Cases
A 66-year-old man with a history of hypertension, type 2 diabetes mellitus, coronary, and
CKD was admitted into nephrology for hypokalemia despite worsening renal insufficiency. The
interrogation revealed the notion of abdominal pain dating back a few months without
episodes of diarrhea.
On physical examination, the blood pressure was normal and the patient’s abdomen was soft,
non-tender, and non-distended, with no palpable masses. The diuresis was 1.5 L/24 h.
Results of laboratory testing demonstrated a worsening of the patient’s CKD, with the rate
of creatinine clearance going from 25 to 15 mL/min. They also showed a serum potassium (K+)
level of 2.8 mmol/L, with collapsed kaliuresis, and metabolic acidosis, with bicarbonates at
14 mmol/L.
These findings suggested a lower digestive leak of K+. An abdominopelvic ultrasound was
therefore requested, revealing a thickened aspect of the last ileal loops at the level of
the right iliac fossa.
For further exploration, the patient underwent a computed tomographic scan of the abdomen
and pelvis showing an aspect in favor of AM complicated by gelatinous disease of the
peritoneum ( Figure 1 and 2 ).
Abdominal-Pelvic Scanner: Aspect of Appendicular Mucocele Complicated by Gelatinous Disease of
the Peritoneum
After K+ supplementation, the kalemia corrected quickly and the patient was entrusted to
the surgeons. The exploration of the abdominal cavity found an important effusion of
gelatinous material, which also involved other abdominal organs and an appendicular
neoplasm.
A right hemicolectomy with peritoneal cleansing was performed followed by intraperitoneal
chemotherapy based on 5-fluorouracil since histology showed appendicular mucinous neoplasm
of low grade, extensive tissue, and serous.
During the follow-up, serum potassium remained normal, and the patient regained his
previous renal function. The evolution of the biological data during the observation period
is represented in Table 1 .
Biological Data During the Observation Period of a Patient With Appendicular
Mucocele.
Intro
In patients with chronic kidney disease (CKD), hypokalemia is uncommon. Instead, there is
usually hyperkalemia whose major risk factors include lower estimated glomerular filtration
rate, use of renin-angiotensin-aldosterone system inhibitors, diabetes, older age, and male
gender ( Seliger, 2019 ).
Hypokalemia among CKD patients is often due to extrarenal, mainly digestive, loss of
potassium.
We report the case of a diabetic, old man with advanced chronic renal failure, who
presented with hypokalemia due to gastrointestinal leakage secondary to appendicular
mucocele (AM). AM is a rare and potentially malignant entity linked to dilation of the
appendix and the abnormal accumulation of mucus in its lumen. Its revelation by hypokalemia
as reported herein has never been previously reported to the best of our knowledge.
Discussion
The classification of AM or mucinous tumors of the vermiform appendix includes a spectrum
of neoplastic lesions ranging from benign proliferation, intraluminal, to invasive
adenocarcinomas ( Anania et al.,
2015 ). It is a rare disease that occurs in 0.2% to 0.3% of appendectomies ( Caspi et al., 2004 ).
The AM is asymptomatic in 20% to 25% of cases and the diagnosis is accidental ( Bronzino et al., 2006 ). In 50% to 65%
of cases, there are signs of acute inflammation of the appendix and in 10% to 20% of cases,
intestinal obstruction or palpable mass in the right lower abdominal quadrant are present
( Cerame et al., 1988 ; Ito et al., 2004 ). Melena ( Wolff & Ahmed, 1976 ),
intussusception ( Ohno et al.,
2000 ), infiltration of the bladder ( Tripodi et al., 1995 ), vaginal penetration with
colporragia ( Didolkar & Fanous,
1977 ), and pyonephrosis by compression of the right ureter ( Deture et al., 1976 ) are rarer occurrences.
The discovery of AM in our patient was made during the assessment of hypokalemia. This is
an exceptional mode of revelation for such neoplasm. Indeed, AM can be, as in this case,
complicated by gelatinous disease of the peritoneum, the « pseudomyxoma peritonei », a
condition caused by cancerous cells (mucinous adenocarcinoma) that produce abundant mucin or
gelatinous ascites. The AM would therefore have been responsible in our patient for an
equivalent of the electrolyte depletion syndrome (Mckittrick-Wheelock syndrome). This latter
is a rare and life-threatening disease characterized by the triad of (1) chronic mucous
diarrhea, (2) renal function impairment with hydroelectrolyte imbalance, and (3) a giant
colorectal tumor ( Villanueva et al.,
2022 ). The mechanism behind severe electrolyte and body fluid depletion in
Mckittrick-Wheelock syndrome is suggested to involve cyclic adenosine monophosphate (AMP)
and prostaglandin E2 acting as mediators, which increase the loss of fluid and electrolytes
from the plasma to the intestinal lumen ( Steven et al., 1981 ). Our patient didn’t present
diarrhea, but the abundant mucosecretion would be responsible for hypokalemia due to
digestive loss.
Most of the reported cases of pseudomyxoma peritonei involve women of reproductive age
(with a history of endometriosis, abdominal surgery, or pelvic inflammatory disease) ( Anania et al., 2015 ). Its occurrence
in an old man, as reported herein, constitutes another particularity of this
observation.
As recommended by most authors in the case of pseudomyxoma peritonei, our patient underwent
a surgical aggressive attitude and intraperitoneal chemotherapy with good evolution ( Anania et al., 2015 ).
Conclusions
AM complicated by gelatinous ascites can cause profound hypokalemia even without diarrhea.
Abundant mucosecretion would even be responsible for severe electrolyte and body fluid
depletion with acute renal failure similar to those observed in Mckittrick-Wheelock
syndrome. Aggressive surgery and intraperitoneal chemotherapy correct these life-threatening
abnormalities.
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