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 Table of Contents  
CASE REPORT
Year : 2023  |  Volume : 10  |  Issue : 1  |  Page : 39-42

Necrotizing fasciitis complicating perforated Meckel's diverticulum in a patient with acute lymphoblastic leukemia relapse: A case report


1 Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Pediatrics, SMS Medical College, Jaipur, Rajasthan, India
3 Department of Radiodiagnosis, SMS Medical College, Jaipur, Rajasthan, India
4 Department of Pathology, SMS Medical College, Jaipur, Rajasthan, India
5 Department of Plastic Surgery, SMS Medical College, Jaipur, Rajasthan, India

Date of Submission02-Nov-2022
Date of Decision06-Dec-2022
Date of Acceptance12-Dec-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Dr. Rahul Gupta
Associate Professor, Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcc.jpcc_87_22

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  Abstract 


The occurrence of necrotizing fasciitis in children with acute lymphoblastic leukemia (ALL) has been reported with malignancies during granulocytopenic episodes from chemotherapy. Perforation of Meckel's diverticulum in children with leukemia is very rare. Only one case has been reported in the literature till now. A 10-year-old male presented with acute abdomen and was diagnosed with perforation peritonitis due to a perforated Meckel diverticulum receiving chemotherapy for relapse of B-cell ALL. In addition, the patient was a known case of celiac disease. Intraoperatively, due to the hemodynamic instability and thrombocytopenia, it was decided to perform a resection of the ileum bearing the pathology with a double-barrel ileostomy. He developed necrotizing fasciitis of the anterior abdominal wall due to Group A streptococci and overwhelming Gram-positive sepsis. The patient was managed comprehensively with multiple platelets concentrates, fasciotomy of the abdominal wall, and multiple debridements under the cover of broad-spectrum antibiotics. Histopathology of the resected Meckel's diverticulum showed ectopic gastric mucosa. Two months later, split-thickness skin grafting was performed. Early diagnosis and prompt surgical management may prevent mortality associated with both the delayed presentation of bowel perforation and necrotizing fasciitis in ALL patients.

Keywords: Acute lymphoblastic leukemia, celiac disease, Meckel's diverticulum, necrotizing fasciitis, perforation, relapse


How to cite this article:
Gupta R, Chaudhary R, Mathur A, Mangal K, Shukla AK, Bhandari A, Jain RK, Garg K. Necrotizing fasciitis complicating perforated Meckel's diverticulum in a patient with acute lymphoblastic leukemia relapse: A case report. J Pediatr Crit Care 2023;10:39-42

How to cite this URL:
Gupta R, Chaudhary R, Mathur A, Mangal K, Shukla AK, Bhandari A, Jain RK, Garg K. Necrotizing fasciitis complicating perforated Meckel's diverticulum in a patient with acute lymphoblastic leukemia relapse: A case report. J Pediatr Crit Care [serial online] 2023 [cited 2023 Feb 3];10:39-42. Available from: http://www.jpcc.org.in/text.asp?2023/10/1/39/368236




  Introduction Top


The occurrence of necrotizing fasciitis in children with acute lymphoblastic leukemia (ALL) has been reported with malignancies during granulocytopenic episodes from chemotherapy.[1] Furthermore, gastrointestinal (GI) manifestations of leukemia occur in up to one-fourth of patients at autopsy, generally during relapse.[2] Perforation of Meckel's diverticulum is very rare in patients of ALL.[3] This is the second case in the literature. Furthermore, we present a multidisciplinary comprehensive management of necrotizing fasciitis complicating perforated Meckel's diverticulum receiving chemotherapy for relapse of B-cell ALL.


  Case Report Top


A 10-year-old male child was receiving ALL-REZ-BFM-2002 chemotherapy protocol for early testicular relapse of B-cell ALL. In addition, the patient was a known case of celiac disease. He initially developed mild dull aching abdominal pain and constipation on day 4 of the chemotherapy cycle, which was managed with proton-pump inhibitors, intravenous antibiotics, and laxatives. The pain episodes and intensity worsened rapidly within 48 h along with the presence of bilious vomiting and fever spikes. On examination, the child was hemodynamically unstable with a pulse rate – 118/min and blood pressure (BP) – 98/64 (associated with postural fall in BP). Abdominal examination revealed signs of peritonitis with distension, tenderness, and muscle guarding. Baseline blood investigations showed anemia with hemoglobin – 9.9 g%, leukocytopenia (total leukocyte count – 1140/mm3), thrombocytopenia (platelets – 33,000/mm3), hypokalemia (serum K+ – 2.9 mEq/mL), and qualitative C-reactive protein was positive. An ultrasound abdomen revealed the presence of hepatosplenomegaly, dilated bowel loops with echogenic ascites, and septations. Abdominal radiographs were suggestive of intestinal perforation [Figure 1]. Contrast-enhanced computed tomography abdomen suggested the presence of Meckel diverticulum with ascites [Figure 1]. A provisional diagnosis of frank perforation peritonitis due to perforated Meckel diverticulum was made. Intraoperatively, there was gross peritoneal contamination with turbid fluid and a perforated Meckel's diverticulum near the tip. Peritoneal toileting and resection of the ileum bearing the pathology were performed. As there was hemodynamic instability, the presence of underlying thrombocytopenia, and frank sepsis, it was decided to perform a double-barrel ileostomy.
Figure 1: Abdominal radiograph (a) shows multiple air-fluid levels with gas shadows under the domes of the diaphragm (green arrows); abdominal CECT images (b and c) show Meckel's diverticulum (red arrows) with the presence of ascites. CECT: Contrast-enhanced computed tomography

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On the 1st postoperative day, the patient developed erythematous, bullous, and mottled, edematous changes of the skin with extensive necrosis of the right side of the abdominal wall extending from the right hypochondrium (one inch above the laparotomy incision) to the right chest wall, associated with severe local pain. These findings were consistent with necrotizing fasciitis of the anterior abdominal wall. Blood cultures revealed sepsis due to Group A streptococci. The patient underwent aggressive surgical management with fasciotomy, surgical excision, multiple wound debridement, and dressings [Figure 2]. Broad-spectrum antibiotics, including vancomycin, meropenem, and metronidazole, were continued for 3 weeks until recovery of the leukocyte count. The patient was managed comprehensively with ionotropic support (dopamine at - 5–15 μg/kg/min) for 48 h, multiple platelets concentrate, fresh frozen plasma, and blood transfusions. Toxic shock syndrome was kept a differential diagnosis in view of hemodynamic instability. A total of three doses of granulocyte colony-stimulating factor (G-CSF; Filgrastim) were given at the dose of 5 μg/kg for granulocytopenia. Meanwhile, the next chemotherapy cycle was withheld.
Figure 2: Postoperative photographs (including an inset image) show (a) a raw abdominal wall with granulation tissue cranial to the diverting stoma following necrotizing fasciitis with an ileostomy bag; (b) good uptake of the partial-thickness skin graft over the wound

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Histopathology of the resected Meckel's diverticulum showed the presence of ectopic gastric mucosa [Figure 3]. Pathologic examination of the debrided tissue showed widespread necrosis at the fascial level compatible with necrotizing fasciitis. Wound cultures also yielded Group A streptococci and Pseudomonas aeruginosa. Antibiotics were guided by an antibiogram (sensitivity to meropenem); anaerobic cultures were negative. Two months later, partial-thickness skin grafting was performed [Figure 2]. The patient is having hematological remission and gaining weight on follow-up with the pediatric oncology division. The restoration of bowel continuity is contemplated after 3 months.
Figure 3: Microphotographs show (a) H and E-stained (×10) foci of the ectopic gastric mucosa (metaplasia) in the resected specimen of Meckel's diverticulum (red arrow); (b) H- and E-stained (×40) foci of the ectopic gastric mucosa (metaplasia) in Meckel's diverticulum

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  Discussion Top


Leukemia is the leading cause of death from malignancy in the pediatric age group.[2],[4] GI complications of leukemia are rare, and preterminal events are present in one-fourth of the patients.[2] GI complications are generally seen during the relapse phase of leukemia.[2] These complications are categorized into three types: (a) leukemic invasion of the bowel and related structures causing intestinal obstructions, hepatosplenomegaly, bleeding, ulceration, and portal hypertension. (b) altered immune system with profound neutropenia causing increased susceptibility to GI infections (peritonitis, appendicitis, cecal perforation, and liver abscess) and opportunistic infections (typhlitis). The opportunistic infections seen in leukemia patients include Pseudomonas, Candida, and Cytomegalovirus.[2] (c) toxicity of antileukemic therapy causing gastritis, mucositis, and constipation.[2],[4] Neutropenic enterocolitis, a necrotizing sequence involving the cecum, ascending colon, and terminal ileum with ischemic necrosis leading to perforation peritonitis may be present due to neutropenia and intensive chemotherapy.[2] These complications are increasingly seen due to a conglomeration of factors such as better critical care, improved antileukemic therapy, and increased patient lifespan.[4]

Meckel's diverticulum is an omphalomesenteric duct remnant, a true diverticulum with all three layers of the small intestine. It is the most common GI tract malformation (2% of the population).[1] Although most are asymptomatic, the estimated lifetime risk of illness is 4%.[3] It presents with painless bright red rectal bleeding, bowel obstruction, small bowel volvulus, or intussusception. Rarely, perforation may be present, resulting in peritonitis and Gram-negative sepsis.

Ectopic gastric mucosa (as observed in our case) is found in 1/3rd to 1/2nd of the resected Meckel's diverticula and has a higher incidence in those presenting with complications as compared to the intraoperative incidental finding. In our case, the perforation of Meckel's diverticulum was possibly due to a combination of reasons: (a) peptic ulcer disease in the lining of the Meckel's diverticula resulting from steroids used during therapy and (b) mucosal injury related to chemotherapy.[3] In our patient, the base of the perforated Meckel's diverticulum was narrow which could have resulted in the inadequate circulation of the secreted acidic fluid from the diverticulum into the alkaline ileal fluid subsequently resulting in a perforation.[3]

Necrotizing fasciitis is an uncommon, severe soft-tissue infection characterized by necrosis of subcutaneous fat and fascia. Despite aggressive therapy, it is associated with severe sepsis and high mortality rates.[5] It is rare in children and has been reported in children during the granulocytopenic phase of induction chemotherapy for ALL. In our case, necrotizing fasciitis precipitated following perforated Meckel's diverticulum during the chemotherapy cycle. In pediatric patients, trunk area involvement is more common than in the extremities. The predisposition factors in pediatric series are local trauma, fresh surgical wound, and rarely, patients with malignancies having granulocytopenic episodes. The causative organisms implicated are a mixture of aerobic and anaerobic bacteria or group A Streptococcus and P. aeruginosa.[1],[5] In our case, due to chemotherapeutic agents, and perforation peritonitis, there was a high possibility of bacteremia from the GI tract to the bloodstream and/or direct inoculation into the abdominal wall with resultant necrotizing fasciitis.

The diagnosis of necrotizing fasciitis in our case was made promptly and it was managed with aggressive surgical debridement, comprehensive wound care, and adjuvant therapy with broad-spectrum antibiotics, platelet transfusions, and G-CSF. Leukocyte response indicative of the production of leukocytes by the bone marrow is associated with a favorable outcome in ALL with necrotizing fasciitis.[1] The administration of G-CSF in neutropenic cancer patients has been associated with a reduction in clinical infections and favorable outcomes.[1] Thus, despite the underlying disease (ALL), chemotherapy, and critical postoperative course following perforation peritonitis, the successful outcome was achieved due to a conglomeration of factors such as a multidisciplinary comprehensive approach and intensive care provided to our patient.

To conclude, a high index of suspicion for GI perforation including Meckel's perforation should be anticipated, especially with symptoms out of proportion to those of mucositis or constipation in a patient with leukemia on chemotherapy. Early diagnosis, prompt surgical management, adjuvant therapy, and multidisciplinary management may prevent mortality associated with both the delayed presentation of bowel perforation and necrotizing fasciitis in ALL patients.

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.

Acknowledgment

We are sincerely thankful to the Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India, for helping in our endeavor

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Jaing TH, Huang CS, Chiu CH, Huang YC, Kong MS, Liu WM. Surgical implications of Pseudomonas aeruginosa necrotizing fasciitis in a child with acute lymphoblastic leukemia. J Pediatr Surg 2001;36:948-50.  Back to cited text no. 1
    
2.
Ebert EC, Hagspiel KD. Gastrointestinal manifestations of leukemia. J Gastroenterol Hepatol 2012;27:458-63.  Back to cited text no. 2
    
3.
Kline RM. Ruptured Meckel diverticulum mimicking mucositis in a patient receiving induction therapy for high-risk acute lymphoblastic leukemia. J Pediatr Hematol Oncol 2007;29:423-4.  Back to cited text no. 3
    
4.
Hunter TB, Bjelland JC. Gastrointestinal complications of leukemia and its treatment. AJR Am J Roentgenol 1984;142:513-8.  Back to cited text no. 4
    
5.
Duncan BW, Adzick NS, deLorimier AA, Longaker MT, Ferrell LD, Zoger S, et al. Necrotizing fasciitis in two children with acute lymphoblastic leukemia. J Pediatr Surg 1992;27:668-71.  Back to cited text no. 5
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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