Wilkie’s syndrome as a cause of anxiety-depressive disorder:A case report and re

lNTRODUCTlON

Superior mesenteric artery(SMA)syndrome is a rare cause of proximal small bowel obstruction,defined as compression of the third part of the duodenum between the aorta and superior mesenteric artery[1].The syndrome has been given many names,including chronic duodenal ileus,Wilkie’s syndrome,arteriomesenteric duodenal compression syndrome,and cast syndrome[1,2].

Its incidence ranges between 0.005 and 0.3%[1,3-7].In fluoroscopic studies,the reported incidence is 0.01%[8].A reported incidence of 0.8% was reported by Neri[9]in a prospective study,while other reports mention a frequency of up to 2.4%[10].In patients with gastric bypass surgery,the reported prevalence is 0.6%[11].

There are approximately 400 cases reported in the literature.SMA syndrome diagnosed after Roux-en-Y gastric bypass has been reported for 14 cases,while one case was reported after sleeve gastrectomy[12].

Despite numerous case reports,no large trials have been published,the diagnosis is often delayed,and patients are treated with ineffective symptomatic therapies[1,13].

His two elder brothers came and knelt before him and confessed what they had done, and said that he alone should inherit the kingdom, and they would always be his faithful subjects!

The importance of this pathology is derived from its association with significant morbidity and mortality due to a delayed diagnosis.This is why it is considered an important differential diagnosis,especially in a setting of recent weight loss[1].Additionally,the choice of treatment requires individual evaluation.

We present the case of a 33-year-old female patient diagnosed with Wilkie’s syndrome 15 mo after the onset of symptoms,initially assumed to be anxietydepressive disorder.

CASE PRESENTATlON

Chief complaints

A 33-year-old female patient presented to the emergency department for diffuse,progressive abdominal pain,nausea,bile vomiting,loss of appetite,and weight loss of 15 kg in the last three months.The pain was characteristically located in the epigastrium,with diffuse extension in the rest of the abdomen,after food ingestion.

History of present illness

However,as the syndrome progresses,nutritional support becomes less successful,and surgical correction is necessary[17].Recently,many studies have suggested an earlier surgical approach,since prolonged medical therapy is associated with multiple admissions,a lower success rate,and increased disease recurrence.They recommended no more than 3 mo for a trial of conservative management[6,22].

History of past illness

She was previously diagnosed with Hashimoto thyroiditis and was under levothyroxine replacement therapy at 125 μg/d.After the onset of her symptoms,she was diagnosed with major anxiety-depressive disorder and placed under treatment with alprazolam 0.5 mg,1 mg/24 h,and escitalopram 10 mg,25 mg/24 h.

Personal and family history

A psychiatric consultation was performed,and the diagnosis of major anxietydepressive disorder was confirmed.Treatment was continued with alprazolam 0.5 mg,1 mg/24 h and escitalopram 10 mg,25 mg/24 h,and regular evaluations were scheduled.

Physical examination

Physical examination revealed a cachectic patient with an anxious face and a body mass index(BMI)of 17.8 kg/m²(normal level 18.5-24.9 kg/m²)at 45 kg and 159 cm height,with tenderness in the epigastrium.

Laboratory examinations

Laboratory results showed an elevated hemoconcentration[hemoglobin 16.8 g/dL(n.v.12-15.5 g/dL),hematocrit 48.8%(n.v.37%-47%)],with hyposodemia[131 mmol/L(n.v.136-146 mmol/L)],and hypocloremia[91 mmol/L(n.v.101-109 mmol/L)],which were easily corrected with fluid resuscitation.

Imaging examinations

Given her symptoms in the superior abdomen,upper digestive endoscopy was performed,and esophagitis and gastritis secondary to bile reflux in the gastric antrum were detected.The first and second parts of the duodenum also had acute erosion and flattened folds.Biopsies were performed to exclude celiac disease and confirm chronic duodenitis.Treatment was initiated with proton pump inhibitor 40 mg,80 mg/24 h,sucralfate 2 tb/12 h,and prokinetic domperidone 10 mg,30 mg/24 h.Vitamins were prescribed.

After one month,given the persistence of the symptoms,computer tomography angiography(Angio-CT)of the abdomen and pelvis was performed.The results revealed emergence at a sharp angle of 13.7° of the superior mesenteric artery,with a reduced distance between the SMA and the anterior wall of the aorta up to a maximum of 8 mm(Figure 1).In this clinical-biological context,a diagnosis of aortomesenteric clamp was established.

That night I paged through a Bible I found in the hotel room and found the verses: I am leaving you with a gift: peace of mind and heart. And the peace I give isn t fragile like the peace the world gives. So don t be troubled or afraid. Remember what I told you: I am going away, but I will come back to you again. If you really love me, you will be very happy for me, for now I can go to the Father, who is greater than I am. I have told you these things before they happen so that when they do, you will believe in me.

Further diagnostic work-up

The patient had a history of irregular diets,with attempts to lose weight under normal weight conditions.Her history included surgical repair of bilateral inguinal hernia and an episode of acute pancreatitis of unknown cause.

MULTlDlSClPLlNARY EXPERT CONSULTATlON

Georgiana Nagy,MD,PhD,Assistant Professor of Gastroenterology,Department of Gastroenterology,“Iuliu Hatieganu” University of Medicine and Pharmacy

The patient presented with a long history(over 1 year)of vomiting,bloating and abdominal pain,symptoms that worsened over the last 3 mo,leading to severe weight loss and the development of a mixed anxiety-depressive disorder.Considering the symptoms,radiologic criteria and failed conservative therapy,we concluded that a surgical approach was the only appropriate treatment.

Radu Drasovean,MD,PhD,Assistant Professor of General Surgery,Department ofSurgery,“Iuliu Hatieganu” University of Medicine and Pharmacy

Given the long period of persisting symptoms,the severity of the weight loss and the absence of major dehydration or electrolyte abnormalities upon admission,due to fluid resuscitation,nutritional and vitamin supplements used in the period before the diagnosis,the patient shows an indication for surgical treatment with laparoscopic duodenojejunostomy.

Years ago, when I started looking for my first job, wise advisers urged, Barbara, be enthusiastic! Enthusiasm will take you further than any amount of experience.

Andrei Lebovici,MD,PhD,University Lecturer,Department of Radiology,“IuliuHatieganu” University of Medicine and Pharmacy

The angio-CT images helped narrow the differential diagnosis by showing some characteristic features of aortomesenteric clamp without any other morphological changes on CT to suggest other pathological conditions.

Doina Colcear,MD,PhD,Psychiatrist,Clinical Infectious Disease Hospital,Cluj-Napoca,Romania

Repeated psychiatric evaluations prior to the diagnosis of Wilkie's syndrome revealed the presence of an anxiety-depressive disorder that was attributed to somatic symptoms.These psychiatric symptoms persisted after the somatic disease was clarified.Pre- and postoperative antidepressant and anxiolytic treatment with 10 mg/d escitalopramum and 0.5 mg/d alprazolamum helped to relieve the symptoms.Specialized monitoring of the progression of her emotional state after discharge with re-evaluation of treatment is recommended.

FlNAL DlAGNOSlS

The final preoperative diagnosis of the presented case was Wilkie’s syndrome in a 33-year-old female patient with a secondary anxiety-depressive disorder.

TREATMENT

Laparoscopic duodenojejunostomy was performed.A standard surgical technique was followed.After pneumoperitoneum was induced,four ports were placed:A periombilical 10 mm optic port,5 mm port in the right lower quadrant,12 mm working port in the left quadrant,midclavicular line,at the level of the umbilicus,and a 5 mm port in the left upper quadrant,below the costal margin.During exploration,a dilated duodenum in the first and second parts was noticed,with a collapsed third part,without ascites or other pathological changes(Figure 2).The second portion of the duodenum was mobilized by a partial Kocher maneuver.At 25 cm distal to the ligament of Treitz,a jejunal loop was prepared and positioned near the duodenum.A side-to-side duodenojejunostomy was performed using a -60 mm endoscopic liner articulated stapler(Figure 3).The common enterotomy was closed with 3-0 polydioxanone absorbable sutures(Figure 4).

When Vasilissa found herself left alone, she examined the hut, wondering to find it filled with such an abundance of everything. Then she stood still, remembering all the work that she had been bidden to do and wondering what to begin first. But as she looked she rubbed her eyes, for the yard was already neatly32 cleaned and the floors were nicely swept, and the little doll was sitting in the storehouse picking the last black grains and wild peas out of the quarter- measure of wheat.

OUTCOME AND FOLLOW-UP

The postoperative course was uneventful,and the patient was discharged home.Nutritional and gastroenterological monitoring was initiated,while psychiatric monitoring was continued.At the 1-mo evaluation,the patient presented a 10 kg weight gain,with complete remission of symptoms(Figure 5).A barium study was conducted showing a free passage of the contrast through the anastomosis(Figure 6).

This case report is an example of SMA syndrome as a cause of anxiety-depressive disorder.In this patient,the persistence of symptoms for a long period of time,without identifying an organic cause,led to the development of an anxiety-depressive disorder that was interpreted as the source of the symptoms.The condition’s progression,with the persistence of symptoms despite treatment of the anxiety,triggered extension of the investigations,with the establishment of a final diagnosis.

Well, Heavens. Didn t you ever come down here to the station? He pointed14 across to his magazine stand. I ve been there all the time. I own it. I ve watched everybody that came up the stairs. “，——？”。“。。。”

DlSCUSSlON

Superior mesenteric artery syndrome occurs preferentially in young adults and adolescents,with an age range of 10 to 39 years,but cases in 88-year-old patients have also been reported[14].It occurs more commonly in women,with a 3:2 ratio compared to men[1,4,13].

The primary reported cause of this disease is the loss of the fat pad between the aorta and SMA,resulting in a narrower angle between the vessels and compression of the duodenum[1,12].This syndrome can be congenital or acquired[1].Based on this fact,two types of SMA syndrome have been described.The irreversible one(either repeated or permanent)is caused by congenital or developmental factors,such as high insertion of the ligament of Treitz,a low origin of the SMA[5],a short ligament of Treitz,abnormal rotation of the intestines,adhesions due to surgery or duodenal stenosis due to inflammation.Abdominal aortic aneurysms or mesenteric root neoplasms have also been reported to be linked[1,15].For this type,surgery has been reported as essential for treatment.The reversible type(transient),caused by gravity,for example,can be treated with a conservative approach,including positional changes,intestinal tract decompression and improved nutrition[15].Furthermore,there might be a genetic component,as suggested by Castro[16],while 40% of cases have no identifiable risk factors[10].

A rare association has been reported after inferior vena cava(IVC)filter placement with a local inflammatory response and compression of the duodenum between the IVC and the SMA[17].

The main difficulty in establishing the diagnosis of SMA syndrome resides in the fact that patients tend to be asymptomatic until significant weight loss is registered,secondary to intentional dieting or illness.The most frequently reported cause used to be the corrective treatment of scoliosis,where a relative lengthening of the spine increases the tension on the mesentery and narrows the angle between the vessels[13].Today,things tend to be different,with the majority of published reports suggesting a very common association with a psychiatric disease[4,13].SMA syndrome can appear secondary to the disease but can also cause psychological and social problems,including depression and anorexia,due to the severity of the disease[2].

” Very soon it was said that the prince must marry, and that the beautiful daughter of a neighboring king would be his wife, for a fine ship was being fitted out

A characteristic of this disease is significant weight loss,described in different situations as cachexia(AIDS,malabsorption,cancer,paraplegia),hypermetabolism or dietary conditions(anorexia nervosa,drug use).Patients usually register a marked weight loss of ＞ 5 kg[7].Less frequent reports are incriminating weight loss following surgery,secondary to bariatric surgery,esophagectomy or abdominal trauma,in its etiology[1,12,13,18,19].

The diagnosis is based on symptoms and radiological testing[1].It needs a high index of suspicion because of its vague and nonspecific symptoms[12].Additionally,the measurements performed on imaging investigations must also be correlated with the clinical complaints[8],and even this diagnostic approach cannot exclude all motility disorder cases[4].Thus,a realistic diagnosis can only be established with a comprehensive panel of investigations,a specific history of symptoms and characteristic imaging changes.

And the Porcelain Maiden agreed to follow him, and after having given up her clothes, the young man bought a small horse for her, which went like the wind

The heterogeneous presentation is aggravated by the presence of comorbidities,such as mental and behavioral disorders(21.3%),infectious disorders(12.5%),and disorders of the nervous system(11.3%)[6].

An acute course of the disease has been described with life-threatening dilatation of the stomach[1].Despite that,a long-standing onset seems to be more characteristic,with symptom persistence between 6 and 24 mo[7,13],while a time lag of 1 to 51 mo until diagnosis is often described[4,6].

The coffee shop, with its excellent location and coffee, had helped make his vacation a pleasant one. But he knew in his heart, had his brother been there to join him just one day, his vacation would have been a perfect one.

Usually,physical examination and laboratory findings are nonspecific[3].Despite the marked weight loss,patients have serum albumin and serum proteins within normal limits,while frequent electrolyte abnormalities are encountered[13].

The most commonly used imaging modalities are contrast-enhanced computed tomography and MRA[23].Computed tomography(CT)angiography is a more specific investigation,while endoscopy and ultrasound are adjunctive diagnostic methods[6].

CT angiography measures the aortomesenteric angle and the distance between the two vessels.A normal aortomesenteric angle is between 25° and 87° with different subintervals in different reports[1-4,8,10,13,17].The normal reported distance between the two vessels is 10 mm-34 mm[2,3,8,21],confirmed by imaging studies[8].The mean reported angle measurements are 12°[13]and 13.5°[8],with a mean measured distance of 4.4 mm[8].However,there are variations regarding the angle dimensions,between 6°-22° in different reports[1,3,4,8,10,13,21],while reported variations regarding distance are between 2 mm and ＜ 10 mm[1-4,8,10,13,21].It has been suggested that the distance is more accurate for SMA diagnosis than the angle[7].

Cases with SMA syndrome with an angle and distance overlapping the normal range have also been reported.An aorto-mesenteric angle ＜ 8°-46° and a distance ＜ 8 mm have been specifically reported,compared to 25°-87° and 10-34 mm identified in control groups as normal values.In these cases,other diagnostic methods must be considered,such as gastric-emptying scintigraphy[23].

Reports suggest an association of the degree of angulation with(BMI)[10,24].Alzervi found a significant reduction in the aortomesenteric angle and distance in patients with SMA syndrome compared to other patients with acute or chronic abdominal pain,while BMI was positively correlated with the aortomesenteric angle and distance.Thus,BMI could be used as a screening factor for SMA syndrome[21].

The most frequently reported symptoms are abdominal pain(92%),nausea,vomiting(77%),and weight loss(69.2%)[1,4,13,20,21],while in some cases,a predominance of vomiting(70%),nausea(66.3%),abdominal pain(65%),anorexia(33.8%),postprandial fullness(33.8%),and early satiety(12.5%)was reported[3,22].Another reported characteristic is epigastric pain,which worsens in the supine position[1,4,10].A sign that can help suggest the diagnosis is the relief of the patient’s symptoms when leaning forward[12]or in the left lateral decubitus position[13].In our case,weight loss was followed by abdominal pain,nausea,and vomiting,a vicious cycle followed by a marked reduction in weight.

Characteristic CT findings are not just numerical cutoff but also distension of the stomach and proximal duodenum or narrowing of the duodenum at the SMA level.Association with compression of the left renal vein,isolated left renal vein thrombosis,enlargement of the left gonadal vein,or left-sided venous collaterals are additional elements to consider for a positive diagnosis[8].

Rotational CT from the supine to prone position can demonstrate improved outflow passing the SMA[12].Additionally,CT has replaced magnetic resonance enterography(MRE)as a standard investigation[1,21].The advantage of showing anatomical variants and consequences,such as delayed transit and obstruction,is highlighted in limited reports using MRE[25].

Diagnosis can also be confirmed by mesenteric artery ultrasonography[12].Color Doppler ultrasound can also measure the aortomesenteric angle[23].

Endoscopy is useful to diagnose complications,such as esophagitis,reflux gastritis,stasis,and chronic duodenal obstruction[1,13,21].However,a suspicion of obstruction of the third part of the duodenum is raised only in some cases during endoscopy[13].A more specific finding for the diagnosis is pulsatile extrinsic compression of the duodenum,excluding other diseases of the superior digestive tract[23].

At the 5-mo follow-up,the patient was asymptomatic,with a 15 kg weight gain.Considering the favorable postoperative outcome and the degree of weight recovery,the patient continued nutritional and psychiatric monitoring,and psychiatric treatment ceased.Long-term evaluation will establish the degree of involvement of psychiatric pathology in the clinical picture,and final conclusions will be drawn.

Barium or gastrografin contrast studies are classic diagnostic procedures,and specific findings are dilatation of the stomach and the first and second parts of the duodenum and the failure of contrast passage in the third part of the duodenum,with antiperistaltic flow[13,23,26].However,these changes are not specific to SMA syndrome[23].

Gastric-emptying scintigraphy is a known imaging modality to evaluate gastroparesis and gastric motility,and it might be of great value for a differential diagnosis between SMA syndrome and gastric motility disorders,especially in patients with diabetes.This investigation provides a qualitative and quantitative analysis of gastric motility,which might allow differential diagnosis of similar diseases and an assessment of the degree of obstruction or stenosis[23].Other differentials include internal hernia,adhesive disease,intussusception[12],and megaduodenum[13].

Another difficult situation occurs in cases associated with systemic sclerosis,in which gastrointestinal involvement creates a clinical picture similar to SMA syndrome,with progression toward malnutrition[27].Another rare condition that has to be differentiated is aortoduodenal syndrome,namely,obstruction of the third portion of the duodenum by a large abdominal aortic aneurysm[28].

If the disease is unrecognized and left untreated,severe complications may occur,such as malnutrition,dehydration,electrolyte abnormalities,gastric pneumatosis and portal venous gas,gastric perforation,duodenal compromise,ischemia,necrosis[1,17],or gastrointestinal hemorrhage[1].Patients with severe hypokalemia,metabolic alkalosis,and acute kidney injury resulting in cardiorespiratory arrest have also been described[29].Recognition of the condition,even in advanced stages with massive gastric dilatation and gastric ischemia lesions,can lead to remission of the pathological changes with decompression and endoscopic surveillance[15,30],without the need for surgery.

The management of SMA syndrome is individualized,with either conservative treatment and nutritional support or invasive treatment[3].Multidisciplinary management is necessary[1].Given that there is a common association with psychiatric disease or dietary conditions[1,4,13],psychotherapy is an important part of treatment[31],while patients should also be educated on lifestyle changes.Most patients require a dietary consultation for their significant weight loss[1]and a close clinical follow-up by a gastroenterologist and a nutritionist[23].

In the conservative approach,the primary focus should be on weight regain with mass restoration while monitoring the caloric needs with care to avoid refeeding syndrome and recovery of the electrolyte balance[2,5].Treatment includes nasogastric decompression,prokinetic agents,fluid resuscitation,electrolyte correction,parenteral nutrition,nasogastric tube insertion,nutritional support through hyperalimentation[1],and the removal of precipitating factors.Nutrition may be provided by small,frequent meals or nasojejunal feeding,with the aim of providing a high-calorie diet to increase the mesenteric fat and expand the angle[26].Positional changes and different maneuvers have also been reported as aids in tolerating enteral feeds,such as the prone position,left lateral decubitus,knee-chest maneuver,or Hayes maneuver.In cases of failure,parenteral nutrition should be considered[5].

Retrospective studies have reported successful treatment of SMA syndrome in short-term follow-up with enteral nutrition[15,17,26,30-34].After a mean period of 10 mo with enteral therapy,65% of patients were without symptoms,while in 15%,their symptoms had improved.Nutrition was administereda nasal jejunal tube and percutaneous endoscopic gastrostomy with a jejunal extension tube.The reported complications were all catheter-related[35].

The success rates for medical management are reported to be between 14% and 83%[6,10],with a 71.3% success rate at a 5-mo follow-up[35].Predictors of a poor response are a chronic course of disease,prolonged medical treatment,or associated psychosocial disorders[6].

On his way out, he went over to the cashier to ask if she had received any lost gloves, but she said no. Then he went to the manager s office to make the same inquiry12. She asked him what colour they were, and when he said black , there came another disappointing answer. Such a pair of expensive gloves, who would give them up? He took his time now, finally waddling13 out of the store.

In children,successful treatment was in most reports obtained by the medical approach,with a decline in the need for surgery from 70% to 14%.An acute presentation was most often registered,while weight loss was not a necessary condition.The expected outcome in these cases was excellent[36].

The symptoms began in the last year,after the initiation of a planned weight loss diet.Over time,her food tolerance gradually decreased,accentuating the symptoms.She presented multiple times to the emergency department with the same symptoms.At every evaluation,an organic cause was excluded,fluid resuscitation was performed,and a psychogenic origin of the symptoms was suspected,given her past medical history.

More than 75% of patients with SMA syndrome require surgical intervention[24].Surgery usually follows a period of refeeding and the correction of electrolyte disorders.Although significant preoperative weight loss is a known risk factor for postoperative complications,there are no data about an optimal period for nutritional support,either enteral or parenteral,or indications for its use.According to some reports,surgery may be safely performed in the presence of normal serum proteins using preoperative nutritional supplementation on an individual basis.Other reports suggest nasogastric decompression and total parenteral nutrition for 7 d before surgery[2].

Several procedures have been described as a possible surgical treatment for SMA syndrome:duodenal circular drainage,derotation procedure,transposition of the SMA to the infrarenal aorta,anterior transposition of the third part of the duodenum,Billroth II gastrectomy,gastrojejunostomy,Ladd’s procedure,Strong’s operation and transabdominal or laparoscopic duodenojejunostomy[1,6,13].All of these procedures are associated with nutritional loss,blind loop syndrome,gastric bile reflux[2,5]or a risk of duodenal entrapment between the pancreaticoduodenal arteries and a persistence of the symptoms in 25% of cases after the Strong procedure[5,10,13].Based on their possible complications,these procedures have all been abandoned,except for duodenojejunostomy[6].Duodenojejunostomy can significantly improve symptoms,physical activity,emotional well-being,and social functioning[12].Laparoscopic duodenojejunostomy has the advantages of an acceptable operating time,a faster recovery,reduced postoperative pain,shorter hospitalization,reduced risk of incisional hernia,and a good cosmetic outcome[1,4,6,10,16,26],which makes it important in young patients with associated psychosocial symptoms secondary to the severity of their disease[2].Blind loop syndrome is the most common complication after this type of procedure[13].

I can quench8 my thirst without you, and bending over the well he lapped up the water so greedily that he plunged9 his face, beard and all, right into the crystal mirror

To close the common enterotomy,a mini laparotomy and a manual suture were performed in most of the reported cases to avoid a narrowed anastomosis.An alternative to this approach is to close the enterotomy with a laparoscopic suture or a linear stapler in a complete laparoscopic maneuver[26],as was done in our patient.

The success rate of surgical treatment is reported to be as high as 75%-100%[1,4,8,10,16].Minimally invasive procedures,including robotic and single-port approaches[37],have reported a success rate of more than 90% with a 7% complication rate[6],while laparoscopic duodenojejunostomy alone has a success rate of 96%[1,10].

In Lee[22]’s case series,where half of the patients presented with associated diseases,a medical treatment success rate of 71.3%,with a 15.8% recurrence rate,was reported,while surgical management had a 92.9% success rate.

The postoperative course is marked by a major weight gain,usually registered during the first postoperative 6 mo with a mean gain between 5 and 15 kg[13].

Intermediate follow-up results were reported by Chang[4]for 26 patients treated with laparoscopic or robotic duodenojejunostomy,with the majority of cases secondary to psychiatric disease.Only 33.3% declared resolution or improvement of their symptoms,while 30% of patients were still severely symptomatic,with 19% of patients still requiring nutritional supplementation.Recurrence was associated with a patent anastomosis and the concomitant presence of dysmotility disorders,such as gastroparesis or global intestinal dysmotility,with an indication for intestinal transplant[4].An important conclusion was that radiographic findings of SMA syndrome should not automatically be assumed to exclude intestinal dysmotility syndrome[4,20].Given the overlapping symptomatology,gastrointestinal transit studies should always be included in the preoperative workup[4].

Without realizing it, little Lauren helped me to learn how truly lucky I am. I was lucky to be able to help Lauren. She taught me a lot about giving and love, and the true meaning of Christmas. That Christmas truly was a memorable15 holiday. Wherever Lauren is, I hope she felt the same way.

Although there are several reports of SMA syndrome,diagnosed as a complication of anorexia nervosa,only a few of them emphasize the evolution of psychiatric disease.Kurisu[38]pointed out that surgical treatment must be cautiously considered in cases of anorexia nervosa,as the long-term results are unclear.They also reported the case of a patient with anorexia nervosa and SMA syndrome treated with surgery,without postoperative improvement.In this case,a psychological approach was necessary to be continued[38].On the other hand,Kornmehl[39]emphasized the overdiagnosis of anorexia nervosa,while SMA syndrome should be considered in these cases.The novelty of our case is represented by the absence of a history of psychiatric diseases,a diagnosis of the disorder after the onset of symptoms,while a restoration of her body weight was registered postoperatively,and psychiatric treatment was stopped.

In their case series,Sun[4]reported a mean weight gain of 3.8 kg at mid-term follow-up,with reported complications such as infection and dumping syndrome.

Cienfuegos[20]presented long-term results(median follow-up of 94 mo)after laparoscopic latero-lateral duodenojejunostomy,with excellent results in 61.5% of cases.Long-term results were also evaluated by Jain[6]with weight gain,complete symptom remission,and no recurrence.

Even though this pathology is rarely described in elderly patients,for this category and in those with significant comorbidities,endoscopic ultrasound-guided gastroenterostomy(EUS-GE)was reported as an appropriate procedure[14,40].EUS-GE using lumen-apposing metal stents seems to be a safe and effective option[40],with a success rate of 90%,adverse events in 5% of patients and a reintervention rate of 11%[41].A rate of 83% in preventing surgery was reported,with a mean period of 8.5 mo until symptom resolution and removal of the LAMS and a 5.6% rate of recurrent symptoms[42].A comparison between EUS gastrojejunostomy and laparoscopic gastrojejunostomy was performed,showing a similar success rate and a significantly higher rate of complications for the surgical procedure[43-45].

On the days when it was Ronny’s turn, I’d give him a silent nod, and he’d fly out of his chair and bound across the room in a blink. He sat awfully12 close -- too close for me in the beginning, I must admit -- and opened the book we were tackling as if he were unearthing13 a treasure the world had never seen.

In addition to the specificity of this complex pathology,some rare associations have been reported in the literature.One of them is the nutcracker syndrome[13,31].The most commonly reported causes of this rare syndrome are anatomical variants such as posterior renal ptosis,a high course of the left renal vein and abnormal SMA branching,but the two syndromes can coexist in the context of major weight loss.Treatment for nutcracker syndrome varies from conservative management to different surgical procedures,with the best results reported after laparoscopic extravascular titanium stent placement[5].

In a small number of cases,an association with cystic fibrosis has been reported.In these cases,BMI below the 5percentile is the best predictor of SMA syndrome.The diagnosis is difficult because of the risk of cystic fibrosis enteropathy[24].Other rare associations are hyperthyroidism-related sympathetic hyperstimulation,vasculopathy and cerebellar infarction[46]and diabetic patients with excessive body weight loss and associated ketoacidosis after using sodium-glucose cotransporter 2 inhibitors[47].Rare associations with rheumatoid arthritis and rheumatoid cachexia have also been reported[48].

This case report highlights the importance of SMA syndrome,especially regarding the consequences when left undiagnosed and untreated for a long period of time,as well as the numerous possibilities of its overlapping with other functional diseases or even being the cause of their onset.

Justin would rub my tummy and talk to his baby sister, telling her she had to wait a little longer because the fruit was not ready to be picked yet. His forays into the plum tree lasted longer each day, as if he was coaxing9 the tree to ripen10 quickly. He talked to the tree and thanked it for letting him know about this important event in his life. Then one day, it happened. Justin came running into the house, his eyes as big as saucers, with a plastic bucket full to the brim11 of juicy purple plums. Hurry, Mommy, hurry! he shouted. She s coming, she s coming! The plums are ripe, the plums are ripe!

The major limitation of this case report is the short follow-up,which allowed us to register the resolution of the anxiety disorder.Only a long-term follow-up will confirm that the syndrome can be a cause for the psychiatric disorder and not the other way around.

CONCLUSlON

SMA syndrome is a rare occlusive pathology that needs an extensive diagnostic workup since it is hampered by a specific mimicry of psychiatric or motility disorders.The vicious cycle of its progression can be interrupted by knowing and considering the large number of differential diagnostic possibilities while individualizing the treatment.Since favorable results depend on the treatment of multiple systems,multidisciplinary management is mandatory.