Stercoral perforation represents a rare but lethal complication of constipation and fecal impaction. Since the first report in 1894, fewer than 200 cases have been evaluated prior to our patient[19]. Maurer et al assert that stercoral perforation persists in 1.2% of all emergency colorectal procedures and 3.2% of all colonic perforations[6]. Most cases of colonic perforation are associated with diverticulitis, malignancy, trauma, inflammatory bowel disease, ischemia, infection, or iatrogenic conditions[20]. In a retrospective review, stercoral perforation occurred in 81% of the patients, particularly associated with a long history of chronic constipation and typically in elderly or bedridden patients with a median age of 62 without propensity for either sex.[19] Patients taking antacids, codeine-containing compounds, narcotics, nonsteroidal anti-inflammatory drugs (NSAIDs), major tranquilizers, or tricyclic anti-depressants have been reported to have a higher risk of stercoral perforation[21, 22]. This is almost certainly due to the propensity of these drugs to travel slowly and transiently through the bowel rather than due to a direct effect on the bowel mucosa. However, some have speculated that NSAIDs, by abolishing the cytoprotective effect of prostaglandins, may compromise intestinal integrity[23].
Upon exploring the published English literature (PubMed search from 1965 through August 2020), only two cases of stercoral colitis in patients with SLE have been reported[24, 25], while constipation is demonstrated in 6% of lupus patients[26]. In one case report, a 45-year-old woman with SLE presented with epigastric pain for 12 hours. She also had other significant comorbidities, including sarcoidosis, hypertension, and a previous history of congestive heart failure. The author thought that the cause of stercoral perforation in the sigmoid colon was possibly related to nonsteroidal anti-inflammatory drug (NSAID) use[24]. The other case report addressed a 44-year-old female presenting with stercoral perforation but a complication of SLE attributed to long-term steroid use who had a worse prognosis25. In addition, our patient is the first pediatric-onset lupus patient complicated by stercoral colitis without a disease flare, and several causes were postulated. First, stenosis of the superior mesenteric arteries[26] and persistent inflammatory vessel status in the gut mucosa[27] have been found in lupus, and the imbalance of SLE patients’ bowel mesenteric blood flow or damage to the gut mucosa should be considered to cause ischemic changes in the bowel. Second, gut microbiome alterations in SLE patients may also contribute to gut inflammation[28, 29]. Third, but most importantly, the consequence of neurologic events (NPSLE and brain abscess) may lead to chronic constipation and this catastrophic result. In terms of the multiple manifestations of lupus described above, making a correct diagnosis is difficult among our population.
A variety of causes of abdominal pain may suggest misdiagnosis in lupus patients, such as lupus enteritis, primary peritonitis and chronic lupus peritonitis[30, 31]. Lupus enteritis, which includes mesenteric arteritis or vasculitis, gastrointestinal vasculitis and intestinal vasculitis, is a rare but life-threatening complication of SLE that can present insidiously with postprandial abdominal pain due to chronic mesenteric ischemia[30]. Primary peritonitis secondary to SLE can develop rapidly during a lupus flare and can present with abdominal pain suspicious for abdominal surgery, although symptoms may be masked by concurrent immunosuppressive use30. Chronic lupus peritonitis usually presents with gradually developing ascites that is painless.[32] Given the challenge of misleading diagnosis mentioned previously, stercoral colitis can be easily neglected but requires prompt treatment due to the rapidly progressive course of the disease.
According to Maurer et al.’s definition[6], there are three diagnostic criteria: (1) round or ovoid colonic perforation more than 1 cm in diameter on the antimesenteric side; 2) fecalomas observed in the colon, protruding through the perforation site or lying within the abdominal cavity; and 3) pressure necrosis or ulceration with chronic inflammatory reactions around the perforation site. In addition, mortality has been reported to be 32%-60% regardless of age[5]. In our case analysis, all of our patients met the diagnostic criteria, were alive and had been discharged, and young age may be attributed to the favorable outcomes.
The early diagnosis of stercoral colitis is still a dilemma. According to a previous review, fever, peritoneal signs, elevation of inflammatory markers, metabolic acidosis, and electrolyte imbalance are not noted in all cases[16, 33]. In addition, urinary retention, incontinence or frequency may mimic the early signs of fecal obstruction[34]. Sometimes, a tubular mass can be palpable in the lower left quadrant, as a stool-filled rectosigmoid and rectal exam can disclose stool impacted proximally located in the sigmoid colon[35, 36]. In our review, one of the patients, at the age of two, could not verbalize the abdominal discomfort; thus, it may be difficult to confirm the diagnosis. In our presentation, our lupus patient had urinary tract infection and retention episodes, which should be one of the early signs of chronic constipation. Our review is also in line with a previous study showing that peritoneal signs and inflammatory laboratory data could only be observed in 60% of cases.
Although previous experts have mentioned in their reports that pain during upright abdominal X-ray can offer clues regarding stercoral colitis or perforation, including distention of the colon at the site of impaction, calcified fecaloma, or free air, fecal matter may obscure these findings[6, 37]. The pivotal diagnostic role of radiologic investigation, including CT, has been mentioned for decades, including large fecaloma with distention > 6 cm of the affected colon, wall thickening > 3 mm of the affected colon, pericolonic fat stranding, mucosal discontinuity, free fluid, pericolonic abscess and extraluminal gas bubbles, which suggest perforation[18, 37–39]. In addition, ascites can be found on CT and may be a result of severe systemic inflammation[40]. However, in our review of patients with a young age, remarkable findings on plain abdominal X-ray could not be found in most patients, and only half of the patients displayed perforation on CT images.
According to Wu et al.’s research, 52% of patients can be treated nonoperatively with a bowel regimen[40]. However, if peritonitis occurs, an emergency laparotomy should be arranged for the possible association of stercoral perforation, and stercoral colitis can be diagnosed straightforwardly by pathology and intraoperatively[6]. The usual operation is Hartmann’s procedure with resection of the possible lesion[41]. Intraoperative colonoscopy should be utilized to identify if there are additional stercoral ulcers and to remove a specimen of the altered or dilated colon to prevent another event of colon stercoral perforation[17]. The sigmoid colon and rectum, particularly the rectosigmoid junction, are the parts of the colon most susceptible to stercoral colitis. There are several reasons for enhancing stone-hard fecaloma formation, including the decreasing water content of stool where feces are dry and inspissated, the relatively narrow diameter and the poor blood supply17. The poor blood supply at the rectosigmoid junction is especially defined as Sudeck’s point with insufficient or absent anastomosis between the superior rectal artery and inferior mesenteric artery branch at the watershed area[17, 42]. Additionally, pathology can demonstrate mucosal hemorrhage, submucosal congestion, sharp demarcation without undermining at the ulcer margins and transmural necrosis on the perforated side.[33, 40] In our case analysis, 30% of patients were treated conservatively rather than surgically, but some patients had to receive a second operation. One of these was a patient with Ehlers-Danlos syndrome, a connective tissue disease that may lead to poor wound healing. The other was our lupus patient. Although the lupus condition was stable, bowel inflammation may also have contributed to the poor tissue recovery and the need for a second operation. The presented pathologic specimens also confirmed the diagnosis of stercoral colitis.