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Malrotation beyond infancy : an often forgotten entity
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A má rotação intestinal é uma anomalia congénita que complica frequentemente com volvo do intestino médio e consequente oclusão intestinal. Esta patologia manifesta-se, geralmente, no primeiro ano de vida com um quadro clínico característico, o que não se verifica em apresentações tardias. A má rotação intestinal, em idades não neonatais, está associada a sintomas gastrointestinais inespecíficos, tornando o diagnóstico difícil, e resultando num aumento de morbilidade e mortalidade, se a perfusão intestinal estiver comprometida aquando do diagnóstico. As publicações sobre este tema são escassas e estão maioritariamente limitadas a casos clínicos isolados, o que contribui para a falta de conhecimento e de baixa suspeição clínica, podendo contribuir para uma maior morbimortalidade. Nesta revisão, foram analisados casos clínicos publicados nos últimos 11 anos, de má rotação intestinal complicada com volvo do intestino médio, após o primeiro ano de vida. O objetivo foi explorar o perfil clínico, identificando características comuns e formas de apresentação frequentes. Analisaram-se dados sobre os seguintes parâmetros: idade, género, sintomas agudos e crónicos, exame objetivo, análises laboratoriais, exames de imagem, intervenção cirúrgica, complicações pós-operatórias e seguimento. A população estudada incluiu 37 doentes. A idade média de apresentação foi 32 anos, com idade mínima de 1 ano e 5 meses e máxima de 79. Sessenta e três porcento dos casos analisados manifestaram-se por quadro de oclusão intestinal aguda. Sessenta e dois porcento dos pacientes referiram queixas abdominais crónicas e destes, 26% reportaram a existência desses sintomas desde a infância. Os sintomas mais frequentes foram dor abdominal, náuseas, vómitos, distensão abdominal e obstipação. O exame objetivo e as análises laboratoriais revelaram alterações inespecíficas, embora pudessem levantar suspeita de isquémia intestinal / compromisso hemodinâmico. Vários exames de imagem revelaram pistas diagnósticas, mas a Tomografia Computorizada com contraste revelou ser o método mais eficaz. Trinta e cinco porcento dos pacientes tiveram complicações pós-operatórias e 29% dos casos totais necessitaram de resseção intestinal extensa. Deve suspeitar-se de má rotação intestinal em todos os pacientes com queixas abdominais inespecíficas agudas e/ou crónicas, independentemente da idade. O reconhecimento e intervenção cirúrgica precoces evitam consequências catastróficas.
Congenital midgut malrotation is a complex gastrointestinal anomaly, that can easily lead to midgut volvulus and gastrointestinal obstruction. This pathology usually becomes symptomatic within the first year of life, with a well-described clinical presentation. However, that is not the case beyond infancy. Malrotation, in older ages, is associated with a variety of non-specific gastrointestinal symptoms, and the diagnosis is often missed for years. This leads to increased morbidity and mortality, sometimes with devastating outcomes when intestinal perfusion is severely compromised at diagnosis. Publications about this matter are scarce and mainly limited to isolated clinical case reports, which contributes to a lack of knowledge and low clinical suspicion, leading to higher morbimortality. In this review, case reports of intestinal malrotation and midgut volvulus beyond the first year of life, published in the last 11 years, were included and retrospectively analyzed. The aim was to explore the clinical case and identify common characteristics and forms of presentation. Data on age at presentation, gender, acute/chronic symptoms, physical examination, laboratory results, radiographic imaging performed, surgical intervention, complications, and postoperative follow-up were collected and evaluated. The study population included 37 patients. The mean age was 32 years ranging from 1 year and 5 months to 79 years old. Sixty-three percent of the analyzed cases, presented with acute bowel obstruction. Sixty-two percent of patients had chronic abdominal complaints, and 26% of these reported painful symptoms since childhood. The most common symptoms were abdominal pain, nausea, vomiting, abdominal distension, and constipation. Physical examination and laboratory test findings were quite nonspecific, although could raise suspicion of “intestinal distress/ hemodynamic compromise”. Diagnosis may be suggested by different imaging techniques, but Contrast-enhanced Computer Tomography (CE-CT) proved to be the most reliable one. Thirty-five percent of the patients had postoperative complications and 29% of the total cases required extensive intestinal resection. Malrotation should be suspected in all patients with varied acute or chronic abdominal symptoms, regardless of their age. Early recognition and surgical management, avoid catastrophic outcomes.
Congenital midgut malrotation is a complex gastrointestinal anomaly, that can easily lead to midgut volvulus and gastrointestinal obstruction. This pathology usually becomes symptomatic within the first year of life, with a well-described clinical presentation. However, that is not the case beyond infancy. Malrotation, in older ages, is associated with a variety of non-specific gastrointestinal symptoms, and the diagnosis is often missed for years. This leads to increased morbidity and mortality, sometimes with devastating outcomes when intestinal perfusion is severely compromised at diagnosis. Publications about this matter are scarce and mainly limited to isolated clinical case reports, which contributes to a lack of knowledge and low clinical suspicion, leading to higher morbimortality. In this review, case reports of intestinal malrotation and midgut volvulus beyond the first year of life, published in the last 11 years, were included and retrospectively analyzed. The aim was to explore the clinical case and identify common characteristics and forms of presentation. Data on age at presentation, gender, acute/chronic symptoms, physical examination, laboratory results, radiographic imaging performed, surgical intervention, complications, and postoperative follow-up were collected and evaluated. The study population included 37 patients. The mean age was 32 years ranging from 1 year and 5 months to 79 years old. Sixty-three percent of the analyzed cases, presented with acute bowel obstruction. Sixty-two percent of patients had chronic abdominal complaints, and 26% of these reported painful symptoms since childhood. The most common symptoms were abdominal pain, nausea, vomiting, abdominal distension, and constipation. Physical examination and laboratory test findings were quite nonspecific, although could raise suspicion of “intestinal distress/ hemodynamic compromise”. Diagnosis may be suggested by different imaging techniques, but Contrast-enhanced Computer Tomography (CE-CT) proved to be the most reliable one. Thirty-five percent of the patients had postoperative complications and 29% of the total cases required extensive intestinal resection. Malrotation should be suspected in all patients with varied acute or chronic abdominal symptoms, regardless of their age. Early recognition and surgical management, avoid catastrophic outcomes.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2023
Palavras-chave
Má rotação intestinal Volvo do intestino médio Procedimento de Ladd Dor abdominal recorrente Casos clínicos