[Wilkie syndrome as a diagnostic challenge in intestinal obstruction: case report]. / Síndrome de Wilkie como reto diagnóstico en obstrucción intestinal: reporte de caso.

Wilkie syndrome is a rare pathology that generates intestinal obstruction due to a decrease of the aortomesenteric angle compromising the third portion of the duodenum. We describe a case of an 18-year-old female patient, diagnosed with Wilkie syndrome, with clinical symptoms of intestinal obstruction and weight loss. The diagnosis was made with abdominal CT. Wilkie syndrome is a rare pathology, which becomes a diagnostic challenge because it presents a similar picture to other more common pathologies. We recommend that it should be suspected in the presence of duodenal obstruction.

Tratamento cirúrgico da síndrome da artéria mesentérica superior em paciente idosa / Surgical treatment of mesenteric superior artery syndrome in an older woman

A síndrome da artéria mesentérica superior (SAM), ou síndrome de Wilkie, é uma afecção rara que acomete geralmente indivíduos jovens do sexo feminino. Caracterizada pela obstrução parcial ou completa do duodeno devido à compressão pela artéria mesentérica superior anteriormente, e pela aorta, posteriormente. Apresentamos um caso não usual de SAM, em paciente idosa, diagnosticado através de história clínica e imagem tomográfica característica. Indicou-se manejo cirúrgico de derivação jejunal e anastomose com duodeno acima da obstrução, na ciência do risco do procedimento. A paciente apresentou excelente evolução e encontra-se assintomática. O diagnóstico precoce, através de uma anamnese adequada e uso dos critérios radiológicos já definidos em literatura, podem afetar positivamente o desfecho dos pacientes portadores em qualquer faixa etária. (AU)

Superior mesenteric artery syndrome (SMAS), or Wilkie syndrome, is a rare condition that usually affects young women. It is characterized by partial or complete obstruction of the duodenum due to compression by the superior mesenteric artery anteriorly and by the aorta posteriorly. We report an unusual case of SMAS in an older woman diagnosed using clinical history and tomographic imaging. Surgical management consisting of jejunal shunt and anastomosis with duodenum above the obstruction was indicated despite the risk of the procedure. The outcome was excellent, and the patient is asymptomatic. Early diagnosis using adequate anamnesis and radiological criteria defined in the literature may positively influence the outcome of patients from any age group. (AU)

[Wilkie Syndrome. A case report]. / Síndrome de Wilkie. Reporte de un caso.

BACKGROUND: Wilkie syndrome, also referred as superior mesenteric artery syndrome, is an unusual cause of a proximal small bowel obstruction. It is characterised by the compression of the duodenum in its third portion due to a narrowing of the space between the superior mesenteric artery and the aorta. Its presentation symptoms are consistent and include the obstruction of the proximal small bowel. However, the physical and laboratory findings are non-specific. Nevertheless, many imaging methods are useful for its diagnosis. The management of this condition varies between observation and surgery, depending on each particular case. CLINICAL CASE: The case is presented of a 19 year-old male who began with acute, intense abdominal pain, nausea, vomiting, and diarrhoea. On examination, he had abdominal wall rigidity and hyperesthesia. Imaging studies were requested, revealing a decreased superior mesenteric artery angle, a shortening of the aortic mesenteric distance, and a decrease in the calibre of the third duodenal portion, all findings concomitant with Wilkie syndrome. Conservative treatment was applied and the patient was discharged without complications. CONCLUSIONS: Wilkie syndrome continues to be an unknown condition to the general practitioner, and the underdiagnosis of this condition may put a patient at risk of serious complications. A high index of suspicion is required to reach a diagnosis. Early treatment should give a good outcome most of the time.

[Superior mesenteric artery syndrome. A case report and review of the literature]. / Síndrome de la arteria mesentérica superior. presentación de un caso y revisión de la literatura.

INTRODUCTION: Superior mesenteric artery syndrome (SMAS) or Wilkie syndrome is a rare disease in the pediatric population. Although their causes are generally well known, the clinic is often subtle, so reaching a diagnosis can be difficult. There are medical and surgical treatment options, which depend on each patient. CLINICAL CASE: Our study shows an atypical case of SAMS, which debuted in a 13-year-old boy without previous diseases, and without a history of risk factors for this pathology. Its symptomatology, the diagnostic process and the treatment are described. COMMENTS: The clinical presentation and age of the patient make an interesting case, so we make a review about the pathology, its current diagnostic methods and available therapeutic alternatives.

INTRODUCCION: El síndrome de la arteria mesentérica superior (SAMS) o síndrome de Wilkie es una patología poco frecuente en la población pediátrica. A pesar de que sus causas son en general bien conocidas, la clínica muchas veces es poco sugerente, por lo que llegar al diagnóstico puede ser difícil. Existen opciones médicas y quirúrgicas de tratamiento, que dependen de cada paciente. CASO CLINICO: Nuestro trabajo muestra un caso atípico de SAMS, que debutó en un adolescente de 13 años sin enfermedades previas, y sin antecedentes de factores de riesgo descritos para esta patología. Se describen su sintomatología, el proceso diagnóstico y el tratamiento realizado. COMENTARIOS: La presentación clínica y la edad del paciente hacen a este caso particularmente interesante, por lo que hicimos una revisión teórica acerca de la patología, sus métodos diagnósticos actuales, y las alternativas terapéuticas disponibles.

Wilkie's syndrome: review of eight cases.

BACKGROUND: The superior mesenteric artery (SMA) syndrome is a rare entity presenting with upper gastrointestinal tract obstruction and abrupt weight loss. Usually seen in tall individual with low bod mass index (BMI), the weight loss may be primary or secondary to a systemic disease or to the syndrome itself Compression of the duodenum between the abdominal aorta and the superior mesenteric artery due to loss of the duodenal pad of fat causes the obstruction. Early recognition prevent complications. Imaging and endoscopy is diagnostic. Aorto-mesenteric angle and SMA aorta distance is lower than 22 degree and eight millimeter respectively. If nutritional support fails, surgery is required (e.g. guodeno-jejunostomy). Studies to determine the optimal methods of diagnosis and treatment, especially in a suburban hospitals with limited expertise are essential. CASE REPORT: We retrospectively evaluated 8 cases presented over three years. Six (75%) patients were males, age ranged from 19 to 70 years and weight from 40 to 55 kg. The mean BMI was 18.7 kg/ m2 (range 16.42 to 25.11 kg/m2). Mean weight loss before diagnosis was 9.88 kg (range 6 to 12 kg). Symptoms developed between 8 to 180 days (median 12 days). Commonest presentation was epigastric pain, vomiting and nausea. Four patients had pre-morbid conditions and the syndrome was idiopathic in the other 4. Median aorto-mesentric angle was 16.5 degree and SMA-aorta distance was 5.15 mm. Four patients required operation. The rest improved on conservative treatment. CONCLUSION: Early recognition, institution of the appropriate conservative measures and timely selection of a definite surgical method are critical to prevent severe complications and death.

Síndrome pinzamiento mesentérico: a propósito de un caso de hiperemesis / Superior mesenteric aartery syndrome: in relation to a case of hypermesis

INTRODUCCIÓN: El Síndrome de Arteria Mesentérica (SAMS) o Pinzamiento Mesentérico es un trastorno adquirido poco frecuente. Su característica principal es la obstrucción de origen vascular de la tercera porción duodenal, entre la arteria mesentérica superior y la aorta abdominal. CASO CLÍNICO: Mujer de 42 años de edad con antecedentes de trastorno depresivo, colecistectomizada en 1994 y múltiples hospitalizaciones por hiperemesis desde 1995 con estudios no concluyentes. Ingresó en diciembre de 2011 por vómitos alimentarios, con gran compromiso nutricional. Se le realizaron múltiples estudios que descartaron enfermedades reumatológicas, hepáticas e infecciosas. Entre los estudios imagenológicos, la endoscopía digestiva alta mostró esofagitis congestiva leve y gastritis crónica antral, al igual que todas las endoscopias previas. En la radiografía seriada esófago-gastroduodenal se observa severo reflujo gastroesofágico,y compresión compatible con pinzamiento. La tomografía computarizada de abdomen y pelvis mostraba el estómago de mayor tamaño que lo habitual y el duodeno presentaba nuevamente esta compresión compatible con pinzamiento. En base a estos últimos hallazgos imagenológicos, y luego de descartar variados diagnósticos diferenciales se planteó un SAMS y se decidió realizar bypass duododenoyeyunal, con buena evolución posterior. DISCUSIÓN: El diagnostico de SAMS es muchas veces de exclusión, basado en la clínica y la imagenología abdominal que descarta otros cuadros clínicos. En este caso se tiene el antecedente de múltiples hospitalizaciones sin diagnóstico concluyente. Finalmente por la larga evolución del cuadro se decide una resolución quirúrgica.

INTRODUCTION: The Superior Mesenteric Artery Syndrome (SMAS) is an uncommon acquired disorder. The main characteristic is the obstruction due to compression of the third portion of the duodenum, between the superior mesenteric arthery and the abdominal aorta. CLINICAL CASE: 42-year-old female patient with a record of depressive disorder, cholecystectomyperformed in 1994 and multiple hospitalizations due to hyperemesissince 1995, with inconclusive studies. Was admitted to the hospital on December 2011 because to alimentary vomit associated with great nutritional compromise. Multiple exams were performed to discard rheumatologic, hepatic and infectious diseases. The imaging test showed high digestive endoscopy with mild esophagitis and antral chronic gastritis, as in previous endoscopies. Stomach-esophagus-duodenum radiography with gastroesophageal reflux and extrinsic compression of the duodenum. Abdominal and pelvic computed tomography revealed a larger than usual stomach’s size and a compression in the duodenum compatible with pinching. Based on these last results and after we discard other options, we diagnosed a superior mesenteric artery syndrome and it was decided to perform a duodenojejunal bypass, with favorable evolution. DISCUSSION: The diagnosis of SMAS many times is made by exclusion based on the clinic and the abdominal images, discarding other pathologies. In this case, multiple hospitalizations with inconclusive diagnostics led to performing several tests for a variety of illnesses. Finally, based on the large evolution of the case it was decided a surgical resolution.

Wilkie’s syndrome: review of eight cases / Wilkie’s syndrome: review of eight cases.

BACKGROUND: The superior mesenteric artery (SMA) syndrome is a rare entity presenting with upper gastrointestinal tract obstruction and abrupt weight loss. Usually seen in tall individual with low bod mass index (BMI), the weight loss may be primary or secondary to a systemic disease or to the syndrome itself Compression of the duodenum between the abdominal aorta and the superior mesenteric artery due to loss of the duodenal pad of fat causes the obstruction. Early recognition prevent complications. Imaging and endoscopy is diagnostic. Aorto-mesenteric angle and SMA aorta distance is lower than 22 degree and eight millimeter respectively. If nutritional support fails, surgery is required (e.g. guodeno-jejunostomy). Studies to determine the optimal methods of diagnosis and treatment, especially in a suburban hospitals with limited expertise are essential. CASE REPORT: We retrospectively evaluated 8 cases presented over three years. Six (75

) patients were males, age ranged from 19 to 70 years and weight from 40 to 55 kg. The mean BMI was 18.7 kg/ m2 (range 16.42 to 25.11 kg/m2). Mean weight loss before diagnosis was 9.88 kg (range 6 to 12 kg). Symptoms developed between 8 to 180 days (median 12 days). Commonest presentation was epigastric pain, vomiting and nausea. Four patients had pre-morbid conditions and the syndrome was idiopathic in the other 4. Median aorto-mesentric angle was 16.5 degree and SMA-aorta distance was 5.15 mm. Four patients required operation. The rest improved on conservative treatment. CONCLUSION: Early recognition, institution of the appropriate conservative measures and timely selection of a definite surgical method are critical to prevent severe complications and death.

Wilkies syndrome: review of eight cases. / Wilkies syndrome: review of eight cases.

BACKGROUND: The superior mesenteric artery (SMA) syndrome is a rare entity presenting with upper gastrointestinal tract obstruction and abrupt weight loss. Usually seen in tall individual with low bod mass index (BMI), the weight loss may be primary or secondary to a systemic disease or to the syndrome itself Compression of the duodenum between the abdominal aorta and the superior mesenteric artery due to loss of the duodenal pad of fat causes the obstruction. Early recognition prevent complications. Imaging and endoscopy is diagnostic. Aorto-mesenteric angle and SMA aorta distance is lower than 22 degree and eight millimeter respectively. If nutritional support fails, surgery is required (e.g. guodeno-jejunostomy). Studies to determine the optimal methods of diagnosis and treatment, especially in a suburban hospitals with limited expertise are essential. CASE REPORT: We retrospectively evaluated 8 cases presented over three years. Six (75

) patients were males, age ranged from 19 to 70 years and weight from 40 to 55 kg. The mean BMI was 18.7 kg/ m2 (range 16.42 to 25.11 kg/m2). Mean weight loss before diagnosis was 9.88 kg (range 6 to 12 kg). Symptoms developed between 8 to 180 days (median 12 days). Commonest presentation was epigastric pain, vomiting and nausea. Four patients had pre-morbid conditions and the syndrome was idiopathic in the other 4. Median aorto-mesentric angle was 16.5 degree and SMA-aorta distance was 5.15 mm. Four patients required operation. The rest improved on conservative treatment. CONCLUSION: Early recognition, institution of the appropriate conservative measures and timely selection of a definite surgical method are critical to prevent severe complications and death.

Superior mesenteric artery compression syndrome - case report

Superior mesenteric artery syndrome is an entity generally caused by the loss of the intervening mesenteric fat pad, resulting in compression of the third portion of the duodenum by the superior mesenteric artery. This article reports the case of a patient with irremovable metastatic adenocarcinoma in the sigmoid colon, that evolved with intense vomiting. Intestinal transit was carried out, which showed important gastric dilation extended until the third portion of the duodenum, compatible with superior mesenteric artery syndrome. Considering the patient's nutritional condition, the medical team opted for the conservative treatment. Four months after the surgery and conservative measures, the patient did not present vomiting after eating, maintaining previous weight. Superior mesenteric artery syndrome is uncommon and can have unspecific symptoms. Thus, high suspicion is required for the appropriate clinical adjustment. A barium examination is required to make the diagnosis. The treatment can initially require gastric decompression and hydration, besides reversal of weight loss through adequate nutrition. Surgery should be adopted only in case of clinical treatment failure. (AU)

A síndrome da artéria mesentérica superior é uma entidade clínica causada geralmente pela perda do tecido adiposo mesentérico, resultando na compressão da terceira porção do duodeno pela artéria mesentérica superior. Esse artigo relata o caso clínico de uma paciente portadora de adenocarcinoma de cólon sigmoide metastático irressecável, que evoluiu com vômitos incoercíveis. Realizou-se, então, trânsito intestinal que evidenciou dilatação gástrica importante, que se prolongava até a terceira porção duodenal, quadro radiológico compatível com pinçamento da artéria mesentérica superior. Diante da condição nutricional da paciente, foi optado por iniciar medidas conservadoras (porções alimentares pequenas e mais frequentes, além de decúbito lateral esquerdo após as refeições). Quatro meses após a cirurgia e as medidas conservadoras, a paciente não apresentava mais vômitos pós-prandiais, nem emagrecimento. A síndrome da artéria mesentérica inferior é incomum e os sintomas podem ser inespecíficos. Sendo assim, um índice elevado de suspeita é exigido no ajuste clínico apropriado. O diagnóstico é feito, habitualmente, através de exame radiológico contrastado. O tratamento pode, inicialmente, exigir a descompressão gástrica e a reposição volêmica, além da reversão da perda de peso com nutrição adequada. A cirurgia deve ser reservada para os casos de falha do tratamento clínico. (AU)

Acute and chronic mesenteric ischemia: MDCT findings / Isquemia mesentérica aguda e crônica: achados tomográficos

Mesenteric ischemia is caused by a reduction in mesenteric blood flow. It can be divided into acute and chronic, based upon the rapidity and the degree to which the blood flow is compromised. The authors retrospectively reviewed 22 cases of mesenteric ischemia, diagnosed by multidetector computed tomography (MDCT) in our service, and confirmed by surgery or clinical follow-up. The frequency of the diagnostic findings of chronic and acute mesenteric ischemia was evaluated. The improvement of three-dimensional (3D) MDCT allows accurate assessment of mesenteric vessels. Therefore, it demonstrates changes in ischemic bowel segments helpful in determining the primary cause of the disease, and can identify the complications in patients with acute and chronic mesenteric ischemia.

A isquemia mesentérica é causada pela redução do fluxo sanguíneo mesentérico. Essa patologia pode ser dividida em aguda e crônica, baseada na rapidez e no grau em que o fluxo sanguíneo está sendo comprometido. Os autores retrospectivamente revisaram 22 casos de isquemia mesentérica, diagnosticados por tomografia computadorizada com mutidetectores (TCMD) em nosso serviço, e confirmados por cirurgia ou seguimento clínico. Os achados diagnósticos de isquemia aguda e crônica e sua frequência foram avaliados. Os avanços na TCMD 3D (tridimensional) permitiram o acesso detalhado aos vasos mesentéricos. Além disso, é possível demonstrar alterações nos segmentos intestinais, auxiliando na identificação da causa primária da doença e podendo identificar as complicações associadas a isquemia mesentérica e crônica.

Síndrome de Wilkie: presentación de un caso / Superior artery syndrome: Wilkie's Syndrome: case report

El síndrome de Wilkie o síndrome de la arteria mesentérica superior es una rara condición médica originada por la compresión vascular de la tercera porción duodenal entre la arteria mesentérica superior y la aorta abdominal. Los factores predisponentes incluyen desórdenes alimenticios, enfermedades catabúlicas severas, enfermedades de la columna vertebral, trauma severo y estados postoperatorios. El síntoma clinico típico es el dolor epigástrico, intermitente, acompañado de vómitos voluminosos. La duodenografía hipotónica con bario constituye el gold standard para su estudio, pero actualmente la tomografía computada con multidetectores ofrece mayores ventajas diagnósticas. El tratamiento quirúrgico incluye duodenoyeyunostomía, gastroyeryunostomía o lisis del ligamento de Treitz, que pueden realizarse por vía convencional o laparoscópica.

Wilkie's syndrome or superior mesenteric artery syndrome is a rare medical condition caused by vascular compression of the third duodenal portion between the superior mesenteric artery and abdominal aorta. The predisposing factors include eating disorders, severe catabolic diseases, and diseases of the spine, severe trauma and postopertive states. The typical symptom is intermittent epigastric pain, accompanied by vomiting. The hypotonic duodenography with barium is the gold standard for study, but now with the multidetector row CT scan offers greater diagnostic advantages. Surgical treatment includes duodenojejunostomy, gastrojejunostomy and lysis of the ligament of Treitz, which may be performed conventional or laparosocopic.

Síndrome de Wilkie: presentación de un caso / Superior artery syndrome: Wilkies Syndrome: case report

El síndrome de Wilkie o síndrome de la arteria mesentérica superior es una rara condición médica originada por la compresión vascular de la tercera porción duodenal entre la arteria mesentérica superior y la aorta abdominal. Los factores predisponentes incluyen desórdenes alimenticios, enfermedades catabúlicas severas, enfermedades de la columna vertebral, trauma severo y estados postoperatorios. El síntoma clinico típico es el dolor epigástrico, intermitente, acompañado de vómitos voluminosos. La duodenografía hipotónica con bario constituye el gold standard para su estudio, pero actualmente la tomografía computada con multidetectores ofrece mayores ventajas diagnósticas. El tratamiento quirúrgico incluye duodenoyeyunostomía, gastroyeryunostomía o lisis del ligamento de Treitz, que pueden realizarse por vía convencional o laparoscópica.(AU)

Wilkies syndrome or superior mesenteric artery syndrome is a rare medical condition caused by vascular compression of the third duodenal portion between the superior mesenteric artery and abdominal aorta. The predisposing factors include eating disorders, severe catabolic diseases, and diseases of the spine, severe trauma and postopertive states. The typical symptom is intermittent epigastric pain, accompanied by vomiting. The hypotonic duodenography with barium is the gold standard for study, but now with the multidetector row CT scan offers greater diagnostic advantages. Surgical treatment includes duodenojejunostomy, gastrojejunostomy and lysis of the ligament of Treitz, which may be performed conventional or laparosocopic.(AU)

[Superior mesenteric artery syndrome (Wilkie syndrome): case report]. / Síndrome de la arteria mesentérica superior (síndrome de Wilkie): Caso clínico.

Superior mesenteric artery syndrome is an uncommon cause of upper gastrointestinal tract obstruction. The syndrome results from compression of the third portion of duodenum as it crosses underneath the superior mesenteric artery, related to conditions that reduce the aortomesenteric angle (acute weight loss) or after scoliosis surgery. Patients may present symptoms of gastrointestinal obstruction, such as upper abdominal distension and epigastric tenderness, usually relieved by posture changing. Diagnose must be complemented with an upper gastrointestinal barium-contrast radiography. Conservative treatment is usually effective with early diagnosis. Surgery is needed when conservative measures are ineffective. We present the case of a 18 year-old patient with Wilkie's syndrome secondary to scoliosis surgery. The patient presented symptoms of gastrointestinal obstruction, and diagnosis was confirmed with upper gastrointestinal barium-contrast radiography. The patient started conservative treatment with proper positioning after eating and nutritional support to provide optimal calories supply.

Síndrome de la arteria mesentérica superior (síndrome de Wilkie): caso clínico / Superior mesenteric artery syndrome (Wilkie syndrome): case report

El síndrome de la arteria mesentérica superior (síndrome de Wilkie) es una causa poco frecuente de obstrucción duodenal de origen vascular. Se asocia con situaciones que disminuyen elángulo entre la arteria mesentérica superior y la aorta (compásaortomesentérico) secundarias a pérdida de grasa retroperitoneal (descenso brusco de peso o caquexia) o procedimientos que determinan hiperextensión dorsal, especialmente enpostquirúrgicos de cirugía correctora de escoliosis. Clínicamente, se manifiesta por obstrucción intestinal alta. El diagnósticoes radiológico y el tratamiento es inicialmente médico. El tratamiento quirúrgico se reserva para casos refractarios. Se describe el caso de un paciente de 18 años con síndrome de Wilkie, secundario a cirugía correctora de escoliosis dorsolumbar. Con la sospecha clínica se realiza seriada gastroduodenal que confirma el diagnóstico. Inicia tratamiento médico con dieta hipercalórica fraccionada y medidas posturales con buena respuesta.

Síndrome de la arteria mesentérica superior: presentación de un caso y revisión de la literatura / Superior mesenteric artery syndrome: a case report and literature review

Se realizó una revisión bibliográfica sobre el síndrome de la arteria mesentérica superior y se presenta un caso, en el que junto a un megaduodeno sintomático se hallaron anomalías de la arteria mesentérica y anomalías congénitas del ángulo duodeno yeyunal y de rotación intestinal anómala. De la revisión bibliográfica realizada se precisa lo poco frecuente del síndrome, negado por algunos autores, y se afirma que no hay tal pinzamiento sino la combinación de factores, entre los que predominan anomalías congénitas(AU)

A bibliographic review on the superior mesenteric artery syndrome was made. It was reported a case with a symptomatic megaduodenum that also presented abnormalities of the mesenteric artery and congenital anomalies of the duodenojejunal angle and of intestinal malrotation. According to the bibliographic review, it was determined that it is a rare syndrome that is denied by some authors, and that there is no such impingement, but the combination of factors, among which the congenital anomalies prevail(AU)

Superior mesenteric artery syndrome after laparoscopic sleeve gastrectomy.

Superior mesenteric artery syndrome is a duodenal obstructive complication, which has been reported after Roux-en-Y gastric bypass. We report a patient who presented a fistula at the angle of His after a laparoscopic sleeve gastrectomy, which was treated with endoscopic procedures and laparoscopic drainage. During this period, there was excessive, rapid weight loss to BMI 22 kg/m2. At 3 months after the fistula had closed, metrorrhagia was treated by blood transfusion and urgent hysterectomy. Following this, diarrhea occurred and worsened the malnutrition, and the BMI was 18. Duodenal obstruction occurred, confirmed by radiological studies and endoscopy. Duodenojejunostomy and choleystectomy were done, and there has been no recurrence of duodenal obstruction in the 14 follow-up months.