Title : A Case Report on Left-sided Appendicitis

First author

Himal Kharel, Tribhuvan University Teaching Hospital, Maharajgunj, POB #1524, Kathmandu,

Nepal

Email: pigzeni@gmail.com

Second author

Druva Shah, Tribhuvan University Teaching Hospital, Maharajgunj, POB #1524, Kathmandu,

Nepal

Email : drshah_dn@live.com

Third author

Namrata Khadka, Tribhuvan University Teaching Hospital, Maharajgunj, POB #1524,

Kathmandu, Nepal

Email : dr.namratakhadka@gmail.com

Fourth author

Zeni Kharel, Medical College, Kolkata 700073, India

Email: zenikharel11@gmail.com

Correspondence

Himal Kharel

Tribhuvan University Teaching Hospital, Maharajgunj, POB #1524, Kathmandu, Nepal

Phone No : +977-9863396053, +977-1-4486607

Email : pigzeni@gmail.com

Keywords : appendectomy; intestinal malrotation; left-sided appendicitis.

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Abstract

Background : Acute appendicitis is a mimicker of wide range of gastrointestinal and

genitourinary pathologies. The diagnosis becomes more challenging when it is associated with

intestinal malrotation. A rare case of acute appendicitis with intestinal malrotation is reported

below.

Case presentation : A 32 year old Hindu male presented with left-sided abdominal pain. CT and

USG of abdomen showed intestinal malrotation with acute appendicitis. Exploratory laparotomy

and appendectomy via midline incision was performed and the patient had no issues on follow-

up.

Conclusion : Given the rarity of acute appendicitis associated with intestinal malrotation, the

guidelines regarding the management is lacking and needs further research.

Introduction

Intestinal malrotation refers to a wide variety of pathological conditions including non-rotation,

incomplete rotation, over rotation and malfixation. The incidence of intestinal malrotation is

around 1 in 6000 live births1. Its manifestation varies greatly in different subjects. Infants

usually present acutely with volvulus whereas adults experience chronic abdominal pain. A vast

majority of adult patients are asymptomatic, and malrotation is usually an incidental finding 2. In

contrast to intestinal malrotation, acute appendicitis is a relatively common disease with overall

lifetime risks for male and female being 8.6 and 6.7%, respectively 3. It is a mimicker of wide

range of gastrointestinal and genitourinary pathologies. The diagnosis becomes more

complicated when it manifests atypically as in intestinal malrotation. A case of intestinal

malrotation with acute appendicitis has been described.

Case presentation

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A 32-year old gentleman without known co-morbidities presented to emergency department of

Tribhuvan University Teaching Hospital, Maharajganj, with complaints of insidious,

progressive, constant pain in his left iliac fossa without radiation, nausea, vomiting and anorexia

for 2 days. There was no history of per rectal bleed, melena, fever or surgical intervention and

his bowel and bladder habits were normal. On examination, the patient was found to be

tachycardic. Abdominal examination revealed tenderness, rebound tenderness, guarding in left

iliac fossa with bowel sounds and normal digital rectal examination.

Routine investigations were normal except for leukocytosis (11,800 with 90% neutrophils).

USG showed non-compressible, blind ending, tubular structure with surrounding hyperechoic

mesentery and fecolith with probe tenderness in left iliac fossa without any free fluid (Figure 1).

The chest Xray showed no abnormalities.Contrast enhanced computerised tomography (CECT)

of abdomen showed large bowel predominantly on the left side and small bowel predominantly

on the right side. Superior mesenteric artery (SMA) and superior mesenteric vein (SMV) relation

was inverted with SMA on the right and SMV on the left side. Duodenum was absent in between

the SMA and aorta and was entirely on the right side (Figure 2). Appendix arising from cecum

was noted in the left iliac fossa (LIF) with thickened wall and periappendiceal fat stranding with

two appendiculoliths of 97 and 65 mm2 size (Figure 3 and 4).

Thus, the diagnosis of acute appendicitis with complete nonrotation of midgut was made.
Due to lack of surgeon's experience in performing laparoscopic surgery, relatively challenging
anatomy and anticipation of bands which may have been missed by imaging, laparoscopic
procedure was not used and surgery via midline incision was deemed appropriate .

The intra operative findings were inflamed appendix with appendiculolith with healthy base

arising from cecum which was fixed in left lower quadrant with minimal free fluid. Duodeno-

jejunal flexure with whole small bowel was seen on right side and the large bowel was seen on

left side (Figure 5). The postoperative period was uneventful and the diagnosis was correlated

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with histopathological examination of appendix. Patient didn't have any issues during first follow

up visit. Patient was lost to further follow up which was later found out to be due to geographical

inaccessibility .The timeline is presented in Table 1.

Discussion

Embryological development of the gut is a complex process in which tubular structure undergoes

rotation and fixation. The structure forms from endoderm lined yolk sac as a result of

cephalocaudal and lateral folding. It is divided into cephalic foregut, caudal hindgut and midgut.

The midgut is temporarily connected to yolk sac. Due to rapid elongation of midgut, rapid

growth and expansion of liver, and relatively small abdominal cavity, physiological umbilical

herniation occurs at around the fifth to tenth week of development. In 10–11 weeks, the

regression of mesonephric kidney, reduced growth of liver and expansion of abdominal cavity

leads to retraction of herniated loops .4 The process of fixation starts from the end of return to

shortly after birth. There is total of 270° counterclockwise rotation of midgut around the

superior mesenteric artery axis with 90° when the midgut herniates out and 180° when the

midgut reduces. If this process fails to occur normally, the result is midgut malrotation.

Malrotation includes under-rotation, over-rotation, reverse rotation and malfixation of intestine.

Malrotation is classified based on the timeframe in which it occurs5.

Acute appendicitis is a fairly common condition 3 caused due to obstruction of lumen and

subsequent bacterial infection. It has a large list differential diagnosis. It can co-exist with

midgut malrotation which can sometimes lead to a confusing clinical picture like in this case. A

significant minority of left sided appendicitis leads to presentation similar to that of right sided

appendicitis with pain on right iliac fossa. 6

Midgut malrotation is associated with heterotaxy syndromes, other anomalies like annular

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pancreas, jejunal stenosis, duodenal stenosis, Hirshprung's disease, congenital diaphragmatic

hernia, imperforate anus, Meckel's diverticulum, biliary atresia and omphalocele. It is especially

important to obtain Chest Xray to rule out situs inversus totalis which may be confused with

intestinal malrotation. More than two-thirds of the left sided appendicitis are due to situs inversus

totalis rather than intestinal malrotation.6

The incidence of midgut volvulus in symptomatic malrotation was 42.1% in the neonatal

period, and 50% beyond the neonatal period7. Midgut volvulus presents as abdominal pain and

bilious vomiting. But this is less common than more chronic presentations of abdominal pain,

failure to thrive and gastroesophageal reflux8.

There are different methods to diagnose midgut malrotation with appendicitis which includes

abdominal X-ray, Upper gastrointestinal (UGI) contrast studies, USG abdomen and CECT

abdomen. Abdominal X-ray shows paucity of small bowel air or may be completely normal. UGI

contrast studies shows abnormal position of duodenojejunal junction which is normally situated

to the left of the left sided pedicles of vertebral bodies. The sensitivity of the upper GI series for

the diagnosis of malrotation has been reported as 95% 9. USG is considered to be the best initial

imaging modality especially for children. USG may show inversion of SMA and SMV

relationship with blind non-compressible aperistaltic tube on the leftside. CECT abdomen may

also show inversion of SMA and SMV 10 along with large bowel predominantly on left ,small

bowel predominantly on right side and concomitant feature of acute appendicitis. Abnormalities

of the SMA and SMV relationship is not entirely diagnostic of intestinal malrotation. USG is

considered to be the best imaging modality in children.

When it presents as volvulus, Ladds procedure is generally performed with derotation of gut,

broadening of mesenteric base, division of Ladds bands, appendicectomy, fixing small bowel on

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the right side and large bowel on the left side. It can be performed as open or laparoscopic

surgery.

If it is found incidentally either in asymptomatic patients or with other intraabdominal

pathology like acute appendicitis then Ladds procedure becomes optional. Laparoscopic

appendicectomy is considered to be gold standard of treatment of left sided appendicitis. But

unlike open procedures, laparoscopic procedures have a steep learning curve and are not well

established in low resource settings like ours. Some recommend only correction of intestinal

pathology without Ladds procedure as the risk of complications following Ladds procedure

outweighs the risk of developing volvulus. While others recommend a more aggressive

approach to treat the asymptomatic malrotation as the consequences of volvulus are usually

disastrous including short gut syndrome and perforation peritonitis. But there is a lack of quality

data to guide the management of such patients.11

Conclusion

Left-sided appendicitis is a rare condition. It may present with features of diverticulitis. At

present, consensus among experts regarding the management of incidental malrotation is lacking.

List of abbreviations

CT = Computerised tomography

USG = Ultrasonography

CECT = Contrast Enhanced computerised tomography

SMA = Superior Mesenteric Artery

SMV = Superior Mesenteric Vein

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 LIF = Left illiac fossa

UGI = Upper Gastrointestinal

Declaration
 Ethics approval and consent to participate
Need for ethical approval waived. Consent from the patient deemed to be enough.
 Consent for publication
Written informed consent was obtained from the patient for publication of this case report and
any accompanying images. A copy of the written consent is available for review by the Editor-
in-Chief of this journal.
 Availability of data and material
Data mentioned in the case report are available to the reviewers if required.
 Competing interests
The authors declare that they have no competing interests
 Funding
None
 Authors' contributions
HK gathered the data and wrote the parts of discussion section. DS and NK performed the
surgery and proof read the manuscript. ZK also wrote part of discussion.
 Acknowledgements
None
References

1. Holcomb GW, Murphy JD, Ostlie DJ. Ashcraft's Pediatric Surgery, 6th ed. US: Elsevier

Health Sciences; 2014. Chapter 31, Malrotation; p. 430-438.

2. Nehra D, Goldstein AM. Intestinal malrotation: varied clinical presentation from infancy

through adulthood. Surgery. 2011 Mar 1;149(3):386-93. DOI:10.1016/j.surg.2010.07.004

3. Addiss DG, Shaffer N, Fowler BS, Tauxe RV. The epidemiology of appendicitis and

appendectomy in the United States. Am J Epidemiol. 1990 Nov 1;132(5):910-25.

PMID:2239906

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4. Sadler TW. Langman's Medical Embryology, 12th ed. US: Lippincott Williams & Wilkins;

2011. p. 225.

5. Stringer DA, Jamieson D. Small bowel. In: Stringer DA, Babyn PS, editors. Pediatric

gastrointestinal imaging and Intervention, 2nd ed. London: B.C. Decker Inc; 2000. p. 311–

475.

6. Akbulut S, Ulku A, Senol A, et al. Left-sided appendicitis: review of 95 published cases and a case

report. World J Gastroenterol. 2010;16: 5598–5602

7. Lin JN, Lou CC, Wang KL. Intestinal malrotation and midgut volvulus: a 15-year review. J

Formos Med Assoc. 1995 Apr;94(4):178-81. PMID:7606179

8. Arthur F, Carachi R. Malrotation in infancy and childhood. Scott Med J. 2016 May;61(2):69-

73. DOI:10.1177/0036933016649870

9. Torres AM, Ziegler MM. Malrotation of the intestine. World J Surg. 1993 May 1;17(3):326-

31. PMID: 8337878

10. Nichols DM, Li DK. Superior mesenteric vein rotation: a CT sign of midgut malrotation. Am

J Roentgenol. 1983; 141:707-708. doi: 10.2214/ajr.141.4.707

11. Graziano K, Islam S, Dasgupta R, Lopez ME, Austin M, Chen LE, et al. Asymptomatic

malrotation: diagnosis and surgical management: an American Pediatric Surgical Association

outcomes and evidence based practice committee systematic review. J Pediatr Surg. 2015 Oct

1;50(10):1783-90. doi:10.1016/j.jpedsurg.2015.06.019

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