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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 8  |  Issue : 1  |  Page : 30-33

Right hemicolectomy in a patient with heterotaxy syndrome


1 Department of General Surgery, AZ Maria Middelares, Gent, Belgium
2 Department of Radiology, AZ Maria Middelares, Gent, Belgium

Date of Web Publication12-Mar-2019

Correspondence Address:
Dr. Maxime Dewulf
Buitenring Sint.Denijs 30, 9000 Gent
Belgium
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/WJCS.WJCS_11_18

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  Abstract 

In this communication, we present a first description of right hemicolectomy in a patient with heterotaxy syndrome (HS). A 78-year-old male was admitted to the outpatient clinic with complaints of dysphagia. Diagnostic workup revealed the presence of an esophageal web. On subsequent colonoscopy, a tumoral lesion was found in the ascending colon. Computed tomography scan illustrated abdominal situs ambiguous with right-sided polysplenia, right-sided stomach, and intestinal nonrotation. Furthermore, a preduodenal portal vein, azygos continuation of the inferior vena cava, and hemiazygos continuation of the left renal vein were observed. After careful assessment of the anatomy, a right hemicolectomy with radical lymphadenectomy was performed. HS consists of a rare and complex situs anomaly, with an abnormal arrangement of the thoracic and/or abdominal organs along the left–right axis. To our knowledge, right hemicolectomy has not been described in patients with HS.

Keywords: Heterotaxy syndrome, polysplenia, preduodenal portal vein, right hemicolectomy


How to cite this article:
Dewulf M, Beckers R, Pletinckx P. Right hemicolectomy in a patient with heterotaxy syndrome. World J Colorectal Surg 2019;8:30-3

How to cite this URL:
Dewulf M, Beckers R, Pletinckx P. Right hemicolectomy in a patient with heterotaxy syndrome. World J Colorectal Surg [serial online] 2019 [cited 2019 Aug 18];8:30-3. Available from: http://www.wjcs.us.com/text.asp?2019/8/1/30/254035


  Introduction Top


In this communication, we present a first description of a right hemicolectomy in a patient with heterotaxy syndrome (HS).


  Case Report Top


A 78-year-old Caucasian male patient presented with rapidly progressive dysphagia. Due to this, a pacemaker implantation for sick sinus syndrome was held.

Initial biochemical results showed an iron-deficiency anemia, with a hemoglobin level of 8.1 g/dl. Further diagnostic workup consisted of gastroscopy and videofluoroscopic swallowing examination. The latter revealed a characteristic esophageal web [Figure 1], and a tentative diagnosis of Plummer–Vinson syndrome was made. After endoscopic treatment with pneumatic dilatation, the dysphagia complaints resolved completely.
Figure 1: Barium swallow esophagram showing esophageal web at the lower cervical level

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Because of persistent iron-deficiency anemia – despite iron infusions – an additional colonoscopy was performed. Hereby, a diagnosis of a tumoral lesion in the ascending colon was made, about 25 cm proximal to the ileocecal valve. Computed tomography (CT) scan of the thorax and abdomen failed to demonstrate distant metastases yet revealed a characteristic anatomy. An abdominal situs ambiguous with right-sided polysplenia, right-sided stomach, and intestinal nonrotation was observed [Figure 2] and [Figure 3]. The liver was grossly situated at the midline. The entire small bowel was located on the right side of the spine, whereas the ileocecal valve and ascending colon were lying entirely in the left hemiabdomen, medial to the descending colon. Furthermore, a preduodenal portal vein (PDPV) [Figure 4], azygos continuation of the inferior vena cava (IVC) [Figure 5], and hemiazygos continuation of the left renal vein were present [Figure 6]. Thoracic CT scan illustrated bilateral bilobed lungs and hyparterial bronchi [Figure 7].
Figure 2: Computed tomography scan revealing characteristic intra-abdominal features of heterotaxy syndrome: right-sided polysplenia (green arrows), right-sided stomach (red arrow), and liver situated at the midline (yellow arrow)

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Figure 3: Reconstructive image of right-sided polysplenia

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Figure 4: Coronal computed tomography image of the preduodenal portal vein

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Figure 5: Azygos continuation of the inferior vena cava (a). Reconstructive image of azygos continuation of the inferior vena cava (b)

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Figure 6: Hemiazygos continuation of the left renal vein

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Figure 7: Sagittal image of thoracic computed tomography scan illustrating bilobed right lung (a). Coronal image showing hyparterial right main bronchus (b)

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Since the diagnosis of adenocarcinoma in the ascending colon was confirmed, a right hemicolectomy was planned.

After extensive preoperative assessment of the anatomy and the main vessels, a laparoscopic approach was initiated. Several intraoperative factors prompted a relatively quick conversion to laparotomy, to be able to perform a safe and radical lymphadenectomy. Mainly due to the prominent PDPV, the short distance between the ascending colon and the PDPV (hence, a short mesocolon), and multiple intra-abdominal adhesions, a laparoscopic procedure was not possible without major compromises.

Subsequently, the right hemicolectomy was performed through a midline laparotomy. A ligation of the ileocolic artery and right colic artery was performed at its origin, and a radical lymphadenectomy along the mesenteric-portal venous axis of the PDPV was completed [Figure 8] and [Figure 9]. The continuity was restored by performing a semi-mechanic isoperistaltic ileocolic anastomosis.
Figure 8: Intraoperative image showing short mesocolon, ligation of the ileocolic (yellow arrow – forceps), and right colic artery (green arrow) at its base. The mesenteric-portal venous axis of the preduodenal portal vein is indicated with the red arrow

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Figure 9: Postoperative images of the specimen and the tumoral lesion (red arrow), ligation of the right colic artery (forceps), and the ileocolic artery (yellow arrow)

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  Discussion Top


HS consists of a rare and complex situs anomaly, with an abnormal arrangement of the thoracic and/or abdominal organs along the left–right axis.[1],[2]

Unlike situs inversus totalis (SIT) – where the anatomic arrangements are a mirror image of the normal situation – HS is characterized by its major variability in presentation.[1],[2] Most authors nowadays advise an individualized description of each patient under the label of “HS.” The terms “polysplenia syndrome” and “asplenia syndrome” should be avoided, as they should be considered as subtypes of HS.[1],[2]

Lacking any pathognomonic finding, the anomalies seen in HS are part of a broad and overlapping spectrum. Conventionally, they have been subdivided into cardiac and extracardiac anomalies.[1],[2],[3]

Despite its low incidence, HS is diagnosed in up to 4.2% of the patients with congenital heart disease. The most frequently reported findings are defects in the systemic and pulmonary venous drainage and the atrioventricular canal.[3] In this case, repeated transthoracic echocardiography revealed no structural heart disease, but he did develop sick sinus syndrome for which a pacemaker implantation was needed at the age of 73 years. Wu et al.[4] reported an increased incidence in sinus node dysfunction in patients with HS, yet the age of onset and the absence of structural cardiac anomalies suggest that this finding may be coincidental and unrelated to the underlying HS.

The term “bilateral right-sidedness” or “right isomerism” in patients with asplenia of “bilateral left-sidedness” or “left isomerism” in patients with polysplenia refers mainly to the findings on the thoracic level. In the first case, both the lungs generally have three lobes, and the main bronchus is located superior to the ipsilateral main pulmonary artery (eparterial bronchus), and in the second case, both the lungs have two lobes and hyparterial bronchi (with the main bronchus inferior to the main pulmonary artery).[1],[2] As illustrated by [Figure 7], the latter is present in this case. In the famous autopsy series of Peoples et al. including 146 patients, only 55% of the polysplenia patients had bilateral bilobed lungs, making it again an inconsistent finding, especially in adult patients with polysplenia.[2],[5]

The abdominal situs anomaly seen in HS is generally referred to as “situs ambiguous.” All possible rotational variations (non, incomplete, and reversed rotations) have been reported in HS, but their incidence remains rather unknown.[2],[6],[7] In a recent patient series, it was found in seven of the eight polysplenia patients.[7] In this case, with all small bowel located on the right side of the spine and the entire colonic arch in the left hemiabdomen, we believe that “nonrotation” is the correct term.

Besides polysplenia, an interruption of the IVC with azygos continuation is the most common variation seen in HS.[1],[2] A PDPV has only been reported in about thirty adult HS patients to date,[8] whereas a hemiazygos continuation of the left renal vein has only been reported anecdotically.[9] All of the forementioned venous anomalies are present in our patient.

To the best of our knowledge, this is the first report of a right hemicolectomy in a patient with HS. There have been several reports of laparoscopic hemicolectomy in patients with situs anomalies, yet all of them were in patients with SIT. Despite an initial laparoscopic approach, a conversion to laparotomy was made to enable a safe and radical lymphadenectomy. We believe that, eventually, a correct oncological resection was performed, with lymphadenectomy along the venous axis and a ligation of the ileocolic and right colic artery at their base, as illustrated by [Figure 8] and [Figure 9].

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: Imaging of the heterotaxy syndrome. Radiographics 1999;19:837-52.  Back to cited text no. 1
    
2.
Tawfik AM, Batouty NM, Zaky MM, Eladalany MA, Elmokadem AH. Polysplenia syndrome: A review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat 2013;35:647-53.  Back to cited text no. 2
    
3.
Degenhardt K, Rychik J. Fetal situs, isomerism, heterotaxy syndrome: Diagnostic evaluation and implication for postnatal management. Curr Treat Options Cardiovasc Med 2016;18:77.  Back to cited text no. 3
    
4.
Wu MH, Wang JK, Lin JL, Lai LP, Lue HC, Hsieh FJ, et al. Cardiac rhythm disturbances in patients with left atrial isomerism. Pacing Clin Electrophysiol 2001;24:1631-8.  Back to cited text no. 4
    
5.
Peoples WM, Moller JH, Edwards JE. Polysplenia: A review of 146 cases. Pediatr Cardiol 1983;4:129-37.  Back to cited text no. 5
    
6.
Ditchfield MR, Hutson JM. Intestinal rotational abnormalities in polysplenia and asplenia syndromes. Pediatr Radiol 1998;28:303-6.  Back to cited text no. 6
    
7.
Fulcher AS, Turner MA. Abdominal manifestations of situs anomalies in adults. Radiographics 2002;22:1439-56.  Back to cited text no. 7
    
8.
Latha G, Kagali NA, Shridhar M, Prasad BS. Preduodenal portal vein in adult with polysplenia syndrome revisited with a case report. Indian J Surg 2014;76:137-42.  Back to cited text no. 8
    
9.
Rameshbabu CS, Gupta KK, Qasim M, Gupta OP. Heterotaxy polysplenia syndrome in an adult with unique vascular anomalies: Case report with review of literature. J Radiol Case Rep 2015;9:22-37.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]



 

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