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REVIEW ARTICLE
Year : 2022  |  Volume : 21  |  Issue : 4  |  Page : 305-308  

Chylous ascites in a case of henoch-schonlein purpura - A case report and literature review


Department of General Surgery, Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra, India

Date of Submission18-Aug-2021
Date of Decision23-Nov-2021
Date of Acceptance08-Jan-2022
Date of Web Publication16-Nov-2022

Correspondence Address:
Joseph Kurian Mundakal
Department of General Surgery, Dr. D. Y. Patil Medical College and Research Centre, Pimpri, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aam.aam_168_21

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   Abstract 


Chylous ascites is a rare form of ascites characterized by the accumulation of lymph fluid in the peritoneal cavity. Henoch-Schonlein purpura (HSP) is a form of vasculitis usually seen in children affecting small vessels. Gastrointestinal (GI) manifestations of HSP are coming to the forefront as a presenting symptom. The presence of a rash usually succeeds the GI manifestations, making diagnosis difficult and leading to unnecessary surgical interventions. Our case shows a 38-year-old female who presented with an acute abdomen followed by an erythematous rash noticed later on, with radiological investigations suggestive of acute appendicitis. Chylous ascites was found as an incidental finding on diagnostic laparoscopy with a healthy appendix.

   Abstract in French 

Résumé
L'ascite chyleuse est une forme rare d'ascite caractérisée par l'accumulation de liquide lymphatique dans la cavité péritonéale. Henoch-Schonlein le purpura (HSP) est une forme de vascularite généralement observée chez les enfants et affectant les petits vaisseaux. Les manifestations gastro-intestinales (GI) de la HSP arrivent au premier plan comme symptôme révélateur. La présence d'une éruption cutanée succède généralement aux manifestations gastro-intestinales, rendant le diagnostic difficile et conduisant à des interventions chirurgicales inutiles. Notre cas montre une femme de 38 ans qui s'est présentée avec un abdomen aigu suivi d'un érythémateux éruption cutanée constatée ultérieurement, avec des investigations radiologiques évocatrices d'une appendicite aiguë. Une ascite chyleuse a été découverte de manière fortuite sur laparoscopie diagnostique avec un appendice sain.

Mots-clés: Abdomen aigu, ascite chyleuse, purpura Henoch-Schonlein

Keywords: Acute abdomen, chylous ascites, Henoch-Schonlein purpura


How to cite this article:
Galam P, Mundakal JK. Chylous ascites in a case of henoch-schonlein purpura - A case report and literature review. Ann Afr Med 2022;21:305-8

How to cite this URL:
Galam P, Mundakal JK. Chylous ascites in a case of henoch-schonlein purpura - A case report and literature review. Ann Afr Med [serial online] 2022 [cited 2022 Dec 10];21:305-8. Available from: https://www.annalsafrmed.org/text.asp?2022/21/4/305/361258




   Introduction Top


Chylous ascites is defined as the extravasation of milky/creamy-appearing peritoneal fluid that is rich in triglycerides. It is caused by the presence of thoracic or intestinal lymph in the abdominal cavity due to disruption in the lymphatic system either due to trauma or obstruction (benign or malignant). The presence of milky fluid with a triglyceride level of more than 200 mg/dl makes the diagnosis of chylous ascites.[1] Asselius first described the lymphatics in a dog's mesentery around 1622, while Morton described it in an 18-month-old child who was diagnosed with disseminated tuberculosis (TB). In the 17th century, trauma arose as the most common cause until later, when other causes such as chronic liver disease came to the forefront.

An incidence of 1 in 20,000 was seen in a university hospital study over 20 years. Although with the advent of aggressive cardiothoracic and abdominal surgeries and longer life span of patients with malignancies, there has been an increase in incident rates.[2] The management of patients with chylous ascites is based on identifying and treating the underlying cause. Nutritional support with a low-fat diet and medium-chain triglyceride supplementation is of paramount importance. Therapeutic paracentesis and diuretics are recommended in patients with underlying cirrhosis.

The most common clinical presentation of chylous ascites is supposed to be increasing painless abdominal distension. Less frequently, postinterventional and posttraumatic chylous ascites can induce an acute abdomen. Although the clinical features and causes of chylous ascites secondary to surgery or trauma are familiar to most clinicians, little is known about the incidence, etiology, and distribution of different clinical symptoms of atraumatic chylous ascites.[3]

Henoch-Schonlein purpura (HSP) is a form of leukocytoclastic vasculitis, which usually affects children under 10 years of age and involves small vessels predominantly. The pathophysiology behind HSP is debatable but is usually self-limiting and harmless. Gastrointestinal (GI) involvement is seen in 50%–75% of patients and is characterized by colicky pain, vomiting, and GI hemorrhage, and these usually precede full-blown skin manifestations, making a diagnosis of acute abdomen tricky at the time. Small-vessel vasculitis of HSP presents with skin rash, colicky abdominal pain, GI hemorrhage, and hematuria.[4]

During what was supposed to be a routine laparoscopic appendectomy, milky-white chylous fluid was seen in subhepatic, pelvic, and subsplenic spaces with lymphatic leak present along the bowel wall of a short segment of ileum, with few necrotic lymph nodes in the omentum. The appendix was normal without any signs of inflammation.


   Case Report Top


A 38-year-old female who was examined with due consent, presented with generalized abdominal pain for 10 days, colicky type, radiating to the back, relieves on proton pump inhibitor intake, gives a history of nausea and vomiting 2–3 episodes/day, non-bilious, non-projectile. The patient gave a history of an erythematous rash over bilateral lower limbs, up to the thigh, for 5 days. History of joint pain with generalized weakness was present. No history of melena or any other bleeding manifestations was present. She was afebrile, and vitals were as follows: pulse rate 86 bpm and her blood pressure 110/80 mmHg. Laboratory investigations were unremarkable including hemogram, liver and renal function tests, and coagulation profiles.

A computed tomographic (CT) scan done showed the appendix with peripheral enhancement with peri-appendiceal fat stranding and enlarged right iliac lymph nodes with no evidence of any collection/ascites. Due to radiological findings not corroborating with clinical findings, a diagnostic laparoscopy was done. Multiple necrotic mesenteric lymph nodes were identified [Figure 1], with milky-white fluid seen in the pelvis, suprahepatic, left and right paracolic gutter, and splenic fossa [Figure 2]. The appendix appeared normal. It was converted into an exploratory laparotomy with lavage and mesenteric lymph node biopsy being taken. An intramural lymphatic leak was observed in a small segment of the distal ileum [Figure 3]. Milky fluid was then sent for culture, microscopy, and lipid profile. With fluid triglycerides at 352 mg/dl and biopsy report was showing small-vessel fibrin deposition in their wall, with associated nuclear debris and neutrophil infiltration. Endothelial proliferation and degeneration were also present, indicative of leukocytoclastic vasculitis. Immunohistochemistry showed increased IgA levels suggestive of HSP. The patient's symptoms resolved with steroids and supportive care which included intravenous fluids during in-patient stay, analgesics, and multivitamins.
Figure 1: Necrotic mesenteric lymph nodes

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Figure 2: Chylous fluid in peritoneal cavity

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Figure 3: Intramural lymphatic leak

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   Literature Review Top


In 1622, Asellius first described the lymphatic system in a dog after observing vessels in the mesentery containing a white milky fluid. Morton, in 1691, performed a paracentesis in an 18-month-old boy with disseminated TB and noted the presence of chylous ascites. Trauma was considered the most common cause in the 17th century, whereas, with increase survival rates, malignancies and cirrhosis have become more prominent causes in modern times.[5]

A large university study by Browse et al., over 20 years, saw only 1 in 20,000 admissions with an increasing trend owing to more cardiothoracic and abdominal surgeries with higher survival rates of cancer patients.[2]

Chylous ascites has been defined as the extravasation of milky or creamy appearing peritoneal fluid rich in triglycerides, caused by the presence of thoracic or intestinal lymph in the abdominal cavity.

A review of literature by Steinemann et al. of atraumatic chylous ascites identified 41 different etiologies of atraumatic ascites. Lymphatic anomalies were the most frequent cause (32%). They were more frequent in the pediatric (84%), compared with the adult population (8%). The most frequent anomaly was lymphangiectasia, diagnosed in 3% of adults and 26% of children and adolescents. The second most frequent etiology was a malignant disease, which was responsible for 17% of patients with atraumatic chylous ascites.[6]

This group comprised various cancers, sarcomas, neuroendocrine tumors, lymphomas, and leukemias. Malignancy was found almost exclusively in adults (25% vs. 2% in children); Finally, in adults, liver cirrhosis (11%) and infections with mycobacteria (10%) were detected as important underlying causes as well.

HSP is a form of IgA vasculitis with immune deposition, resulting in necrosis of the wall of small- and medium-sized arteries. The GI tract is usually free of damage due to its medium-sized vessels involved in its redundant circulation. The small intestinal villi contain loops of vessels with end-capillaries that, when obstructed, resulting in necrosis of the villus tip. The diagnostic criteria for HSP established in 2006 states that palpable purpura (a mandatory criterion) in the presence of at least one of the following:[1] (1) diffuse abdominal pain; (2) any biopsy showing predominant IgA deposition; (3) arthritis or arthralgia; and (4) renal involvement (hematuria and/or proteinuria) is used to establish the diagnosis.[7]

Abdominal pain is associated with 51%–74% of patients, which is characteristically colicky and present around epigastric and periumbilical regions, which usually worsens after meals. The abdomen may be tender and distended or resemble an acute abdomen, resulting in unnecessary surgery.[8]

Among the GI manifestations of HSP, acute appendicitis is rarely seen. Although multiple case reports have been identified where a diagnosis of acute appendicitis was established, some showed that, intraoperatively, a normal appendix was identified or reported upon on histopathology. Case reports by Mohammed and Kim et al. showed cases of HSP that presented as acute appendicitis.[9],[10] Binstadt and Fleegler performed a case series in which a purpuric rash followed three cases of appendicitis postappendectomy. These studies show a correlation of appendicitis as a presenting feature.[11]

In contrast, a study by Akosa and Ali showed that patients presented with severe colicky abdominal pain mimicking acute appendicitis, but a normal appendix was removed in each case. However, the lymph nodes biopsied at the time revealed follicular granulomas, follicular microabscesses, and leukocytoclastic vasculitis. Immunohistochemical studies revealed deposition of IgA, IgG, and C3 in the vessel walls, the finding of IgA supporting a diagnosis of HSP.[12]


   Discussion Top


Chylous ascites is a rare form of ascites generally associated with poor outcomes and occurs due to a source of lymphatic obstruction or leak causing chylous effusion into the peritoneal or retroperitoneal cavities. The most common cause is any neoplasm obstructing lymphatics followed by trauma. Typically, chyle appears as milky, turbid appearance with a triglyceride level of >200 mg/dl. CT abdomen can be used to identify pathological intra-abdominal lymph nodes and masses, with a biopsy of the nodes showing leukocytoclastic vasculitis.

HSP is a form of systemic vasculitis characterized by predominantly IgA vascular wall deposits typically involving small vessels in skin, gut, and glomeruli and associated with purpura colic, hematuria, and arthralgia or arthritis. HSP can present with GI symptoms of colicky abdominal pain, nausea, vomiting, malena, or hematuria. Abdominal symptoms usually precede the development of purpuric rash. Intra-abdominal manifestations occur due to GI submucosal and subserosal hemorrhage and edema. Vasculitic involvement of the ileum or ascending colon may mimic acute appendicitis and lead to an unnecessary appendectomy. As HSP involves intramural hemorrhages confined to the mucosa and submucosa, the development of complications such as intussusception, hemorrhages, perforation and stricture is less common, and most cases resolve with conservative management.

In such cases, the presentation of acute abdomen needs to be thoroughly investigated with serial abdominal examination and sonography to avoid untoward laparotomies and surgical interventions.

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.
Frick E, Schölmerich J. Etiology, diagnosis, and management of non- cirrhotic ascites. Ascites and Renal Dysfunction in Liver Disease 2nd ed. (2005). 294-302.[Doi: 10.1002/9780470987476 ch24].  Back to cited text no. 1
    
2.
Browse NL, Wilson NM, Russo F, al-Hassan H, Allen DR. Aetiology and treatment of chylous ascites. Br J Surg 1992;79:1145-50.  Back to cited text no. 2
    
3.
Bhardwaj R, Vaziri H, Gautam A, Ballesteros E, Karimeddini D, Wu GY. Chylous ascites: A review of pathogenesis, diagnosis and treatment. J Clin Transl Hepatol 2018;6:105-13.  Back to cited text no. 3
    
4.
Ebert EC. Gastrointestinal manifestations of Henoch-Schonlein purpura. Dig Dis Sci 2008;53:2011-9.  Back to cited text no. 4
    
5.
Aalami OO, Allen DB, Organ CH Jr. Chylous ascites: A collective review. Surgery 2000;128:761-78.  Back to cited text no. 5
    
6.
Steinemann DC, Dindo D, Clavien PA, Nocito A. Atraumatic chylous ascites: Systematic review on symptoms and causes. J Am Coll Surg 2011;212:899-905.e1.  Back to cited text no. 6
    
7.
Ozen S, Ruperto N, Dillon MJ, Bagga A, Barron K, Davin JC, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006;65:936-41.  Back to cited text no. 7
    
8.
Chang WL, Yang YH, Lin YT, Chiang BL. Gastrointestinal manifestations in Henoch-Schönlein purpura: A review of 261 patients. Acta Paediatr 2004;93:1427-31.  Back to cited text no. 8
    
9.
Kim CJ, Chung HY, Kim SY, Kim YO, Ryu SY, Kim JC, et al. Acute appendicitis in Henoch-Schönlein purpura: A case report. J Korean Med Sci 2005;20:899-900.  Back to cited text no. 9
    
10.
Mohammed R. Acute appendicitis: A complication of Henoch-Schönlein purpura. J R Coll Surg Edinb 1982;27:367.  Back to cited text no. 10
    
11.
Binstadt BA, Fleegler EW. Perforated appendicitis in a child with Henoch-Schönlein purpura. J Pediatr Surg 2005;40:E24-7.  Back to cited text no. 11
    
12.
Akosa AB, Ali MH. Lymph node pathology in Henoch-Schönlein purpura. Histopathology 1989;15:297-301.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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