A 17-year-old previously healthy, fully vaccinated girl presents with 2 weeks of fatigue following an initial 2-week period of fever, diarrhea, and vomiting. Her initial illness began 4 weeks before presentation with acute onset generalized abdominal pain, nonbloody/nonbilious vomiting, large-volume watery diarrhea, and subjective fevers. Following the resolution of her vomiting and diarrhea, she experienced continued abdominal pain as well as fatigue, anorexia, chills, and night sweats. Her subjective fevers persisted intermittently. She denies joint or musculoskeletal pain or swelling, lymphadenopathy, easy bruising, genitourinary symptoms, vision changes, or other systemic complaints. She does report intermittent right upper quadrant (RUQ) abdominal “aching.” She was “unsure” of any weight loss. Her family history is unremarkable, and she has no sick contacts, recent travel, or recent animal bites or exposures. She denies sexual activity in the preceding 6 months, and prior activity was always with the use of condoms.Because of the persistence of her symptoms, she presented to an urgent care clinic where laboratory evaluation was significant for anemia with hemoglobin of 8.4 g/dL (reference range, 12–16 g/dL) and a leukocytosis of 37.6 × 103 white blood cells/mcL (reference range, 4.5–13 white blood cells/mcL). Given the laboratory abnormalities, she was referred to the emergency department for further evaluation and management. She is pale and tired-appearing on arrival, afebrile with a temperature of 36.8 °C, with a normal mean arterial pressure (MAP) of 69 mm Hg (normal >60 mm Hg), and with mild tachycardia of 109 bpm (normal <100 bpm). Notable examination findings include a nondistended, nonrigid abdomen that is moderately tender to palpation in the RUQ and mildly tender in all other quadrants with no rebound tenderness or costovertebral tenderness. There are no skin lesions or rashes and no cervical, axillary, or inguinal lymphadenopathy.Her initial blood work reveals elevated inflammatory markers with an erythrocyte sedimentation rate of 85 mm/h (reference range, 0–20 mm/h) and C-reactive protein level of 19.9 mg/dL (reference range, <1.0 mg/dL). A repeat complete blood count (CBC) confirms her previously noted anemia with a hemoglobin concentration of 7.1 g/dL and her leukocytosis of 28.1 × 103 cells/mcL with a neutrophilic predominance of 25.6 × 103 cells/mcL (reference range, 1–8 cells/mcL). Her red blood cell indices are normal. A comprehensive metabolic panel is notable for normal levels of liver transaminases and an elevated alkaline phosphatase of 204 U/L (reference range, 45–87 U/L) and gamma-glutamyl transferase of 108 U/L (reference range, 7–21 U/L). Her urinalysis is within normal limits, and a urine pregnancy test is negative.Given her elevated inflammatory markers, leukocytosis, and history of subjective fevers, antibiotics are started after obtaining blood cultures. The patient is admitted for further evaluation and treatment. Serology results for HIV, syphilis, Epstein-Barr virus, cytomegalovirus, and parvovirus B19 are nonreactive. Her gastrointestinal pathogen panel result is negative and fecal calprotectin level is borderline at 71.9 mcg/g (normal range, 10–50 mcg/g; borderline, 50–200 mcg/g). Because of the initially watery diarrhea, now resolved, with ongoing abdominal pain and borderline fecal calprotectin level, the gastroenterology consultation team recommends an abdominal radiograph and abdominal ultrasonography as a noninvasive way to evaluate free air and appendiceal or biliary pathologies, although they were unsure about specific diagnoses. Both are unrevealing. Overnight she has evidence of uncompensated distributive shock with declining blood pressures and increasing heart rates, persisting after 40 mL/kg of isotonic crystalloid fluid boluses.The differential diagnosis for a sexually active adolescent girl presenting with abdominal pain, vomiting, diarrhea, anorexia, fevers, and night sweats was broad and included both infectious and noninfectious etiologies. Pelvic inflammatory disease, including the possibility of perihepatitis (Fitz-Hugh-Curtis syndrome), was a leading concern given the patient’s sexual activity and constellation of systemic and abdominal symptoms. However, it was made less likely by the absence of recent sexual activity and absence of symptoms and signs associated with pelvic inflammatory disease. Ectopic pregnancy was considered but was unlikely given her sexual history, her fever, and the RUQ as the site of her greatest pain. Infectious causes such as Epstein-Barr virus (mononucleosis) and HIV were also entertained considering her systemic symptoms and prolonged course. The subacute nature of her illness made acute surgical conditions such as cholecystitis and appendicitis less likely. Inflammatory bowel disease was part of the differential diagnosis given the duration of her gastrointestinal symptoms, her elevated markers of inflammation, and neutrophilia raising the possibility of inflammatory bowel disease–related intrabdominal abscess, but this was felt to be less likely given the mild elevation of her fecal calprotectin level. Hematologic malignancies, especially acute leukemia and lymphoma, were in the differential diagnosis given her subjective fever, night sweats, and anemia, although there was no adenopathy, and the CBC results pointed more to an infectious process.Because of her continued instability, the gastroenterology team recommended a computed tomography (CT) scan of the abdomen. Imaging revealed a 4.2-cm abscess in the anterior right hepatic lobe (Figure 1); thrombosis of the right portal vein extending into the right hepatic lobe with signs of portal hypertension concerning for thrombophlebitis of the portal vasculature, known as pylephlebitis (Figure 2); a thrombus in the superior mesenteric vein; and a ring-enhancing mass in the mesenteric root, concerning for an additional abscess (Figure 3). A review of the imaging by the radiologist and the general surgery team revealed a retrocecal appendix with wall thickening and fat stranding, changes consistent with a previously perforated appendicitis. In summary, our patient had a ruptured retrocecal appendicitis with secondary pylephlebitis and an intrahepatic abscess.The patient presented with several weeks of gastrointestinal symptoms and was ultimately found to have a perforated retrocecal appendicitis with pylephlebitis and intrahepatic abscesses. Liver abscesses are a documented complication of appendicitis, but a subacute presentation with prolonged watery diarrhea in pediatric patients is rare. This case highlights a protracted clinical course following appendiceal perforation, emphasizing the need for awareness of such atypical presentations of appendicitis in children, which may not be detected by ultrasonography.1Retrocecal positioning of the appendix is a relatively common anatomical variant, occurring in more than 20% of cases.2 However, its posterior and retroperitoneal location can obscure clinical signs of inflammation and delay diagnosis. Atypical presentations, including RUQ pain or back pain rather than classic right lower quadrant tenderness, have been well-documented and are more common in retrocecal appendicitis.3 Several case reports have described prolonged courses and perforation because of delayed diagnosis in these patients.4Perforation is a well-known complication of appendicitis and in 1 retrospective case series was found to be more likely in retrocecal appendicitis.5 In most cases of perforated retrocecal appendicitis, abscesses are found in adjacent anatomical compartments such as the retroperitoneal space or psoas muscle or the subhepatic and perinephric space via the paracolic gutter.6 Notably, our patient did not have any of these typical abscess locations, highlighting the unusual nature of the disease course.Pylephlebitis is a rare but serious complication of intra-abdominal infections, including appendicitis.7 Although more commonly associated with diverticulitis and cholecystitis in adults, which contrasts our patient’s presentation, pylephlebitis has been described in association with both perforated and nonperforated appendicitis in pediatric patients.8,9 Following appendicitis, bacteria spread via the appendiceal and mesenteric veins to the portal vein and liver, leading to intrahepatic abscess formation.10 In children, this complication has a mortality of up to 50% if left untreated; therefore, prompt CT imaging is necessary for diagnosis.11,12Given concern for an infectious etiology, piperacillin-tazobactam was started for enteric coverage. Thrombi on imaging prompted a coagulation panel, which revealed an elevated prothrombin time, partial thromboplastin time, and fibrinogen. On hospital day 1, the patient developed an MAP below 60 mm Hg, raising concern for distributive shock from sepsis and possible disseminated intravascular coagulation in the setting of venous thrombosis and abnormal coagulation studies. She was transferred to the pediatric intensive care unit, and antibiotic coverage was changed to meropenem and vancomycin to cover for extended-spectrum beta lactamase–producing organisms and methicillin-resistant Staphylococcus aureus because of clinical decompensation of unclear origin.The interventional radiology (IR) team was consulted for needle drainage of the hepatic abscess, yielding approximately 6 mL of purulent material. Gram stain was positive for gram-positive cocci in pairs, gram-negative bacilli, and gram-positive bacilli, ultimately growing Bacteroides fragilis, Fusobacterium necrophorum, Streptococcus anginosus, and other aerobic and anaerobic flora, despite her blood cultures obtained on presentation with no growth. Antibiotic coverage was narrowed to piperacillin-tazobactam after the culture results returned.In conjunction with the initial IR drainage, the patient underwent a laparoscopic appendectomy with paracolic gutter irrigation, revealing a perforated appendix with adhesions and right lower quadrant inflammation. Although initially improving on piperacillin-tazobactam, persistent fevers and abdominal pain raised concern for poor blood supply and inadequate source control from the abscess. Imaging revealed new right hepatic lobe abscesses. Meropenem was initiated, and a second IR drainage was performed, yielding similar results. After the procedure, the patient’s markers of inflammation and symptoms improved. She was discharged on amoxicillin-clavulanate and enoxaparin. She completed 4 weeks of antibiotics with complete resolution of her abscesses and pylephlebitis on follow-up CT scan.The diagnosis of retrocecal appendicitis requires a high index of suspicion and should be considered in patients with less specific appendiceal symptoms, such as RUQ or flank pain, particularly when the appendix is not visualized on ultrasonography.Ruptured retrocecal appendicitis with a prolonged clinical course and secondary complications, including intra-abdominal abscesses and/or pylephlebitis, are reported complications described in the literature.Intrahepatic abscesses may develop because of pylephlebitis secondary to appendicitis and have a significant mortality rate when untreated; effective management often requires both source control and appropriate antimicrobial therapy.We would like to extend gratitude to Dr Reena Tam for her mentorship and guidance on this write-up as part of her global dedication to resident education.
Zhang et al. (Sun,) studied this question.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: