CASE REPORT Year : 2023  |  Volume : 14  |  Issue : 1  |  Page : 45-46

A focal submucosal lipomatosis: An extremely rare cause of appendicitis

Shirish Sahebrao Chandanwale, Payal Patel, Anubhaw Verma, Nirali Patel
Department of Pathology, Dr. D. Y. Patil Medical College, Pune, Maharashtra, India

Date of Submission27-Aug-2022Date of Decision19-Oct-2022Date of Acceptance30-Oct-2022Date of Web Publication31-Jan-2023

Correspondence Address:
Dr. Shirish Sahebrao Chandanwale
75/1 + 2/1, Krishna Apartment, New Sangvi, Pune - 411 027, Maharashtra
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injms.injms_107_22

Gastrointestinal lipomatosis is less frequent condition and it differs from lipoma due to the lack of capsule. The most common location of gastrointestinal lipomatosis is colon, followed by the ileum and jejunum. Isolated lipomatosis of the appendix is rare. It can be diffuse, asymmetric, or focal. In the localized form, only few patients present with symptoms. Obstruction of the lumen of the appendix is thought to be the primary pathology of appendicitis. Various pathologies such as hard fecal mass, stones, lymphoid hyperplasia, and neoplasia have been implicated. Obstruction of the appendiceal lumen due to isolated submucosal lipomatosis of the appendix is extremely rare. It can be one of the causes of appendicitis. Although radiological modalities such as ultrasonography, computed tomography scan, and magnetic resonance imaging are used for diagnostic workups, definitive diagnosis requires histopathological examination.

Keywords: Appendicitis, lipomatosis, obstruction, submucosa

How to cite this article:
Chandanwale SS, Patel P, Verma A, Patel N. A focal submucosal lipomatosis: An extremely rare cause of appendicitis. Indian J Med Spec 2023;14:45-6
How to cite this URL:
Chandanwale SS, Patel P, Verma A, Patel N. A focal submucosal lipomatosis: An extremely rare cause of appendicitis. Indian J Med Spec [serial online] 2023 [cited 2023 Feb 15];14:45-6. Available from: http://www.ijms.in/text.asp?2023/14/1/45/368623   Introduction 

Appendicitis typically presents acutely within 24 h of onset but can also present as a more chronic condition. Acute appendicitis is one of the most common indications for abdominal surgeries worldwide. Obstruction of the lumen of the appendix is thought to be the primary pathology of appendicitis. Various pathologies such as hard fecal mass, stones, lymphoid hyperplasia, and neoplasia have been implicated.[1],[2] Often the exact etiology of acute appendicitis in many cases remains unknown.

Gastrointestinal lipomatosis is less frequent condition and the most common location is colon.[3],[4] Isolated lipomatosis of the appendix is extremely rare. We came across with handful of reports in Indian and Western literature.[5],[6] Isolated focal submucosal lipomatosis of the appendix as a presenting symptom of acute abdomen is extremely rare. We report one such case of a 25-year-old male who presented with acute abdomen.

  Case Report 

A 25-year-old male was admitted to one of the surgical units with the complaint of pain in the right lower quadrant of the abdomen which started gradually and increased in severity. There was no history of nausea, vomiting, diarrhea, or fever. Vital signs were within normal limits. Physical examination of the abdomen revealed a soft abdomen with tenderness in the right lower quadrant.

Ultrasonography (USG) of the abdomen revealed mild probe tenderness in the right lower quadrant of the abdomen and stranding of periappendicular fat. A diagnosis of appendicitis was suspected. Computed tomography (CT) scan of the abdomen and pelvis revealed the appendix in subcecal position with maximum wall-to-wall diameter of 11 mm and homogeneous enhancement of the wall and periapendicular fat stranding. No other pathology was detected in the abdomen and pelvis. A diagnosis of appendicitis was made. Laboratory tests revealed mild elevated total white blood cell (WBC) count (13,200/mm3) and C-reactive protein (CRP) (11.2 mg/L).

Laparoscopic appendicectomy was performed. Specimen of the appendix measuring 7 cm was received for histopathological examination. On the cut section, a small lumen was noted in the body of the appendix. In the tail region, the lumen was obliterated and the wall was thick which showed yellow discoloration [[Figure 1]a yellow arrow]. There was no hard fecal mass or stone or neoplasm grossly. Multiple sections taken were formalin fixed and paraffin processed. The 3–5 thick micron sections were cut and stained with hematoxylin and eosin. Histopathological examination showed focal submucosal deposits of mature adipose tissue [Figure 1]b. There was a sparse neutrophilic inflammatory infiltrate of muscularis propria. A final histopathology diagnosis of submucosal lipomatosis of the appendix with acute appendicitis was given.

Figure 1: (a) Appendix shows obliterated lumen in the tail of the appendix (yellow arrow) and thick wall, (b) histopathology section shows mature adipose tissue in the submucosa

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  Discussion 

Appendicitis can develop at any age but it is more frequent in the second and third decades of life. Often the exact etiology of appendicitis is not known in many cases. Obstruction of the lumen with hard fecal mass, stones, lymphoid hyperplasia, and neoplasms is found in many cases. Obstruction leads to an increase in the intraluminal and intramural pressure which results in small vessel occlusion and lymphatic stasis. This leads to ischemia and inflammation. Bacterial overgrowth in obstructed lumen contributes to inflammation and necrosis.[1]

Appendicitis typically presents as an initial generalized or periumbilical pain which subsequently localizes to the right lower quadrant of the abdomen. It may be accompanied by anorexia, nausea, vomiting, and fever. Similar types of pain can be caused by diverticulitis, distal ureteral lithiasis, Crohn's disease, tubo-ovarian inflammatory diseases, and ectopic pregnancy.[7]

Appendicitis is traditionally a clinical diagnosis; however, several imaging modalities are used for definitive diagnosis. USG is less sensitive and specific. CT scan of the abdomen and pelvis is >95% accurate. Similar observations were made in our study.[6] A combination of total and differential WBC count and CRP results have a specificity of 98% for the exclusion of acute appendicitis.[8] Similarly, both increasing levels of CRP and WBC count correlate with significant increase in the likelihood of complicated appendicitis. In our patient, both were mildly elevated.

Gastrointestinal lipomatosis is characterized by infiltration of mature adipose tissue in the submucosal layer. It differs from lipoma due to the lack of capsule.[1],[6] The most common location of gastrointestinal lipomatosis is colon, followed by the ileum and jejunum. It can be diffuse, asymmetric, or focal. In the localized form, only few patients present with symptoms.[3] In our case, focal submucosal lipomatosis was seen.

The pathogenesis of appendiceal lipomatosis remains unclear. However, it is suggested that chronic inflammation of the appendix could play a role.[1] In our case, a possible cause of appendicitis was obstruction of the lumen of the appendix due to the deposition of mature adipose tissue in the submucosa. We report this case due to the rarity of the disease and focal nature of the lesion. After the surgery, the patient achieved early recovery and no complications were registered.

  Conclusion 

Gastrointestinal lipomatosis is an uncommon lesion and focal submucosal lipomatosis is extremely rare. Only few patients present with symptoms. Radiological modalities such as USG, CT scan, and magnetic resonance imaging should be used for diagnostic workups. However pathogenesis of submucosal lipomatosis is not clear. Obstruction of the lumen of the appendix is the primary pathology of appendicitis. Various pathologies such as hard fecal mass, stones, lymphoid hyperplasia, and neoplasia have been implicated. Submucosal lipomatosis can also be one of the causes of obstruction. Definitive diagnosis requires histopathological examination.

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Conflicts of interest

There are no conflicts of interest.

 

  References 
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    3.Pei MW, Hu MR, Chen WB, Qin C. Diagnosis and treatment of duodenal lipoma: A systematic review and a case report. J Clin Diagn Res 2017;11:PE01-5.  
    4.Dajti G, Vagliasindi A, Bosi S, Guerra E, Zanzi F, Raulli GD. An unusual clinical presentation of colonic lipomatosis: A case report. Int J Surg Case Rep 2020;75:273-5.  
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    6.Sanches LP, Rahal Júnior A, Falsarella PM, Carvalho VO, Valle LG, Francisco Neto MJ, et al. Caecal appendix lipomatosis in a pregnant patient mimicking acute appendicitis. Einstein (Sao Paulo) 2020;18:eRC5415.  
    7.Hoeffel C, Crema MD, Belkacem A, Azizi L, Lewin M, Arrivé L, et al. Multi-detector row CT: Spectrum of diseases involving the ileocecal area. Radiographics 2006;26:1373-90.  
    8.Withers AS, Grieve A, Loveland JA. Correlation of white cell count and CRP in acute appendicitis in paediatric patients. S Afr J Surg 2019;57:40  
    
  [Figure 1]