.
This study aims to explore maternal and pregnancy-related risk factors for colonic atresia (CA) and assess the national total prevalence, mortality, and frequency of co-occurring anomalies of this rare malformation in 2004-2017. This case-control study involved 36 cases with congenital CA identified from several Finnish registers. All cases were identified based on the ICD-9/ICD-10 codes and classified based on co-occurring anomalies. Five controls without gastrointestinal congenital malformations matched for residence and time of conception (± 1 year) were randomly selected for each case. Maternal risk factors were analyzed with data from the same registers. Total prevalence of CA was 0.45/10,000, birth prevalence was 0.37/10,000 and live birth prevalence was 0.36/10,000. The overall prevalence trend did not change (p = 0.11) during the study period. There were 15 (41.7%) isolated cases, 3 (8.3%) were associated with known syndromes and 18 (50.0%) had multiple congenital anomalies. Together there were 19.4% (n = 7) terminations or neonatal mortalities. An association was observed with maternal diabetes and CA (p = 0.03). The prevalence of CA in Finland is low with no significant change over the study period. Despite the high frequency of associated anomalies, the overall survival of CA is very high, 97%.
Publicações recentes
Successful Surgery for Type III Colonic Atresia Using the Side-to-Side Santulli Procedure: Rescue Treatment During the Practice of Global Pediatric Surgery.
Intestinal Atresia in Finland: Maternal Risk Factors, Prevalence, Associated Anomalies and Survival.
Prevalence, risk factors and mortality associated with colonic atresia: a population-based case-control study.
A neonate with meconium peritonitis, first-trimester hepatitis A affected pregnancy: A case report.
Distal Ileal Atresia in a Preterm Infant with Minor Omphalocele: A Case Report.
📚 EuropePMC105 artigos no totalmostrando 62
Successful Surgery for Type III Colonic Atresia Using the Side-to-Side Santulli Procedure: Rescue Treatment During the Practice of Global Pediatric Surgery.
CureusIntestinal Atresia in Finland: Maternal Risk Factors, Prevalence, Associated Anomalies and Survival.
Acta paediatrica (Oslo, Norway : 1992)Prevalence, risk factors and mortality associated with colonic atresia: a population-based case-control study.
Pediatric surgery internationalA neonate with meconium peritonitis, first-trimester hepatitis A affected pregnancy: A case report.
SAGE open medical case reportsDistal Ileal Atresia in a Preterm Infant with Minor Omphalocele: A Case Report.
Surgical case reportsColonic web in infants and toddlers: a cause of chronic bowel obstruction.
BMJ case reportsFactors influencing early surgical outcomes of intestinal atresia in a resource-limited tertiary center.
Pediatric surgery internationalA Rare Case of Apple Peel Ileal Atresia With Coexisting Colonic Atresia: Surgical Management and Outcomes.
CureusTransverse colonic atresia with rectal atresia in a neonate - A rare case of double-site intestinal atresia.
International journal of surgery case reportsBalloon dilatation therapy for postoperative bowel stricture following primary anastomosis of colonic atresia: A case report.
Pediatrics and neonatologyProgressive colonic stenosis in an infant: Successful treatment with endoscopic balloon dilation.
JPGN reportsDecisions in Diversion: Enterostomy vs. Primary Anastomosis for Colonic Atresia.
Journal of pediatric surgeryColonic atresia and Hirschsprung's disease in a neonate: A case report.
International journal of surgery case reportsAcquired colonic atresia in children: a report of three cases and review of the literature.
Journal of surgical case reportsManagement and Outcomes of Intestinal Atresia - A Single Institution Experience From 1947 to 2019.
Journal of pediatric surgeryMinimally Invasive Placement of Pedicle Screws Using Robotic-Assisted Navigation and Magnetically Controlled Growing Rods in a Patient with Early-Onset Scoliosis: Technical Note and Case Report.
Journal of orthopaedic case reportsCLINICAL FEATURES OF THE COURSE OF HIRSCHSPRING'S DISEASE INCHILDREN OF THE FIRST YEAR.
Georgian medical newsSigmoid atresia: Case report and literature review.
International journal of surgery case reportsCongenital Segmental Dilatation of Ascending Colon with Distal Microcolon: A Diagnostic Dilemma.
Journal of Indian Association of Pediatric SurgeonsType III colonic atresia in a 6-week-old kitten.
JFMS open reportsColonic Atresia Associated with Biliary Atresia.
Journal of Indian Association of Pediatric SurgeonsImaging Features of Neonatal Bowel Obstruction.
Radiographics : a review publication of the Radiological Society of North America, IncDistal colonic atresia: a case report.
Journal of surgical case reportsColonic atresia and hirschsprung disease: a case report and review of the literature.
Journal of medical case reportsWindsock or Cobra Head Sign: A Distinctive Imaging Sign to Differentiate Type 1 Colonic Atresia From Hirschsprung's Disease.
CureusFactors associated with mortality in congenital malformations of the gastrointestinal tract in a tertiary center in Senegal.
World journal of pediatric surgeryMidgut Atresia: Diagnostic and Management Challenges From Northern Tanzania.
Clinical medicine insights. PediatricsIntestinal atresia and necrotizing enterocolitis: Embryology and anatomy.
Seminars in pediatric surgery[Delayed meconium passage due to malrotation and colon atresia].
Nederlands tijdschrift voor geneeskundeLarge Colon Volvulus in a Neonatal Foal Secondary to Atresia Coli.
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Indian journal of pediatricsGastroschisis Complicated by Colonic Atresia.
The American surgeonStaged Repair Using Modified Bishop-Koop Procedure in Complicated Congenital Colonic Atresia in a Neonate.
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International journal of surgery case reportsColonic atresia: a rare entity in the newborn. A six-case report and a bibliographic review.
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Pediatric surgery internationalCongenital multiple colonic atresias with intestinal malrotation: a case report.
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Journal of neonatal surgeryAlimentary Tract Atresias associated with Anorectal Malformations: 10 Years' Experience.
Journal of neonatal surgeryGallbladder Duplication Associated with Gastro-Intestinal Atresia.
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Case reports in pediatricsRole of intraluminal bowel echogenicity on prenatal ultrasounds to determine the anatomical level of intestinal atresia.
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Birth defects research. Part A, Clinical and molecular teratologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Successful Surgery for Type III Colonic Atresia Using the Side-to-Side Santulli Procedure: Rescue Treatment During the Practice of Global Pediatric Surgery.
- Intestinal Atresia in Finland: Maternal Risk Factors, Prevalence, Associated Anomalies and Survival.
- Colonic web in infants and toddlers: a cause of chronic bowel obstruction.
- Balloon dilatation therapy for postoperative bowel stricture following primary anastomosis of colonic atresia: A case report.
- Prevalence, risk factors and mortality associated with colonic atresia: a population-based case-control study.
- A neonate with meconium peritonitis, first-trimester hepatitis A affected pregnancy: A case report.
- Distal Ileal Atresia in a Preterm Infant with Minor Omphalocele: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:1198(Orphanet)
- OMIM OMIM:303650(OMIM)
- MONDO:0010562(MONDO)
- GARD:1446(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q55782541(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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