Invest Clin 61(3): 242- 264, 2020 https://doi.org/10.22209/IC.v61n3a05
Wilkie’s syndrome. Analysis of 150 cases of the Iberian and Ibero-American medical literature.
Ezequiel Trejo Scorza1, Oscar Luis Colina Cedeño1, Edgar José Brito Arreaza1 and Carlos José Trejo Scorza2
1Centro Médico de Caracas, and Escuela de Medicina “Luis Razetti”, Facultad de Medicina, Universidad Central de Venezuela, Caracas, Venezuela.
2Clínica “La Floresta”, Caracas, Venezuela.
Síndrome de Wilkie. Análisis de 150 casos de la literatura Ibérica e Ibero-americana.
Invest Clin 2020; 61 (3): 242-264
1. El 50% de los casos ocurrieron en menores de 20 años, con el mayor número de casos en escolares y adolescentes. Los síntomas más frecuentes fueron do- lor abdominal, vómitos y pérdida de peso. La enfermedad tuvo dos formas de presentación: aguda con signos de obstrucción del intestino superior (16,47%) y crónica (83,53%). Los estudios de diagnóstico radiográfico mostraron en el tracto gastrointestinal superior: estrechamiento de la porción horizontal del duodeno y retraso en el tránsito del contraste a través de la región gastroduo- denal con o sin dilatación gastroduodenal en 85% de los casos; en tomografía computarizada: disminución del ángulo y la distancia aórtica mesentérica; y en endoscopia superior: estrechamiento de la parte horizontal del duodeno, con o sin dilatación gastroduodenal en 61,90%. De los 144 pacientes que recibieron tratamiento, 94 casos recibieron tratamiento médico, con una tasa de éxito del 62,77%; y 84 casos requirieron tratamiento quirúrgico, 59,52% de estos, al ingreso. El procedimiento quirúrgico más practicado fue la duodenoyeyunos- tomía laterolateral en 70,24%. Concluimos que el síndrome de Wilkie es una entidad que resulta de la reducción del ángulo y de la distancia mesentérica aórtica, con dos formas de presentación: aguda y crónica. Se requirió trata- miento quirúrgico en el 58,33% de los casos.
Received: 31-03-2020 Accepted: 15-07-2020
The constriction of the horizontal part of the duodenum by the root of the mesentery or Wilkie’s syndrome, is also known as vascu- lar compression of the duodenum, superior mesenteric artery syndrome, aortomesenter- ic clamp syndrome, cast syndrome, arterio- mesenteric duodenal compression, arterio-
mesenteric ileus, gastro-mesenteric ileus, chronic duodenal ileus, arterio-mesenteric duodenal obstruction and mesenteric-aortic impingement. It results, in partial or total obstruction of the horizontal part of the du- odenum, and its clinical manifestations are abdominal pain, vomiting, weight loss, and sometimes, abdominal distension. It is an uncommon entity and can compromise the
patient’s life. Due to its low frequency, it is little known, and thus is not included among the first diagnostic possibilities, which delays the diagnosis and proper treatment. Most authors point out that Rokitansky, was the first to describe the compression of the hori- zontal part of the duodenum by the superior mesenteric artery, in 1861; but Bermanski et al (1), who presented a complicated case by pancreatitis and performed a very com- plete historical review, indicated that: 1.- the first description of the superior mesenteric artery syndrome was made by Boernerus in 1752; 2.- Rokitansky in 1842, described the acute dilation of the stomach resulting from compression of the duodenum by the root of the mesentery. 3.- Stavely, in 1910 was the first to practice a successful duodenojeju- nostomy in a patient with gastro-mesenteric ileus. Wilkie in 1921 (2), presented four cas- es and concluded that: 1.- the compression of the horizontal part of the duodenum by the root of the mesentery is a true clinical and pathological entity; 2.- the most proper treatment is drainage of the duodenum by duodenojejunostomy. In 1927 (3) he pre- sented a series of 75 cases, where the female sex predominated in a ratio of 2.26: 1 and the youngest patient was a 7-year-old boy. Of these, 64 cases were operated by the author, and 57 with follow-up with significant cure or improvement in about 81% of the cases. After these papers’ publication, this disease is known worldwide as Wilkie’s Syndrome.
We reviewed the papers published on children cases (4-8) and found that: 1.- The compression of the horizontal part of the duodenum by the superior mesenteric artery represents 23% of extrinsic causes and 15% of the total causes of duodenal obstruction. 2.- the surgical treatment that combines the lowest morbidity and the earliest recovery of the gastrointestinal function, consists in the duodenal derotation procedure (mobiliza- tion, and placement of the duodenum in the right paravertebral canal), proposed by Louw in 1957. This surgical technique is similar to
what is done in the Ladd procedure for intes- tinal malrotation, but the mesentery in the region of the ileocecal valve and the lateral unions of the ascending colon, should be left joined to the retroperitoneum to prevent volvulus of the middle intestine.
We reviewed the world medical litera- ture on this syndrome and were able to com- pile and analyze the papers published by Ibe- rian and Ibero-American authors, regardless of the language of publication of the papers (Spanish, Portuguese or English). We found 58 papers in Latin America with reports of 78 cases (9-66), and 44 papers with 72 cases (67-110) in the Iberian Peninsula; for a total of 102 papers with 150 cases. In all these papers, we reviewed and analyzed the epide- miology, etiopathogenesis, clinical, diagnos- tic, therapeutic procedures and the results obtained.
Through the search engines of medical papers PubMed, Lilacs, Scielo, Medes, Dial- net, Medigraphic, using the search criteria: Wilkie’s Syndrome and Superior Mesenteric Artery Syndrome, we found 106 publica- tions of Iberian and Ibero-American authors and were able to access 102 of them. We reviewed the data of all patients reported with Wilkie’s Syndrome diagnosis in the 102 reviewed publications. The data extracted and analyzed included sex, age, symptoms presented, medical and surgical history, du- ration of symptoms, means of diagnosis and findings, treatment and results. For its anal- ysis, all the information was entered into a spreadsheet of the LibreOffice program (Ver- sion: 6.1.5.2 Build ID: 1:6.1.5-3+deb10u5; OS: Linux 4.19) and divided them into three groups: a) those that responded to medical treatment, b) those who did not responded to medical treatment and needed surgical treatment, and c) those who needed surgical treatment upon admission.
Patients with anatomical causes that decreased the aortomesen- teric distance were 17 cases and their causes are shown in Table II.
Patients with personal or family his- tories were four cases: family history of Wilkie’s Syndrome (1 case); Raynaud’s syndrome history and psychological disor-
-
-
-
ders manifested by depression and anxi- ety (1 case); personal medical history of Wilkie’s Syndrome and celiac disease (1 case); and family history of adenomatous polyposis, who underwent ileoanal anasto- mosis, which was the cause of the vascular compression of the duodenum (1 case).
Patients with histories of many surgi- cal interventions on the abdominal cav- ity were five cases and in all of them, the symptoms persisted and only disappeared when an enteric bypass procedure was practiced: duodenojejunostomy in four cases, and gastrojejunostomy in Y of Roux in one case.
WILKIE’S SYNDROME. CAUSES OF WEIGHT LOSS.
CAUSES | Number of cases and percentage |
Anorexy | 5 (20.83%) |
Disabling neurological diseases | 5 (20.83%) |
Low-calorie diets | 4 (16.67%) |
Oncological diseases | 3 (12.50%) |
Endocrine diseases (hyperthyroidism) | 2 (8.33%) |
Plastic surgery (patients undergo diets with losing weight before
plastic surgery) 2 (8.33%)
Burns 1 (4.17%)
Debilitating diseases and chronic infections 2 (8.33%) Repetitive episodes of lung infection; hepatitis at age 16;
chickenpox in childhood; disseminated herpes simplex; 1
autoimmune hemolytic anemia and bronchiectasis
Chronic malnutrition exacerbated and family history of
tuberculosis and abdominal tuberculosis demonstrated by 1
lymph node biopsy
WILKIE’S SYNDROME. ANATOMICAL CAUSES THAT DECREASED THE DISTANCE AORTOMESENTERIC.
CAUSES Number of cases Percentage
Diseases of the spine, or orthopedic or surgical procedures on the spine
10 58.82%
Peripheral vascular diseases 5 (Abdominal aortic aneurysms 4)
29.41%
Previous surgical interventions (ileoanal anastomosis) 1 5.88%
A large mass located in the hepatic angle of the colon (colon adenocarcinoma) The tumor that pulled and tensed from the transverse mesocolon causing the duodenal compression.
1 5.88%
WILKIE’S SYNDROME. CLINICAL MANIFESTATIONS.
groups studied, to predict what symp- toms could establish the need for sur- gical treatment, the Chi-square values
Clinical manifestations
Number of cases
Percentage
obtained were: for pain 1,105824541,
for vomiting 11,051156656 and for
Abdominal pain 114 76%
weight loss 18,78133536. As the value
of the degrees of freedom was 2 and the
Vomiting
Bilious vomiting
113
41 (36.28%)
of the 113 cases with vomiting
75.33%
reliability of 99%, the critical value cor- responded to 18,4662 and the only val- ue of the Chi-square test that exceeded the critical value, was the value of Chi-
Nausea 5 3.33%
Food intolerance 1 0.67%
Weight loss 73 48.67%
Abdominal
distension 27 18.00%
surgical treatment upon admission: 50 patients. We excluded six patients, five in which the treatment received was not specified and one that died before being able to receive the treatment. The clinical manifestations of each of these groups are shown in Table IV. The incidence of vomiting and weight loss were more frequent in patients who re- quired surgical treatment as you can see in Table V. Bilious vomiting was more frequent in patients who need sur- gical treatment upon admission. When we applied the Chi-square test to each of the clinical manifestations (pain, vomiting, and weight loss) in the three
square test for weight loss.
a. - Standing X-ray of the abdomen showed gastric dilation, accompanied or not by duodenal dilation, in 62.50% of cases. b.- Upper gastrointestinal tract radiogra- phy showed the narrowing of the hori- zontal part of the duodenum and delay in contrast transit through the gastroduo- denal region accompanied or not by gas-
WILKIE’S SYNDROME.CLINICAL MANIFESTATIONS BY GROUPS.
Abdominal pain | Vomiting | Weight loss | Total Patients | |
Patients responded to medical treatment | 47 (42.34%) | 39 (34.51%) | 17 (23.94%) | 60 (41.67%) |
Patients did not respond to medical treatment and needed surgical treatment | 24 (21.62%) | 30 (26.55%) | 20 (28.16%) | 34 (23.61%) |
Patients needed surgical treatment upon admission | 40 (36.03%) | 44 (38.93%) | 34 (47.89%) | 50 (34.72%) |
Total | 111 | 113 | 71 | 144 |
WILKIE’S SYNDROME. CLINICAL MANIFESTATIONS IN PATIENTS THAT RESPONDED TO MEDICAL TREATMENT VS CLINICAL MANIFESTATIONS IN PATIENTS THAT NEEDED SURGICAL TREATMENT.
Abdominal pain | Vomiting | Weight loss | Total Patients | |
Patients responded to medical treatment | 47 (42.34%) | 39 (34.51%) | 17 (23.94%) | 60 (41.67%) |
Patients needed surgical treatment | 64 (57.66%) | 74 (65.49%) | 54 (76.06%) | 84 (58.33%) |
Total | 111 | 113 | 71 | 144 |
WILKIE’S SYNDROME. RADIOLOGICAL FINDINGS IN THE X-RAYS OF THE STANDING ABDOMEN IN 24 CASES.
Radiological findings found Number of cases Percentage
Gastric dilatation 13 (two cases with
significant gastric dilatations)
54.17%
Gastric and duodenal dilatation 2 8.33% Air-fluid level in upper abdomen and gastric chamber distension 2 8.33% Air-fluid level in right iliac fossa 1 4.17%
Double bubble image 1 4.17%
Dilatation loop small intestine 1 4.17%
Intestinal pneumatosis 1 4.17%
Study reported without alterations or inconclusive 3 12.50%
Total 24 100%
WILKIE’S SYNDROME. RADIOLOGICAL FINDINGS IN THE UPPER GASTROINTESTINAL TRACT RADIOGRAPHS IN 101 CASES.
Radiological findings Number of cases Percentage
Gastroduodenal dilatation with duodenal constriction and delay in contrast transit
50 50%
Duodenal constriction and delay in contrast transit 35 35%
Gastroduodenal dilatation 11 11%
Study reported as normal 02 2%
Radiological findings suggestive of Wilkie’s syndrome 01 1%
Duodenal ulcer with delay in contrast transit 01 1%
Total 100 100%
WILKIE’S SYNDROME. FINDINGS FOUND IN UPPER DIGESTIVE TRACT ENDOSCOPY IN 42 CASES.
Findings | Number of cases | Percentage |
Gastroduodenal dilatation with narrowing of the horizontal part of the duodenum | 13 | 30.95% |
Narrowing of the horizontal part of the duodenum | 13 | 30.95% |
Gastroduodenal dilatation | 03 | 7.14% |
Stomach operated Billroth I | 01 | 2.38% |
Gastric cancer | 01 | 2.38% |
Gastritis and gastroduodenitis | 04 | 9.52% |
Bile inside the stomach | 01 | 2.38% |
Gastric stasis | 03 | 7.14% |
Phytobezoar in the duodenum | 01 | 2.38% |
Study reported as normal | 02 | 4.76% |
Total | 42 | 100% |
troduodenal dilation in the 85% of cases. c.- Computed tomography showed: the aortomesenteric angle was reported di- minished, without indicating its valor in 9 cases. In 37 cases the valor of the aortomesenteric angle was reported with precision, varying between 6º to 40º and with an average of 16.1º. In the rest of the 11 cases, the valor of the aortomesenteric angle was reported as follows: less than 13º (one case), less than 15º (one case), less than 20º (six cases) and less than 25º (three cases). The aortomesenteric distance was reported diminished, without indicating its valor in 14 cases, one of them with an aneurysm. The valor of the aortomesenteric distance was reported in 22 cases. In 19 cases the valor of aortomesenteric distance was re- ported with precision, varying between from 0.53 mm to 10.5 mm with an aver- age of 4.38 mm. In three cases the valor of the aortomesenteric distance was report- ed as follows: less than 5 mm, between 4 and 5 mm, and between 6 and 8 mm. d.- Upper gastrointestinal tract endoscopy showed the narrowing of the horizontal part of the duodenum, accompanied or not
by gastroduodenal dilation in the 61.90%. e.- Magnetic resonance imaging was prac- ticed in three cases, reporting the value of the aortomesenteric angle in two cases (10º and 17º) and in the remaining case, they reported extrinsic compression of the horizontal part of the duodenum. f.- Arteriography in two cases, the aor- tomesenteric angle was reported di- minished, without indicating its valor.
g. - Abdominal ultrasound was performed in 29 cases, showing bowel dilation in
13 cases, decreased aortomesenteric angle in three cases, decreased aorto- mesenteric distance two cases in both of 3 mm, compression of the horizon- tal part of the duodenum one case and pneumobilia and fluid in pelvis one case. h.- Diagnostic laparoscopy in one case revealed extrinsic compression of the duodenum by the mesenteric vessels at the level of the birth of the middle colic artery.
94 (65.27%) received medical treat- ment and of these, 59 patients (62.77%) presented a satisfactory response. One
62-year-old male patient, with the diag- nosis of well-differentiated gastric ade- nocarcinoma classified as Borrmann II, died for bronchoaspiration with sepsis of respiratory origin, while waiting for a laparoscopic approach to gastric can- cer and Wilkie syndrome. Of the 84 pa- tients who required surgical treatment, 50 patients (59.52%) required it upon
admission, and 34 patients (40.48%) af- ter receiving medical treatment with an unfavorable response. Table IX shows the surgical procedures performed. Lat- erolateral duodenojejunostomy alone or in combination with other surgical
procedures was the surgical interven- tion most used and was performed in 59 cases (70.24%).
Postoperative results, complications, and mortality: Of the 84 patients who received surgical treatment, 79 cases (94.05%) had satisfactory results, the- re were four deaths (4.76%) and one case (1.19%) did not report results. Intra and postoperative complications occurred in seven patients and are shown in Table X.
SURGICAL PROCEDURES IN WILKIE’S SYNDROME.
Surgical procedures | Nº of Cases | Percentaje |
A. Duodenal derotation (1) | 2 | 2.38% |
Treitz ligament section with duodenal descent (Strong technique)
as a unique surgical procedure (2) 11 13.10%
Treitz ligament section with duodenal descent (Strong technique) combined with another surgical procedure in an only surgical intervention
with laterolateral duodenojejunostomy | 10 | 11.91% | |
with superior mesenteric vessel reimplantation in the infrarenal aorta | 1 | 1.19% | |
3. Enteric bypass by | |||
3.1. | Gastrojejunals anastomosis | ||
gastrojejunostomy due to significant gastric dilatation | 3 | 3.57% | |
terminoterminal gastrojejunostomy en Roux Y by previous Billroth I. | 1 | 1.19% | |
3.2. | Duodenoduodenostomy | 1 | 1.19% |
3.3. | Duodenojejunostomy as a unique surgical procedure | ||
laterolateral duodenojejunostomy | 47 | 55.95% | |
Roux-en-Y duodenojejunostomy | 3 | 3.57% |
Duodenojejunostomy ahead of the superior mesenteric vessels with
segmental resection of the duodenum 1 1.19%
Total gastrectomy due to gastric necrosis 2 2.38%
Other surgical procedures
jejunostomy for enteral feeding 1 1.19% resection of a large colon tumor that pulled and tensed the
transverse mesocolon causing the duodenal compression. 1 1.19%
one case presented perforated ulcer at the level of the horizontal part of the duodenum that required resection of third and fourth part of the duodenum and terminoterminal anastomosis of the second part of the duodenum to jejunum.
two cases required a second intervention (laterolateral duodenojejunostomy) in one accompanied by a gastrec- tomy partial.
SURGICAL COMPLICATIONS IN WILKIE’S SYNDROME.
Surgical complications | Nº of Cases | Percentaje |
1. Need for surgical reinterventions | 5 | 71.42% |
Obstruction of the efferent loop by adhesions | 1 | |
Anastomosis leak | 1 | |
A patient undergoing duodenal derotation that required two reoperations: one by a bowel obstruction by adhesions, and another by an ulcer perforated in the horizontal part of the duodenum that required resection of the horizontal and ascended parts of the duodenum with a terminoterminal duodenumjejunal anastomosis | 1 | |
Unsuccessful response to Strong’s technique | 2 | |
2. Pancreatic injury with fistula that responded to medical treatment | 1 | 14.29% |
3. Postoperative shock in a patient with large colon tumor that it pulled and tensed the transverse mesocolon causing a duodenal compression | 1 | 14.29% |
CAUSES OF DEATH IN WILKIE’S SYNDROME.
Causes of death Nº of Cases Percentaje
a 56-year-old male patient with a history of hypertension, alcoholism and chronic renal failure with aortic stent due to aneurysm
1 16.67%
1 16.67%
1 16.67%
1 16.67%
66-year-old male patient 1 16.67%
Wilkie syndrome or vascular compres- sion of the duodenum is the constriction of the horizontal part of the duodenum by the root of the mesentery with partial or total duodenal obstruction. It is a rare and little known entity with an incidence between
0.0024% and 0.53% (111), not included in the initial diagnostic possibilities, which de- lays the diagnosis (38) and treatment.
Its embryological and anatomical bases are well known (111,112): in humans, the superior mesenteric artery under normal conditions arises from the abdominal aorta at the level of the first lumbar vertebra at an
angle that averages 42.4 degrees, with a wide range of variation from 18 to 70 degrees.
The extrinsic compression of the duo- denum by the superior mesenteric artery occurs when the aortomesenteric angle de- creases to values between 1 to 40 degrees with an average of 15.2 degrees (Fig. 3). The left renal vein and the uncinate process of the pancreas, located over the horizontal part of the duodenum, occupy the narrowest part of the aortomesenteric angle (Fig. 3). When the amplitude of the angle decreases, the left renal vein is pressed by the superior mesenteric artery, causing the nutcracker syndrome, which can occur alone (113) or in combination with the vascular compres- sion syndrome of the duodenum (105,114).
In the etiopathogenesis of this disease are distinguished predisposing and precipi- tating factors. The predisposing factors that contribute to the development of the compression of the duodenum by the supe- rior mesenteric artery are (111,115): 1.- The crossing of the horizontal part of the duode- num at the level of the fourth lumbar vertebra where the lumbar curvature reaches its most anterior position and is more pronounced. Under normal conditions, the horizontal part of the duodenum crosses the abdomen at the level of the third lumbar vertebra; oc- casionally, in women, the crossing is at the
level of the fourth lumbar vertebra. 2.- ex- aggerated lumbar lordosis, or orthopedic or surgical procedures on the spine: normally, the aortomesenteric distance is from 10 to 28 mm and an exaggerated lumbar lordosis decreases it to 2 to 8 mm. 3.- A shortened or hypertrophied of the suspensory muscle of the duodenum causes an upward movement of the duodenum at the angle between the superior mesenteric artery and the aorta. 4.- An abnormally low origin of the superior mesenteric artery. The precipitating factors are the significant weight loss or rapid growth without weight gain (8,115) associated with an extensive loss of retroperitoneal and mes- enteric fat that contribute to a decrease in the aortomesenteric angle and distance (8).
We found a predominance of the female sex over the male in a proportion of 2.57, and the 50% of cases occurred in patients under 20 years of age, with the highest inci- dence in schoolchildren and adolescents, as it has been reported in the literature (111). The age range was from one day of life to 83 years of age, with an average of 26.093 years. Medical-surgical background and associated pathologies were present in 53 patients, with weight loss being present in 24 patients (45.28%) (Table I) and anatomical causes that decreased aortomesenteric distance in 17 patients (32.07%) (Table II).
The clinical manifestations of this syn- drome are abdominal pain, vomiting, weight loss (Table III), and sometimes, abdominal distention. The disease has two forms of pre- senting: acute, with signs of high small bow- el obstruction (115-117), and chronic, with an insidious medical history of intermittent abdominal pain associated with vomiting, early satiety, and anorexia, many times for many years. In the chronic presentation, the symptoms are less specific and compatible with those of peptic ulcer disease, pancreati- tis, biliary colic, and patients who are mis- diagnosed and mistreated for many years, (116,118) delaying diagnosis and proper treatment. In our analysis, we could compile the time of apparition of symptoms in 85 cases that ranged from 12 hours to 27 years. The disease manifested in an acute form in 14 cases (16.47%) (in hours in one case and days in 13 cases) and in a chronic form in 71 cases (in months in 39 cases and years in 32 cases) (83.53%). In our review, the most frequent symptoms were abdominal pain, vomiting, and weight loss. The abdominal distention, when it was present was a sign of gastric dilation (79,81,100,119,120) and could be complicated with gastric necrosis (81,100).
To make the diagnosis is necessary a high index of clinical suspicion and the meticulous evaluation of the radiological studies.
The acute presentation form manifests with signs of high partial obstruction of the small intestine. Simple X-ray of abdomen shows a gastric air bubble with few amounts of gas in the intestine distal to the site of ob- struction (5,7,66) See Fig. 4. In the chronic presentation form, the symptoms often do not have the intensity that in the acute pre- sentation form, the vomiting may not have bile, and the clinical picture can be confused with anorexia nervosa and bulimia delaying the diagnosis by months and years.
panied or not by gastroduodenal dilation, and fight sign of the stomach. Under normal conditions, the descending part of the duo- denum empties the barium so quickly, that it is difficult to get a radiograph that shows this part of the duodenum well-distended; while in patients with vascular compression of the duodenum, the descending part of the duodenum remains distended (7); 2.- the narrowing of the horizontal part of the duo- denum, as a vertical abrupt cut-off over or to the right of the spine and 3.- few amounts of contrast and gas, in the intestine distal to the site of obstruction; 4.- the relief of ob- struction with postural changes (121,122).
The computed tomography as a diag- nostic study replaced the hypotonic duode- nography and the aortic and superior mes-
enteric artery angiography, because it is: 1. a fast, reliable, non-invasive method, 2. shows the exact anatomic position of the duode- num in the aortomesenteric angle, 3. allows to calculate the values of the aortomesenter- ic angle and the distance aortomesenteric, and 4. excludes other causes of obstruction (111,122).
In the papers reviewed (121-128), the normal value of the aortomesenteric angle varied between 28º to 65º and the of the aor- tomesenteric distance between 10 and 34 mm. The values of the aortomesenteric angle and distance are considered abnormal when these are less than 22º to 25º and of 8 to 10 mm respectively (66,121-128). In our review, the value of the aortomesenteric angle varied from 6º to 40º with an average of 16.1º, and the of the aortomesenteric distance varied from 0.53 to 10.5 mm with an average of 4.38 mm; and these values coincide with those re- ported in the literature (111,112).
The upper gastrointestinal tract en- doscopy allows: to show the presence of the narrowing of the horizontal part of the duodenum sometimes reported as pulsatile compression, accompanied or not by gas- troduodenal dilation, also rule out associ- ated pathologies such as bezoars (97) and peptic ulcers (107,129-131) and, allows the placement of nasojejunal tubes beyond the obstruction site to provide enteral nutrition support (118). When it is performed with curvilinear array endoscopy ultrasound, the value of the aortomesenteric angle (132) can be determined.
In the differential diagnosis, it is con- venient to keep in mind the diagnosis of inverse duodenum, which is a duodenal anomaly that has also been associated with obstructive symptoms and that simulate the clinical manifestations of Wilkie’s syndrome and its upper gastrointestinal tract radiogra- phy has been interpreted as compatible with the superior mesenteric artery syndrome (133-135).
In the absence of pathological condi- tions that need immediate surgical explora-
tion, for example, an aneurysm, the treat- ment of the Wilkie’s syndrome is initially medical and consists of gastric decompres- sion, correction and maintenance of the hydro-electrolytic balance, antibiotic thera- py and enteral nutritional support through of nasogastric (110) or nasojejunal tube (136-137), which causes weight gain with an increase in the peripheral fat to the superior mesenteric artery that augmentation the angle and the distance between the superior mesenteric artery and the aorta, with the disappearance of the symptomatology. Sur- gical treatment is necessary when medical treatment fails, or when there are clinical manifestations of intestinal obstruction (51, 66,118,137-139).
There are three types of surgical inter- ventions:
Enteric bypass procedure. Of the three surgical procedures included in this section, the laterolateral duodenojeju- nostomy, proposed by Bloodgood (140), first performed successfully by Stavely
(141) and popularized by Wilkie (2-3), is the most frequently performed surgi- cal procedure with satisfactory results and with a very low complication rate. Gastrojejunostomy has been performed when there is a significant gastric dila- tion but it is not recommended because the duodenal content before the site of obstruction must pass to the stomach to drain to the jejunum (137,139). Sometimes, during the surgical inter- vention, the inframesocolic duodenum does not look dilated and the diagnosis of vascular compression of the duode- num could be missed (4). There is an intraoperative maneuver that consists of the injection of 150 to 300 cc of air through a nasogastric tube, observing after the air injection, dilatation of the duodenum from 4 to 5 cm, when there is a vascular compression of the duode- num (139).
the duodenal-jejunal angle: In 1958, | malrotation. The mesentery should be | |
Strong described one case of arteriome- | left attached to the retroperitoneum | |
senteric duodenal obstruction in which | in the region of the ileocecal valve, and | |
he practiced the section of the sus- | the lateral attachments of the ascend- | |
pensory muscle of the duodenum with | ing colon should also be preserved to | |
surgical mobilization of the ascending | prevent midgut volvulus (8). It has been | |
part of the duodenum and relocation of | practiced successfully and has the ad- | |
the duodenojejunal angle from the level | vantage that it does not need the use of | |
of the second to the third lumbar ver- | anastomosis (4-8,143-147). | |
tebral body, with complete remission | When analyzing the medical treatment | |
of the symptomatology (142). In our | vs. surgical treatment of 150 patients, six pa- | |
analysis, we found this surgical techni- | tients were excluded: five in whom the treat- | |
que was performed as an only surgical | ment received was not specified, and one that | |
procedure in 11 patients with a success | died before being able to receive the treat- | |
rate of 81.82%. Two of these cases re- | ment. Of the remaining 144 patients, 94 re- | |
quired a second intervention (laterola- | ceived medical treatment, with a satisfactory | |
teral duodenojejunostomy). In eleven | response in 62.77% of cases, and one death | |
cases, it was performed with another | for bronchoaspiration with sepsis of respira- | |
surgical procedure in a single surgical | tory origin, in a 62-year-old male patient with | |
intervention; in 10 cases with duodeno- | the diagnosis of well-differentiated gastric | |
jejunostomy and in one case with reim- | adenocarcinoma, classified as Borrmann III, | |
plant of the superior mesenteric artery. | who expected by a therapeutic laparoscopic | |
3. | Duodenal derotation: described by | approach to gastric cancer and Wilkie syn- |
Louw and recommended by Wayne | drome. Of the 84 patients who required sur- | |
and Burrington for the treatment of | gical treatment, 59.52% of the cases required | |
children with this pathology (6-8), | it upon admission and 40.48% had received | |
in our analysis, it was only performed | medical treatment with an unfavorable re- | |
in 2 cases and one of them required | sponse. Intra or postoperative complications | |
a second intervention by had perfo- | occurred in seven cases (8.235%), and of | |
rated ulcer in the horizontal part of | these, five cases (5.88%) required surgical re- | |
the duodenum that required resec- | intervention. The causes of surgical reinter- | |
tion of horizontal and ascending part | ventions are shown in Table X. Once the surgi- | |
of the duodenum and terminotermi- | cal complications were overcome, all patients | |
nal anastomosis of the descending | undergoing surgical treatment responded | |
part of the duodenum to the jejunum. | satisfactorily, and the symptoms disappeared. | |
The duodenal derotation also is known | Causes of death. As can be seen in Table | |
as Ladd’s procedure (143-144), consist- | XI, the causes of death found are related to | |
ing of lysing the suspensory muscle of | pathologies concomitant with the duodenal | |
the duodenum with of mobilizing the | vascular compression syndrome (aneurysms, | |
entire duodenum and the proximal je- | malignant tumors, senility, immunological | |
june. The opening at the root of the | alterations). | |
mesentery is enlarged to at least 10 | From this review, we can conclude that | |
cm in length, and the entire jejunum is | vascular compression of the duodenum is a | |
passed to the right through this open- | pathological entity that results from the de- | |
ing. The entire duodenum and proximal | crease in angle and distance aortomesenteric, | |
part of the jejunum now lie in the right | whose highest incidence is in patients under | |
paravertebral gutter, much as they do | 20 years of age, predominating in schoolchil- | |
after the Ladd procedure for intestinal | dren and adolescents. This syndrome has two |
ways of manifesting clinically: 1. an acute clin- ic form, with a high partial intestinal obstruc- tion; and 2. a chronic, insidious presentation, which is often confused with anorexia nervo- sa. It is diagnosed by upper gastrointestinal tract radiography, computed tomography, and upper gastrointestinal tract endoscopy. The diagnostic radiological criteria are: 1. abrupt vertical and oblique compression of the mucosal folds, produced by the constric- tion of the horizontal part of the duodenum by the superior mesenteric artery; 2. delay in contrast transit through the gastroduodenal region; 3. dilatation of the first and second portions of the duodenum, with or without gastric dilatation; 4. an antiperistaltic flow of barium proximal to the obstruction pro- ducing to-and-fro movements; 5. relief of obstruction when the patient is placed in a position that diminishes the drag of the small bowel mesentery (the left later decubitus, prone, or the knee-chest position) (148). The tomographic criteria are: 1. abrupt obstruc- tion in the third portion of the duodenum, with active peristalsis, 2. an aortomesenteric angle less than 25º and an aortomesenteric equal to or less than 8 mm 3. high fixation of the duodenum by the suspensory muscle of the duodenum or anatomical variants of the superior mesenteric artery (46,149). The up- per gastrointestinal tract endoscopy allows:
to show the presence of the narrowing of the horizontal part of the duodenum, some- times reported as pulsatile compression, ac- companied or not by gastroduodenal dilation,
rule out associated pathologies such as be- zoars and peptic ulcers and, 3. the placement of nasojejunal tubes beyond the obstruction site for providing enteral nutrition support.
The treatment is initially medical (110) and consists of gastric decompression, fluid electrolytes imbalance correction, antibi- otic therapy, and nutritional support either through total parenteral nutrition or nasoje- junal tube feeding (46,150). The nutritional support is aimed at the gain of weight, to the restoration of retroperitoneal fat, with increase in retroperitoneal and mesenteric
fat that contributes to increasing the values of the aortomesenteric angle and distance. Posturing maneuvers during meals and pro- kinetic medications may be helpful in some patients with nutritional support. If medi- cal treatment fails there are three surgical treatment options (137): 1- Section of the suspensory muscle of the duodenum with the descent of the duodenal-jejunal angle;
laterolateral duodenojejunostomy, and
duodenal derotation. Of these, the most practiced is the laterolateral duodenojeju- nostomy with satisfactory results.
We would like to thank the staff of the “Dr. Francisco Baquero González” Library for the search for medical research papers; Drs. Hilda Martorelli and Jorge Barba Flores, who reviewed the paper and for its transla- tion into the English language.
/10.1177/153857449502900308.
Br J Surg 1921;9:204-214. https://doi. org/10.1002/bjs.1800093405.
artery — Does it exist in children? J Pediatr Surg 1974;9(5):733-741. Doi: https://doi. org/10.1016/0022-3468(74)90112-2.
térica superior: relato de um caso. Pediatria (São Paulo) 2003;25(3):134-137.
Síndrome da artéria mesentérica superior. Rev Col Bras Cir 2000;27(2):128-130.
9363201100040001.
141. Available from: http://files.bvs.br/ upload/S/0101-7772/2012/v31n4/a3929. pdf.
aorta abdominal: relato de caso. Available from: http://iradtelerradiologia.com.br/ wp-content/uploads/2017/12/RELATO- DE-CASO-SAMS.pdf.
429.
lable from: http://www.scielo.org.co/pdf/ cesm/v28n1/v28n1a12.pdf
tarric 2015;57(3):145-148. Available from: http://actamedica.medicos.cr/index.php/ Acta_Medica/article/download/893/811
Molina Proaño GA, Cobo Andrade MM, Guadalupe Rodríguez RA, Gálvez Salazar PF, Cadena Aguirre DP, González Poma GV, Gutierrez Granja BM. Wilkie’s syndro- me, a missed opportunity. J Surg Case Rep 2018;9:1–3. Doi: 10.1093/jscr/rjy246.
Cir Endoscop 2009;10(1):49-53. Available from: https://www.medigraphic.com/pdfs/ endosco/ce-2009/ce091j.pdf.
125. Available from: https://www.medigra- phic.com/pdfs/revmexcirped/mcp-2009/ mcp093c.pdf.
281. Available from: https://www.medigra- phic.com/pdfs/cirgen/cg-2018/cg184j.pdf.
48. Available from: https://www.revistacen- tromedico.org/ediciones/2019/2/art-3/
290. http://www.aeped.es/sites/default/ files/anales/45-3-14.pdf.
Pediatr 2008;21:228-231. Available from: https://www.secipe.org/coldata/upload/re- vista/2008;21.228-31.pdf
178. Available from: http://portal.scptfe. com/wp-content/uploads/2013/12/2013- 2.a.pdf
mesenteric artery syndrome: a rare cause of intestinal obstruction due to the lack of fat. Sapd online 2017;40(4):193-195. Available from: https://www.sapd.es/revis- ta/2017/40/4/06/pdf.
Wilkie’s syndrome in monozygotic twins treated by 3-D laparoscopic duodenojejunos- tomy. Asian J Endosc Surg 2019;12(1):125-
127. Available from: https://onlinelibrary. wiley.com/doi/epdf/10.1111/ases.12489.
¿Infrecuente o infradiagnosticado? Rev Esp Pediatr 2014;70(4):183-188. Availa- ble from: https://www.seinap.es/wp-con- tent/uploads/Revista-de-Pediatria/2014/ REP%2070-4.pdf#page=6
695403314005694-main.pdf.
134. https://doi.org/10.1016/j.gastrohep.
2008.09.012.
complicating Crohn disease of the upper gas- trointestinal tract. GE Port J Gastroenterol 2017;24:50–52. Doi: 10.1159/000450873.
Available from: https://www.karger.com/ Article/Pdf/450873.
28. Available from: http://www.scielo.mec. pt/pdf/rpc/n37/n37a05.pdf.
Vascular Compression of the Duodenum. In Fischer JE. Editors. Mastery of Surgery. 5th Edition Lippincott Williams & Wilkins 2007. p. 955-961.
302. Available from: http://www.scielo.org. mx/pdf/bmim/v71n5/v71n5a7.pdf.
Barsoum MK, Shepherd RF, Welch TJ. Pa- tient with both Wilkie syndrome and nutcrac- ker syndrome Vasc Med 2008;13(3):247-50. Doi: 10.1177/1358863X08092272. Availa-
ble from: https://journals.sagepub.com/ doi/pdf/10.1177/1358863X08092272.
/00000658-197102000-00017.
appears in Radiographics 2015 ;35(3):973].
Radiographics 2014;34(1):93–115. Doi: 10. 1148/rg.341125010.
261-012-9852-z
Makary MS, Rajan A, Aquino AM, Chamar- thi SK. Clinical and radiologic considera- tions for idiopathic superior mesenteric ar- tery syndrome. Cureus 2017;9(11):e1822. Doi: 10.7759/cureus.1822. Available from: https://www.ncbi.nlm.nih.gov/pmc/arti- cles/PMC5755943/
Thompson NW, Stanley JC. Vascu- lar Compression of the duodenum and peptic ulcer disease. Arch Surg 1974; 108(5): 674–679. Doi: 10.1001/archsurg.
1974.01350290038005.
9610(57)90557-3.
unsuspected entity. J Gastrointest Endos- cop 2009; 69(2):S234. Doi: 10.1016/j.gie. 2008.12.097.
jpedsurg.2012.10.066.
jpedsurg.2014.07.008.
5. Doi: 10.1016/j.jpedsurg.2017.07.004.