Laparoscopic treatment of celiac axis compression syndrome (CACS) and hiatal hernia: Case report with bleeding complications and review

Abstract

INTRODUCTION

Median arcuate ligament (MAL) malposition is a rare cause of celiac axis compression syndrome (CACS) or Dunbar syndrome.

PRESENTATION OF CASE

A 26-year-old female presented with severe postprandial epigastric pain, weight loss, heartburn and regurgitation unresponsive to medical therapy. CT angiography and duplex ultrasound demonstrated the MAL crossing anterior to the celiac artery (CA). Reconstructions demonstrated CA compression, while the superior mesenteric artery (SMA) was normal. The MAL was laparoscopically divided, releasing the celiac axis. A concomitant Nissen fundoplication was performed. At 3-months follow-up, the CT-scan demonstrated no evidence of CACS with complete symptom resolution.

DISCUSSION

Dunbar's syndrome can be treated with endovascular surgery, laparoscopic MAL division or vascular surgery.Six anatomical and morphologic variations of aortic and esophageal hiatus are described. The result of the analysis of these anatomical data leads to the conclusion that hiatus hernia, Dunbar's syndrome and GERD have a common etiopathogenesis and physiopathology.

CONCLUSION

Laparoscopic treatment is useful and feasible in centers with experience in majorlaparoscopic surgery with reduced invasiveness, better cosmetic effect and shorter postoperative course.

1. Introduction

Celiac artery compression syndrome (CACS) was first described by Harjola.¹ In 1965, Dunbar reported the first surgically treated patients.² Several pathogenetic hypotheses have been proposed. The origin of the celiac artery may be abnormally high, leading to compression by an anatomically normal median arcuate ligament (MAL). Alternatively, an exceedingly long MAL could compress a normal celiac artery. The blood steal hypothesis suggests that large collateral vessels can and will acquire a greater percentage of total blood flow postprandially, depriving smaller vessels of the flow they require.³ The last hypothesis stems on a neurogenic cause. There is a wide range of variation regarding the location, morphology, and neural interconnections of the plexus.⁴ Reports in the literature have shown that this plexus, when fibrotic, can restrict blood flow to the celiac artery.^5,6 The pathology is more common in young women with low BMI, with a clinical history of chronic postprandial abdominal pain, diarrhea, vomiting, epigastric bruit and weight loss. CACS diagnosis is radiological in all patients by digital subtraction angiogram, computed tomogram scan, MRI or duplex ultrasound.⁷ Computed tomography (CT) angiography allows visualization not only of the stenosed vessel but also the underlying median arcuate ligament and adherent tissue using 3D imaging. Inspiratory and expiratory MRI may discriminate between normal variations of the celiac trunk or pathological narrowing of the proximal celiac segment.⁸

2. Case report

A 26-year old, female patient with a body mass index of 21 complained of 8-kg weight loss in a year associated with post-prandial abdominal pain. The pain which was predominantly localized to the upper and mid-abdomen; there was no evidence of organic or functional pathology. Color-flow duplex sonography showed an “ascending” course of the celiac trunk, with reduction of the ostial segment's caliber, in expiration, and mild post-stenotic ectasia with systo/diastolic blood flow acceleration (>300 cm/s). These data were consistent with a significant stenosis (Fig. 1). There was also evidence of an increase of the systolic-peak in the initial portion of the superior mesenteric artery (200 cm/s). CT angiography confirmed the diagnosis of Dunbar's syndrome, showing that the celiac trunk was constricted during expiration (Fig. 2). Her symptoms were also consistent with a degree of gastro-oesophageal reflux disease confirmed on oesophageal pH studies. Given the clinical presentation the patient was offered laparoscopic MAL division and a concomitant Nissen–Rossetti fundoplication.

Fig. 1.

Preoperative Eco-color-doppler of aorta and celiac trunk preoperative during inspiration and expiration.

Fig. 2.

AngioTc showing the preoperative angle formed by aorta and celiac trunk and its postoperative increase during follow up. Abbreviations: MAL, medium arcuate ligament; CACS, celiac artery compression syndrome; GERD, gastroesophageal reflux disease.

Following pneumoperitoneum induction, four ports were inserted (one 10 mm port for the camera, three 5 mm operating ports). The pars flaccida of the lesser omentum was opened and the right crus of the diaphragm was isolated. The MAL superficial fibers were identified. The common hepatic artery and the left gastric artery were identified and followed to the origin of the celiac trunk. The MAL fibers were disected and divided and the celiac trunk was skeletonized. During skeletonization, a small puncture occurred in the proximal celiac trunk. An additional 5 mm port was placed in the left periumbilical space. The bleeding was controlled with a combination of a haemostatic mixture (Human Trombin + Gelatin Matrix) and one polypropylene suture. A routine Nissen¹⁹ fundoplication was performed. Her postoperative course was unremarkable. Abdominal CT with and without contrast and duplex ultrasound were performed on the sixth postoperative day and were repeated three months post-operatively. There was a clear and progressive widening of the aorto-celiac angle (Fig. 2). Her postprandial cramping pain disappeared and she had gained 2 kg in weight three months after the operation.

3. Discussion

Dunbar's syndrome can be treated with endovascular treatment, with open or laparoscopic MAL section and with vascular surgery.⁷ Delis et al.⁹ used several endovascular procedures, with or without stenting, but with poor results because the prolonged compression had caused persistent changes to the vascular wall. They demonstrated that stent placement brought temporary symptomatic improvement but with early recurrence. They suggested that angioplasty-induced trauma produced intimal and medial fissures and lacerations, rendering the vessel more susceptible to collapse because of the extrinsic compression.¹⁰ The stent may be subject to displacement and fragmentation due to the strong compression of the MAL.

Cina and Safar treated the pathology with an aorto-celiac bypass with Dacron or saphenous vein graft¹¹ or with vascular reconstruction by saphenous vein patch.⁷ The most common procedure is open or laparoscopic surgical resection of MAL fibers compressing the celiac trunk and of the overlying neural and lymphatic celiac plexus tissue.¹² Baccari et al.,¹³ with 16 cases treated laparoscopically, showed the feasibility, safety and the advantages coming from a reduction of the intraoperative blood loss and a shorter postoperative course. Celiac truck injury during skeletonization is the most important cause of conversion to open surgery. Therefore it is advisable to minimize coagulating energy during the final part of aortic and celiac trunk dissection. Small injuries can be treated as described in our case, avoiding conversion to open surgery.

Baccari's data suggest a conversion rate of 12.5% and symptom resolution in 100% of cases. Roseborough¹⁴ published a series of 15 patients in 2009 reporting a conversion rate of 27% with resolution of the symptoms in 95% of cases. The third series was described by Tulloch¹⁵ and registered a conversion rate of 20%. The case we reported is unusual because the potential association between hiatus hernia and Dunbar's syndrome is poorly documented in the literature. According to Loukas’ hypothesis¹⁶ some anatomical variations of diaphragmatic crura, especially in their contribution to the esophageal and aortic hiatus, could be the physiopathological basis of hiatal hernia, Dunbar's syndrome and gastroesophageal reflux. Diaphragmatic crura exhibit six anatomical patterns which feature different distributions of muscular and tendinous tissue. It is possible that the morphologic orientation of the muscle fibers, as well as their composition (muscular vs. tendinous), could have a constrictive effect on the celiac trunk. If there is no true MAL, and the muscular arms of the crura are thinned by posterior extensions of the esophageal hiatus, the aortic and esophageal openings may become practically confluent, although there is always some connective tissue between them. An abnormal thickening of this connective tissue was reported by Bech in several cases.⁴ The results from surgical series of CACS treatment are summarized in Table 1.

Table 1.

Summary of other authors’ experience in the treatment of CACS.

Author	Year	#Cases	Procedure's duration	Blood loss	Duration of hospitalization	Follow up	Improvement of symptoms
Roayaie20	2002	1	135 min	55 cm3	15 h	3 months	Yes
Dordoni21	2002	1	125 min	65 cm3	48 h	6 months	Yes
Carbonell22	2005	1		–	72 h	7 months	Yes
Baldassarre23	2007	1	130 min	–	–	3 months	Yes
Jaik24	2008	1	160 min	50 cm3	48 h	6 months	Yes
Jarry25	2008	1	168 min	–	24 h	1.5 months	Yes
Duffy26	2008	1	218 min	–	24 h	10 months	Yes
Vaziri27	2009	3	151 min (range:114–217)	<50 cm3	24 h	6 months	Yes
Rotellar12	2009	7	111 (range:90–120)	<50 cm3	65 h	6 months	Yes
Tartaglia	2011	1	240	30 cm3	11 g	3 months	Yes
Baccari13		16	90 min	50 cm3	72 h	28.3 months	Yes
Berard28	2009	11	80 min	20 cm3	–	35 months	Yes
Tulloch15	2010	10	220 min	0.5 cm3	–	14 months	Yes
Roseborough14	2009	8	189 min	–	72 h	44.2 months	Yes

Anatomically, the celiac artery is typically found to originate between the 11th thoracic and the 1st lumbar vertebrae.³ Loukas et al.¹⁷ underline the variability of both the celiac trunk's origin and diaphragmatic crura morphology. The celiac artery may originate above the level of T11; a superior origin of the celiac artery would subject this vessel to compression by the MAL. Generally a more proximal origin of the celiac artery occurs more frequently in women than in men, accounting for the female predominance of CACS. The angle of origination of the celiac trunk, as well as the angle of declination of the diaphragmatic crura could influence celiac axis compression. If the diaphragmatic crura deviate acutely relative to the abdominal aorta/vertebral column (long muscular arches) they may constrict the celiac trunk, via the MAL. The inverse must then be considered with the celiac artery deviating obtusely toward the diaphragm.¹⁸

Six anatomical and morphologic variations of aortic and esophageal hiatus are described. The most common type (Type I, 45%) comprises an esophageal hiatus formed by muscular contributions arising solely from the right crus. In Type II (20%) the esophageal hiatus is formed by muscular contributions from the right and left crura. In Type III (15%), the right and left muscular contributions arise from the right crus with an additional band from the left crus. In Type IV (10%) the right and left muscular contributions arise from the right crus, with two additional (anterior and posterior) bands arising from the left crus. Type V (5%) arises solely from the left crus. In Type VI (5%) the right and left contributions originate from the left crus with two additional bands, one from the right crus and one from the left crus.

The result of the analysis of these anatomical data leads to the conclusion that hiatus hernia, Dunbar's syndrome and GERD have a common etiopathogenesis and physiopathology. Notably, in Tulloch's series GERD was described among his three patients’ comorbidities. Symptoms of hiatal hernia are often the same as that of GERD and also Dunbar's syndrome symptoms could be misinterpreted for GERD. Baccari et al. also observed that an accentuated release of the celiac artery from abnormal MAL, which often disrupt the anatomy of the diaphragmatic crura, can cause symptoms due to the postoperative GERD or to the evidence of hiatal hernia. In our patient the conditions coexisted, and were treated simultaneously. Careful patient selection is needed to achieve successful laparoscopic treatment; poor candidates are those with previous intra-abdominal operations and intraperitoneal adhesions.

4. Conclusion

Laparoscopic treatment of CACS is technically feasible and a useful alternative, in centers with experience in major laparoscopic surgery. Its minimal invasiveness, better cosmetic outcome, shorter postoperative course, and duration of hospitalization are the greatest benefits. The conversion risk is arises from the potential for a subadventitial celiac trunk laceration due to an excessive skeletonization with harmonic scalpel.

Conflict of interest

None declared.

Funding

None.

Ethical approval

We have obtained written consent to data treatment for this case report from the patient and we can provide this should the Editor ask to see it.

Author contributions

Lorenzo di Libero: study design and data collections; Antonio Varricchio: writing and data collections; Ernesto Tartaglia: writing and editing images; Igino Iazzetta: data collections; Alberto Tartaglia: first operator, reviewer, and proof editor, study design; Antonella Bernardo: data collections and tables; Rosanna Bernardo: writing; Giovangiuseppe Triscino: study design; Domenico Lo Conte: writing and data collections and research of references.