Síndrome de la arteria mesentérica superior o Síndrome de Wilkie. Presentación de tres casos clínicos y revisión de la literatura

Objetivo: Presentar una causa rara de obstrucción duodenal como es el sindrome de arteria mesentérica superior (SAMS). Material y Método: se presentan tres casos clínicos de sindrome de SAMS. Resultados: el tratamiento médico y nutricional fue exitoso en los primeros dos casos, mientras que en el último fue necesario realizar una derivación quirúrgica. Discusión: en este sindrome, el ángulo aortomesentérico normal se encuentra disminuído, causando la compresión de la tercera porción del duodeno entre la aorta y la arteria mesentérica superior. Esto determina una obstrucción duodenal que se ve más frecuentemente en pacientes con pérdida importante y rápida de peso. Esto determina alteraciones hidroelectrolíticas y desnutrición severas. El tratamiento médico consiste en soporte nutricional y corrección de anormalidades hidroelectrolíticas. Los procedimientos quirúrgicos se reservan frente a los casos de falla del tratamiento médico. Conclusión: el SAMS es una entidad poco frecuente y un reto diagnóstico. El cuadro clínico puede ser grave con desnutrición severa y trastornos hidroelectrolíticos que ponen en riesgo la vida del paciente. El soporte hidroelectrolítico y nutricional, junto a la duodenoyeyunostomía, son los tratamientos de elección.

Aim: To present a rare cause of duodenal obstruction known as the superior mesenteric artery syndrome (SMAS). Material and Method: we present three cases of SMAS. Results: medical and nutritional treatment were effective in the first two cases while in the last case, a derivative procedure was necessary. Discussion: in this syndrome, the normal aortomesenteric angle is narrowed, causing compression of the third portion of the duodenum between the aorta and the superior mesenteric artery. This leads to duodenal obstruction more frequently observed in patients with severe and fast weight loss. Medical treatment consists in nutritional support and correction of hydro electrolyte imbalance. Surgical procedures are reserved for cases with failure of medical treatment. Conclusion: SMAS is a rare entity and a diagnosis challenge. Clinical setting may be severe with extreme malnutrition and life-threatening dehydration and electrolyte disorder. Nutritional support, hydro electrolyte correction, combined with duodenojejunostomy, if necessary, are the treatments of choice.

Causa rara de dolor abdominal recurrente; coexistencia del síndrome de Wilkie y el síndrome del cascanueces / A rare cause of recurrent abdominal pain; the coexistence of Wilkie's syndrome and nutcracker syndrome

El síndrome del cascanueces es un síndrome que presenta síntomas clínicos como hematuria, proteinuria ortostática, congestión pélvica, varicocele del lado izquierdo, hipertensión y dolor en fosa renal. Estos síntomas se producen por la compresión de la vena renal izquierda entre la aorta y la arteria mesentérica superior. En el síndrome de Wilkie, la tercera porción del duodeno está comprimida entre la arteria mesentérica superior y la aorta abdominal, lo que provoca diversos síntomas gastrointestinales. La coexistencia de estos dos síndromes constituye una afección rara y se incluye como casos clínicos en la bibliografía. En este artículo, se presentan los resultados clínicos y radiológicos de un paciente de 17 años que presentaba dolor abdominal recurrente debido al síndrome de Wilkie, acompañado del síndrome del cascanueces que le provocaba proteinuria, por lo que el paciente fue derivado a los consultorios externos de reumatología pediátrica con un diagnóstico preliminar de fiebre mediterránea familiar.

Nutcracker syndrome is a syndrome that has clinical symptoms such as hematuria, orthostatic proteinuria, pelvic congestion, left-sided varicocele, hypertension, and flank pain. These symptoms occur because of the compression of the left renal vein between the aorta and the superior mesenteric artery. In Wilkie's syndrome, the third part of the duodenum is compressed between the superior mesenteric artery and the abdominal aorta, causing various gastrointestinal symptoms. The coexistence of these two syndromes is a rare condition and is included as case reports in the literature. This article presents the clinical and radiological results of a 17-year-old male patient who had recurrent abdominal pain due to Wilkie's syndrome, which was accompanied by nutcracker syndrome that caused proteinuria, and for this reason, the patient was referred to the Pediatric Rheumatology outpatient clinic with a preliminary diagnosis of familial Mediterranean fever.

Wilkie's and Nutcracker's syndromes overlapping a case of functional dyspepsia

We present the case of a patient suffering from a mixed-type functional dyspepsia who markedly reduced his diet to improve his symptoms leading him to malnourishment and a subsequent Wilkie's and Nutcracker's syndromes which exacerbated his pain. Our aim by presenting this case is to raise awareness as to what extent a so-called functional dyspepsia can evolve and of the possible overlap with these two entities in case of severe malnutrition.

Presentamos el caso de un paciente diagnosticado de dispepsia funcional de tipo mixto, el cual redujo marcadamente su dieta para mejorar sus síntomas, llevándolo a una desnutrición y a un posterior síndrome de Wilkie y del Cascanueces que agudizó su dolor. Nuestro objetivo con la presentación de este caso es concienciar de hasta qué punto puede evolucionar una denominada dispepsia funcional y del posible solapamiento de estas dos entidades en caso de desnutrición severa.

Combined superior mesenteric artery syndrome and nutcraker syndrome presenting as acute pancreatitis: a case report / Síndrome da artéria mesentérica superior em combinação com síndrome de quebra-nozes manifestando-se como pancreatite aguda: relato de caso

Abstract Superior mesenteric artery syndrome designates compression of the third part of the duodenum between the superior mesenteric artery and the aorta. This condition has a low incidence, being more common in thin young women. Nutcracker syndrome is compression of the left renal vein between the superior mesenteric artery and the aorta. Both entities are rare, and their coexistence has been reported in a few cases. Conservative treatment targeting weight gain is sufficient in most cases. An association between the superior mesenteric artery syndrome and acute pancreatitis has rarely been reported. We intend to describe the case of an 18-year-old girl who was admitted to the emergency room with epigastric pain and emesis. Our investigation revealed acute acalculous pancreatitis. During work-up, we discovered superior mesenteric artery syndrome and a compressed left renal vein. The patient is on conservative treatment, and her symptoms have improved.

Resumo A síndrome da artéria mesentérica superior designa compressão da terceira parte do duodeno pela artéria mesentérica superior e a aorta. Essa condição tem uma baixa incidência, sendo mais comum em mulheres jovens magras. A síndrome de quebra-nozes resulta da compressão da veia renal esquerda pela artéria mesentérica superior e a aorta. Ambas as entidades são raras, e a sua coexistência foi descrita em poucos casos. Tratamento conservador com o objetivo de ganho ponderal é suficiente na maioria dos casos. A associação entre a síndrome da artéria mesentérica superior e a pancreatite aguda foi raramente relatada. Pretendemos descrever o caso de uma jovem de 18 anos que recorreu ao serviço de urgência com epigastralgia e vômitos. A investigação realizada revelou pancreatite aguda alitiásica. Durante o estudo complementar, foi identificada a síndrome da artéria mesentérica superior, bem como uma veia renal esquerda comprimida. A paciente encontra-se em tratamento conservador, apresentando melhora clínica.

Síndrome de cascanueces acompañando un caso de síndrome de Wilkie: a propósito de un caso / Nutcracker Syndrome Combined with Wilkie Syndrome: Case Report

Resumen Objetivo: describir la presentación clínica, el diagnóstico y el tratamiento quirúrgico de un paciente con síndrome de la arteria mesentérica superior o síndrome de Wilkie combinado con síndrome de cascanueces o síndrome de compresión de la vena renal izquierda, en un centro de alta complejidad de un país latinoamericano. Descripción del caso: paciente masculino de 25 años, procedente de Estados Unidos, quien consultó por un año de pérdida de peso y episodios de obstrucción intestinal de etiología desconocida tras múltiples estudios de imagen. Se le realizaron estudios endoscópicos sin hallazgos; en la sala de recuperación desarrolló dolor abdominal que requirió ingreso al servicio de urgencias. La enterotomografía mostró dilatación de asas de colon e intestino delgado, con disminución del ángulo aortomesentérico y la serie gastrointestinal con paso filiforme del medio de contraste. Se intentó el manejo conservador como terapia inicial, con intolerancia al soporte nutricional entérico. Finalmente, se optó por el tratamiento quirúrgico, con un proceso de recuperación tórpido inicialmente, pero al final con resolución de los síntomas y aumento de peso. Conclusión: el síndrome de Wilkie es una enfermedad rara y un desafío diagnóstico en pacientes con pérdida de peso y dolor abdominal. Describimos un caso de compresión de la arteria mesentérica superior en el que se logró el diagnóstico con múltiples estrategias diagnósticas y resolución completa luego del tratamiento quirúrgico. La disminución del ángulo aortomesentérico puede comprimir la arteria mesentérica superior, así como la vena renal izquierda, que en ese caso resultó en un síndrome combinado de Wilkie y de cascanueces.

Abstract Objective: to describe the clinical presentation, diagnosis, and surgical treatment of a patient with superior mesenteric artery syndrome or Wilkie syndrome combined with the nutcracker syndrome or left renal vein compression syndrome in a tertiary referral center in a Latin American country. Case description: a 25-year-old male patient from the United States who attended for a year of weight loss and intestinal obstruction episodes of unknown etiology after multiple imaging studies. Endoscopic studies were performed without findings. While in the recovery room, he developed abdominal pain requiring admission to the emergency service. The CT enterography showed dilation of the colon loops and small intestine with a decrease of the aortomesenteric (AOM) angle and the gastrointestinal series with the filiform passage of the contrast medium. Conservative management was attempted as initial therapy with intolerance to enteric nutritional support. Finally, we initially opted for surgical treatment, with a slow recovery process, but in the end, with a resolution of symptoms and weight gain. Conclusion: Wilkie syndrome is a rare disease and a diagnostic challenge in patients with weight loss and abdominal pain. We described a superior mesenteric artery compression case in which diagnosis was achieved with multiple diagnostic strategies and complete resolution after surgical treatment. The decreased aortomesenteric angle may compress the superior mesenteric artery and the left renal vein. In this case, it resulted in a combined Wilkie and nutcracker syndrome.

A Rare Case of Wilkie’s Syndrome or Superior Mesenteric Artery Syndrome

Superior Mesenteric Artery (SMA) syndrome (known as Wilkie’s syndrome) is a rare cause of upper gastrointestinal obstruction. It is an acquired disorder, causes compression of the third part of the duodenum between the SMA and the aorta, due to loss of fatty tissue as a result of a variety of debilitating conditions. We report a case of 18 year old female presented with history of intermittent abdominal pain, vomiting of 6 months duration followed by significant weight loss for which she is hospitalized. Patient underwent extensive invasive and non-invasive tests, thereafter revealed her diagnosis. This case emphasizes the challenges in the diagnosis of SMA syndrome and the need for increased awareness of this entity.

Therapeutic laparoscopy for superior mesenteric artery syndrome in 5 children: treatment experience and lite-rature review / 中华实用儿科临床杂志

Objective:To investigate the clinical characteristics and therapeutic laparoscopy treatment for superior mesenteric artery syndrome(SMAS) in children.Methods:A retrospective study on 5 children with SMAS who failed to respond to the conservative treatment in the Second Hospital of Shandong University from March 2017 to March 2022 was carried out.All the patients underwent the laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure.The clinical data were analyzed and literature retrieved from Wanfang and PubMed databases were reviewed.Results:There were 3 boys and 2 girls in the 5 cases with SMAS.The average age was (12.4±1.4) years, and the preoperative body mass index was (15.2±0.8) kg/m 2.The patients were admitted to the hospital with vomiting, abdominal pain and emaciation symptoms, and the duration of symptoms was (8.4±7.8) months.Gastroscopy, laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure were performed successfully in all the patients, without conversions to open surgery.The average operation duration was (66.0±10.7) min.Food intake was allowed 1 day after surgery.Chylous fistula occurred 4 days after operation in one patient, whose symptom relieved after 11 days of conservative treatment.One patient still had nausea and vomiting symptoms 10 days after operation and was healed by knee-chest posture treatment.No other short-term complications were observed.The median postoperative duration of stay in the hospital was 7 (6-22) days.The patients were followed up for 9-56 (median: 21) months.All of them recovered well and gained weight.One patient had mild recurrent symptoms, and was cured after conservative treatment.A total of 15 literature on the treatment of SMAS by laparoscopic lysis of the ligament of Treitz was retrieved, and the cure rate was 40%-100%.Only one group of these cases had a cure rate below 75%. Conclusions:The laparoscopic lysis of the ligament of Treitz and duodenal lowering procedure is safe, effective, simple and minimally invasive treatment for children with SMAS.It can be used as the first choice for most pediatric patients.

SMA Syndrome – Wait & Nurture

@#Superior mesenteric artery (SMA) syndrome is rare with the common presentation of megaduodenum from entrapment of the third part of the duodenum (D3) by the SMA. We present a case report of a thin, fit 16-year-old boy, active smoker complaining of generalized colicky abdominal pain associated with persistent postprandial vomiting. Small bowel study demonstrated partial duodenal obstruction from compression of the third part of the duodenum by the SMA. Computed tomography of the abdomen shows an aorto-mesenteric angle of 13 degrees with aorto-mesenteric distance of 0.32 cm, indicating SMA syndrome. His symptoms resolved spontaneously with watchful waiting and nutritional care plan. The success was contributed to restoring the fat cushion around the SMA, hence, widening the aorto-mesenteric angle. In conclusion, watchful waiting with a nutritional care plan is a feasible initial strategy in the approach to SMA syndrome. However, should this strategy be unsuccessful, the choice of surgical treatment is duodenojejunostomy.

Síndrome de Wilkie: caso clínico / Wilkie syndrome: case report

Resumen Introducción: El síndrome de Wilkie es una causa poco frecuente de obstrucción del tracto digestivo superior, se caracteriza por una disminución del ángulo aorto-mesentérico que resulta en compresión de la tercera porción del duodeno que produce síntomas obstructivos altos. Caso Clínico: Presentamos el caso de una mujer de 64 años, con historia de dolor abdominal, pirosis, saciedad precoz e intolerancia a la vía oral, en quien los estudios iniciales mostraron esofagitis, pero en quien los síntomas no mejoraron con el tratamiento clásico para enfermedad ácido péptica y en quien estudios imagenológicos adicionales sugerían el síndrome de Wilkie como causa de los síntomas. Discusión y Conclusión: Se trata de una patología que debe conocerse y considerar en paciente con historia de pérdida de peso, marcada intolerancia a la vía oral y falta de respuesta al manejo.

Introduction: Wilkie syndrome is a rare cause of upper gastrointestinal tract obstruction, it is characterized by a decrease in the aorto-mesenteric angle that results in a compression of the third portion of the duodenum causing high obstructive symptoms. Case Report: We present the case of a 64-year-old woman, with a history of abdominal pain, heartburn, early satiety, and intolerance to the oral route, in whom initial studies showed esophagitis, but in whom symptoms did not improve with the classic treatment for peptic acid disease, additional imaging studies suggested Wilkie syndrome. Discussion and Conclusión: It is a pathology that must be known and suspected in patients with a history of weight loss, marked intolerance to oral intake, and lack of treatment response.

Superior Mesenteric Artery Syndrome - An Uncommon Complication After Surgical Corrections of Spinal Deformities / Síndrome da artéria mesentérica superior - Uma complicação incomum após correções cirúrgicas de deformidades da coluna

Abstract Vascular compression of the third part of the duodenum by the superior mesenteric artery results in an unusual occlusion of the duodenal transit known as superior mesenteric artery syndrome. This syndrome can occur after surgeries to correct spinal deformities in a rate ranging from 0.5% to 4.7%. It results from a positional alteration of the artery emergency point due to a change in trunk length after surgery. It is associated with risk factors such as low body mass index and weight loss. Patients usually present with intestinal occlusion, abdominal pain, nausea, bilious vomiting, and early satiety. Superior mesenteric artery syndrome must be recognized early to institute an adequate treatment, which can be clinical (with gastric tube for decompression and nutritional support) or require a surgical procedure. Secondary complications related to superior mesenteric artery syndrome include delayed surgical and nutritional recovery, healing problems, and prolonged hospitalization. The present study aims to report a case of superior mesenteric artery syndrome in a patient with neuromuscular scoliosis secondary to a transverse myelitis who underwent surgical treatment for spinal deformity correction.

Resumo A compressão vascular da terceira parte do duodeno pela artéria mesentérica superior resulta no desenvolvimento de uma condição incomum de oclusão do trânsito duodenal conhecida como síndrome da artéria mesentérica superior. Este fenômeno pode acontecer após cirurgias de correção de deformidades da coluna, e sua taxa de ocorrência é de 0,5 a 4,7% dos casos. Isso ocorre em virtude da alteração do posicionamento do ponto de emergência da artéria, decorrente da mudança do comprimento do tronco após a cirurgia, e está associado a fatores de risco, como baixo índice de massa corpórea e perda ponderal. Os pacientes costumam se apresentar com um quadro de oclusão intestinal, com dor abdominal, náusea, vômito bilioso e saciedade precoce. O reconhecimento desta condição é importante para instituir o tratamento adequado, que varia do tratamento clínico, com sondagem e descompressão gástrica associados a suporte nutricional; à necessidade de abordagem cirúrgica. Complicações secundárias relacionadas à síndrome da artéria mesentérica superior incluem: recuperação cirúrgica e nutricional retardadas, problemas com a cicatrização e hospitalização prolongada. O objetivo do presente estudo é relatar um caso de síndrome da artéria mesentérica superior, ocorrido em um paciente com escoliose neuromuscular secundária a sequela de mielite transversa, submetido ao tratamento cirúrgico da deformidade da coluna.

Duodenoyeyunostomía laparoscópica en el Síndrome de la Arteria Mesentérica Superior / Laparoscopic duodenojejunostomy for Superior Mesenteric Artery Syndrome

Introducción: El Síndrome de la Arteria Mesentérica Superior es causa poco frecuente de obstrucción duodenal. Los síntomas varían desde una obstrucción leve hasta emergencias agudas. El examen físico no siempre ayuda al diagnóstico, que se basa en los hallazgos imagenológicos. Objetivo: preconizar, recomendar el uso de las técnicas mínimamente invasivas en el manejo quirúrgico del Síndrome de la Arteria Mesentérica Superior. Presentación del caso: Mujer de 49 años de edad, antecedentes de pérdida de peso después de quimioterapia con náuseas, vómitos y dolor abdominal posprandial de dos años de evolución. Tratada exitosamente con duodenoyeyunostomía laparoscópica. El tratamiento inicial suele ser conservador. Publicaciones recientes respaldan que la duodenoyeyunostomía laparoscópica es un método seguro y efectivo para su tratamiento. Conclusiones: El tratamiento quirúrgico es cada vez más exitoso, la mayoría de los pacientes no presenta síntomas después de la cirugía(AU)

Introduction: Superior Mesenteric Artery Syndrome is an uncommon cause of duodenal obstruction. Symptoms may vary from mild obstruction to acute life-threatening emergencies. Physical examination does not always help in establishing the diagnosis which is based on findings from imaging studies. Objective: To recommend the laparoscopic approach for the surgical treatment of this entity. Case presentation: A 49-year old female patient with prior history of weight loss after chemotherapy and a two-year history of postprandial nausea, vomiting, and abdominal pain who was successfully treated by laparoscopic duodenojejunostomy is presented. Discussion: Initial treatment is usually conservative. Recently published studies support that laparoscopic duodenojejunostomy is a safe and effective method of treatment. Conclusions: Surgical treatment is becoming more successful and the majority of patients are symptom-free after surgery(AU)

Síndrome de la arteria mesentérica superior en una adolescente con anorexia nerviosa durante la pandemia del COVID - 19. Reporte de un caso clínico / Superior mesenteric artery syndrome in an adolescent with anorexia nervosa during the COVID-19 pandemic. A clinical case report

RESUMEN Se describe el caso de una paciente adolescente con diagnóstico de Síndrome de Arteria Mesentérica Superior causado por emaciación resultante de un Trastorno de Conducta Alimentaria (Anorexia Nerviosa, AN) cuya evolución fue severamente acentuada por la pandemia de COVID-19. El Síndrome se debe a la compresión de la tercera porción del duodeno entre la arteria mesentérica superior y la aorta. Se describen las características clínicas, etiopatogénicas y diagnósticas más saltantes de la AN, cuyo diagnóstico precoz es fundamental para mejorar un pronóstico complicado por las consecuencias de baja de peso y desnutrición. La comorbilidad ansiosa y depresiva asociada al estrés causado por el confinamiento y los rasgos de personalidad obsesiva de la paciente requirieron de un tratamiento conjunto de los servicios de Pediatría y de Psiquiatría del Niño y Adolescente, este último con intervenciones psicoterapéuticas individual y familiar.

SUMMARY The case of an adolescent patient diagnosed with Superior Mesenteric Artery Syndrome caused by emaciation resulting from an eating disorder (Anorexia Nervosa, AN) es described. Its clinical course was severely accentuated by the COVID-19 pandemic. The Syndrome is due to the compression of the third portion of the duodenum between the superior mesenteric artery and the aorta. The main clinical, etiopathogenic and diagnostic characteristics of AN are described; its early diagnosis is essential to improve a prognosis complicated by the consequences of weight loss and malnutrition. Anxious and depressive comorbidities associated with the stress caused by the confinement, and the patient's obsessive personality traits required a joint treatment by the Pediatry and Child and Adolescent Psychiatry Services, the latter with individual and family psychotherapy interventions.

Distensión gástrica aguda causada por síndrome de arteria mesentérica superior en una paciente bulímica

La dilatación gástrica aguda se fundamenta por dos teorías, la mecánica que es explicada por el síndrome de arteria mesentérica superior, y la atónica que es producto de una condición médica en la que el estómago progresivamente se vuelve hipotónico y se sobredistiende aún en ausencia de una causa mecánica obstructiva; con mayor frecuencia la dilatación gástrica aguda se presenta en pacientes con trastornos de la alimentación y puede conllevar a una complicación rara pero letal como la perforación gástrica. Presentamos el caso de una paciente de 18 años, quien acude al servicio de emergencia con dolor abdominal agudo, es intervenida quirúrgicamente, donde se encontró perforación de la pared gástrica anterior. Se le realizó una gastrectomía parcial proximal, y se envió el espécimen para estudio patológico. La histopatología mostró necrosis transmural predominantemente en fondo gástrico.

Acute gastric dilation is based on two theories, the mechanical one that is explained by the superior mesenteric artery syndrome and the atonic one, the latter is the product of a medical condition in which the stomach progressively becomes hypotonic and over-distended even in the absence of an obstructive mechanical cause; it most often occurs in patients with eating disorders; which can lead to a rare but fatal complication such as gastric perforation. We present the case of an 18 year old patient who comes to the emergency service of our hospital with acute abdominal pain, undergoes surgery, where there is perforation of the anterior gastric wall, and a proximal. Partial gastrectomy is performed, and a sends the specimen for pathology study. Histopathology shows transmural necrosis predominantly in the gastric fundus.

Síndrome de la arteria mesentérica superior: abordaje quirúrgico. Reporte de caso / Superior mesenteric artery syndrome: surgical approach, case report

El síndrome de arteria mesentérica superior (SAMS), causa infrecuente de obstrucción intestinal proximal; ocurre por compresión externa de la tercera porción duodenal por la arteria mesentérica superior debido a la formación de un ángulo inadecuado de la unión aortomesentérica. Incidencia de 0.013% - 0.3. Se presenta caso de paciente masculino de 27 años con diagnóstico de SAMS, historia de pérdida de peso, dolor postprandial recurrente, náuseas, vómitos de contenido biliar de un año de evolución. Paciente se ingresa al servicio de emergencia de Cirugía de Adultos del Hospital Roosevelt, Guatemala, donde se programa y realiza abordaje con técnica de Strong, además de la realización de duodeno-yeyuno anastomosis. Evoluciona satisfactoriamente por lo que se concluye que la técnica de Strong aunada a la realización de duodeno-yeyuno anastomosis son herramientas más efectivas como abordaje quirúrgico del síndrome de arteria mesentérica superior. (AU)

The superior mesenteric artery syndrome (SAMS), a rare cause of proximal intestinal obstruction; It occurs by external compression of the third duodenal portion by the superior mesenteric artery due to the formation of an inappropriate angle of the aortomesenteric junction. Incidence of 0.013% - 0.3. A case of a 27-year-old male patient is presented with a diagnosis of SAMS, history of weight loss, recurrent postprandial pain, nausea, vomiting of bile content of one year of evolution. Patient is admitted to the emergency service of Adult Surgery of the Roosevelt Hospital, Guatemala, where an approach with Strong's technique is programmed and performed in addition to the duodenum-jejunum anastomosis, it evolves satisfactorily, which is why it is concluded that the Strong's technique combined duodenum-jejunum anastomosis are more effective tools as a surgical approach to superior mesenteric artery syndrome. (AU)

Caracterización del síndrome de cascanueces / Characterization of Nutcracker Syndrome

Introducción: El síndrome de cascanueces es una anomalía vascular poco buscada y diagnosticada, por su baja incidencia y por la dificultad de sospecharla ante manifestaciones clínicas frecuentes. Objetivo: Examinar las características clínicas e imagenológicas de los pacientes diagnosticados con el síndrome de cascanueces. Método: Estudio descriptivo retrospectivo y prospectivo en pacientes atendidos en el Hospital Pediátrico Universitario William Soler entre enero de 2013 - diciembre de 2017. Cuando se sospechó el síndrome se realizó ultrasonido buscando la dilatación de la vena renal izquierda y el ángulo de salida de la arteria mesentérica superior. En 7 de los pacientes se realizó angio-TAC. Se revisaron variables epidemiológicas, clínicas, de laboratorio e imagenológicas. Presentación de los casos: Del total de 9 pacientes en que se diagnosticó la anomalía vascular, 6 (66,7 por ciento) pertenecen al sexo masculino. La edad media al diagnóstico fue 11,4 años (rango: 7-15). Se diagnosticó cascanueces anterior en 7 (77,7 por ciento), posterior en 1 (11,1 por ciento) y síndrome de Wilkie con fenómeno cascanueces asociado en 1 (11,1 por ciento). En el 100 por ciento de los casos hubo dolor abdominal o lumbar, asociado a hematuria en 4, orinas espumosas en 4 y varicocele izquierdo en 2 de los 6 varones (33,3 por ciento). Conclusiones: El síndrome de cascanueces debe buscarse en los pacientes que presentan dolor abdominal, lumbar o en flanco izquierdo en forma recurrente, episodios de hematuria recurrente o proteinuria ortostática. En el varón con varicocele izquierdo debe descartarse esta posibilidad. El fenómeno cascanueces, puede diagnosticarse cuando se investiga al paciente por causa no relacionada(AU)

Introduction: Nutcracker syndrome (NCS) is a vascular anomaly which is rarely searched and diagnosed due to its low incidence and because of the difficulty to suspect on it among other frequent clinical manifestations. Objective: To examine clinical and imaging characteristics of patients diagnosed with NCS. Methods: Descriptive, retrospective and prospective study in patients admitted in William Soler University Pediatric Hospital in the period from January, 2013 to December, 2017. When it was suspected the presence of the syndrome, it was made an ultrasound looking for dilation of the left renal vein and the output angle of the superior mesenteric artery. In 7 patients it was made an angio-TAC. The epidemiologic, clinical, laboratory and imaging variables were reviewed. Presentation of cases: From the total of 9 patients diagnosed with a vascular anomaly; 6 (66,7 percent) were males. The mean age was 11,4 years (range 7-15 years). It was diagnosed front NCS in 7 patients (77.7 percent), back NCS in 1 patient (11.1 percent) and Wilkie syndrome with associated NCS in 1 patient (11.1 percent). 100 percent of the cases presented abdominal or lumbar pain related to hematuria (4 patients), foaming urine (4 patients) and left varicocele in 2 of the male patients (33.3 percent). Conclusions: Nutcracker syndrome should be searched in patients presenting frequent abdominal, lumbar or left side pain, recurrent hematuria or orthostatic proteinuria episodes. In males with left varicocele should be discounted this possibility. Nutcracker phenomenon can be also diagnosed when the patient is being investigated due to not related causes(AU)

Wilkie's syndrome in an adolescent female patient

One of the rare cases of the upper gastrointestinal tract obstruction is superior mesenteric artery syndrome (SMAS) but potentially life-threatening if not recognized early. It is due to loss of fat pad between the aorta and SMA which will lead to a compression of the third portion of the duodenum. It has a different angle which will lead to different presentations as well as severity. We are reporting the 15 year-old (y/o) female who is medically free, presented to the emergency department (ED) complaining of severe colicky epigastric abdominal pain for the last 5 days in the epigastric area. The diagnosis of SMAS was made after clinical and radiological investigation. After proper supportive resuscitative measures, definitive management of the surgery was done by laparoscopic approach (duodenojejunostomy). The diagnosis of SMA syndrome is considered challenging due to many presentations and might be confused with other clinical conditions. Unless early diagnosed and treated, the outcome might be catastrophic. Medical treatment is attempted first in many cases depending on the severity and presentation but if failed, surgery will be the best option.

Superior mesenteric artery syndrome masquerading as chronic gastric volvulus in a child with severe acute malnutrition: a case report

Superior mesenteric artery (SMA) syndrome is a rare cause of high intestinal obstruction. It is commonly seen in severely malnourished patients presenting with repeated episodes of vomiting. We report a case of SMA syndrome in a 3 years old male child provisionally diagnosed as chronic gastric volvulus with severe acute malnutrition. The diagnosis of SMA syndrome was confirmed on abdominal exploration. SMA syndrome is a rare case of intestinal obstruction in paediatric age group. Chronic malnutrition is one of the common causes of this entity. There should be a high index of suspicion for this syndrome when we encounter a child with severe malnutrition and recurrent vomiting.

Síndrome de Wilkie secundario a hipertiroidismo exógeno en una paciente con hipotiroidismo primario autoinmune: reporte de un caso y revisión de la literatura / Wilkie's syndrome caused by exogenous hyperthyroidism in a patient with primary autoimmune hypothyroidism: a case report and literature review

RESUMEN El síndrome de Wilkie o de arteria mesentérica superior es una causa poco común de obstrucción intestinal proximal, relacionada a pérdida de peso reciente. Reportamos el caso de una mujer de 19 años que se presenta a la clínica con pérdida de peso, dolor abdominal, nausea y vomito. Los exámenes de laboratorio reportaron anemia, hipoalbuminemia, hipomagnesemia y una hormona estimulante de la tiroides suprimida secundario al uso con levotiroxina. Se realizó una serie esofagogastroduodenal con datos compatibles con dilatación gástrica severa, gastroparesia y una tomografía axial computada reveló un ángulo aortomesentérico de 11,7°. Se inicio manejo conservador a base de nutrición enteral y parenteral total, siendo este el tratamiento de elección. En casos refractarios, la cirugía es una opción segura y efectiva.

ABSTRACT Wilkie's syndrome or superior mesenteric artery syndrome is an unusual cause of proximal intestinal obstruction, primarily attributed to recent weight loss. We report the case of a 19-year-old woman comes to our clinic and reports weight loss, abdominal pain, nausea, and vomiting. Laboratory tests revealed anemia, hypoalbuminemia, hypomagnesemia, and a suppressed thyroid stimulating hormone secondary to levothyroxine. A barium swallow test showed gastric dilatation, delayed gastric emptying and an axial computed tomography revealed an aortomesenteric angle of 11.7°. Conservative management with total parenteral and enteral nutrition was initiated, being the first-line treatment. In refractory cases surgery is a safe and effective option.

Oclusión gastroduodenal crónica por síndrome de Wilkie: reporte de un caso / Chronic gastroduodenal occlusion by Wilkie syndrome: a case report

RESUMEN El síndrome de Pinza Aortomesentérica o "Síndrome de Wilkie" es una entidad muy rara, se caracteriza por una pérdida de peso impor-tante y vómitos de alimentos parcialmente digeridos. La sospecha diagnóstica se fundamenta por la presencia de factores predisponen-tes y/o de riesgo. Estudios imagenológicos como un esofagogastroduodeno seriado y ecografía Doppler permiten su confirmación. El tratamiento primario se apoya en una adecuada nutrición. Frente al fracaso del tratamiento médico, se indica el manejo quirúrgico con diferentes técnicas para resolver la oclusión. Se presenta el caso de una adolescente con oclusión gastroduodenal crónica, fallo en el tratamiento médico y resolución quirúrgica con buenos resultados.Palabras claves: Síndrome de la arteria mesentérica superior, obstrucción duodenal, reflujo duodenogástrico, laparotomía, anastomosis quirúrgica; gastroparesia.

ABSTRACT Superior mesenteric artery syndrome or "Wilkie syndrome" is a very rare entity, characterized by significant weight loss and vomiting of partially digested food. The diagnostic suspicion is based on the presence of predisposing and/or risk factors. Imaging studies such as serial esophagogastroduodenum and Doppler ultrasound allow its confirmation. The primary treatment is supported by adequate nutrition. Following the failure of medical treatment, surgical management is indicated with different techniques to resolve the occlusion. We present the case of a teenager with chronic gastroduodenal occlusion, failure in medical treatment and surgical resolution with good results.Keywords: Superior mesenteric artery syndrome; duodenal obstruction; duodenogastric reflux; laparotomy; anastomosis, surgical; gas-troparesis

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)