Appendicitis: common and commonly missed – the story of Alice Tapper

Clinical presentation: day 1, Saturday

In the morning, Alice’s mother called their pediatrician to report she has been vomiting and complaining of stomach cramps. The pediatrician recommended clear fluids and acetaminophen. In the evening, her mother reported Alice is very weak but able to take sips and is no longer vomiting. The pediatrician recommended going to the emergency department (ED) if patient is lethargic.

Clinical presentation: day 2, Sunday

In the morning, the patient’s mother reported Alice had a temperature of 102 °F. She was still drinking fluids, but unable to get up and walk. The pediatrician suggested she has the “stomach flu going around” and recommended ibuprofen for fever. In the afternoon, her mother reported Alice had worsening stomach cramps, decreased urination and drinking, but was able to eat a bagel with peanut butter. An at-home COVID-19 test negative. The pediatrician recommended going to the ED for intravenous fluids. Alice was reluctant, so the pediatrician advised if able to drink a large amount of water and has good urination, it would be acceptable to defer ED visit.

Clinical presentation: day 3, Monday

The patient’s mother reported copious diarrhea and weakness. She took Alice to the ED. The patient’s pediatrician recommended an abdominal ultrasound to evaluate for appendicitis. In the ED, Alice’s chief complaint was documented as “stomach cramps, diarrhea, and dehydration.” Her temperature was 36.9 °C, blood pressure 101/72 mmHg, and heart rate 137/min. Physical examination was documented as having no hesitation with position changes; her abdomen was noted to be soft with minimal tenderness without distention. The ED’s initial working diagnosis of gastroenteritis was similar to the one entertained by the pediatrician and laboratory evaluation was ordered. However, imaging was deferred due to a reassuring abdominal examination. Importantly, the nursing notes reflected ongoing abdominal pain that Alice rated as six on a 10-point scale.

Clinical presentation: day 3, Monday, continued

Alice was given 2 L of normal saline intravenously and administered dicyclomine for abdominal cramping. Initial laboratory evaluation revealed a white blood cell count of 20,000 per microliter, (91 % neutrophils, 0 % bands) and a mild hypokalemia. The remainder of the CBC and electrolytes were normal. Five hours after presentation and after fluids, her temperature was 38 °C, blood pressure 85/39 mmHg, and pulse 118 beats per min. An additional liter of normal saline was given. Three hours later (8 h after ED presentation), Alice’s parents expressed concern that she was not improving. They repeated the pediatrician’s recommendation that an ultrasound be performed. The decision was made to admit Alice to a pediatric inpatient facility. Prior to transfer, her pain was again noted to be six on a 10-point scale; minimal abdominal tenderness was present. Documentation at the time of transfer stated that she was a “14-year-old female present[ing] with nausea/vomiting/diarrhea. Patient tachycardic on arrival, reassuring exam otherwise. Noted to be persistently tachycardic with soft blood pressures after several fluid boluses. We will plan to transfer by private vehicle to inpatient facility for further work-up.”

Before transfer the patient’s father reported to the pediatrician that “Alice still has abdominal pain, especially when she walks.” The pediatrician suggested by phone to the parents that with abdominal pain, fever, pain with walking, and elevated while count, “when at the second hospital, you want them to evaluate for appendicitis or ruptured [appendicitis].” Upon presentation to the admitting facility, the physical examination of the abdomen was noted to be “soft, non-distended, with diffuse tenderness to palpation. No McBurney’s point tenderness. Murphy Sign negative. Rovsing sign negative. Negative Obturator sign. able to climb out of bed and jump twice.” The differential diagnosis at admission included food-borne illness, gastroenteritis, and appendicitis. However, appendicitis was thought less likely due to non-focal physical examination findings and lack of peritoneal signs. She was given fluids and serial examinations planned, and an ultrasound of the abdomen to be considered if she did develop right lower quadrant pain. The family repeats the pediatrician’s suggestion for an ultrasound and are again told the diagnosis is gastroenteritis, most likely viral and that an ultrasound is not necessary. This may be an example of diagnostic momentum, in which a diagnosis, once made, is not reconsidered.

Clinical presentation: day 4, Tuesday

After admission, Alice continued to have diffuse abdominal cramping and nausea without vomiting or fever. She had four green watery bowel movements. She was able to tolerate some oral intake, and her cramping was noted to improve with bowel movements. Her vital signs included a temperature of 38 °C, heart rate 95 bpm, respiratory rate 18 breaths per minute, and blood pressure 108/57 mmHg. She was noted to be tired appearing on examination without acute distress. Repeat abdominal examination the next morning by a second attending physician was identical (verbatim) to the exam on admission. “Soft and nondistended with diffuse tenderness to palpation but without McBurney’s point tenderness; Murphy Sign, Rovsing sign, and obturator sign were all absent. She was able to climb out of bed and jump twice.” The patient’s mother, increasingly concerned, asks why if a viral syndrome is present no household contacts were sick. Alice was administered acetaminophen and famotidine. Polymerase chain reaction (PCR) testing for common enteric pathogens was negative. She was seen by her pediatrician, who consulted with the inpatient team, and agreed with the diagnosis of “severe gastroenteritis.” As the day progressed, she was noted to have altered mental status with difficulty answering questions. Her pain worsened, and she was administered ketorolac intravenously. Increasingly concerned that Alice is in extreme pain, the patient’s mother called the father, who was able to convince a hospital administrator to call the clinical team to insist on an abdominal radiograph. At the same time, Alice had relayed her concern that “my belly is getting bigger” to the provider team. As the radiograph was ordered, the family were told “she doesn’t need this.”

Clinical presentation: day 5

The abdominal radiograph showed air-fluids levels. CT scan of the abdomen and pelvis revealed “Perforated appendicitis with associated distal small bowel obstruction, diffuse peritonitis, large amount of intraperitoneal fluid. The largest pocket of fluid (10 cm) is located in the dependent areas of the pelvis.” At 12:40 am, piperacillin-tazobactam was started. At 10:19 am blood pressure was 85/51 mmHg and Alice was transferred to the surgical intensive care unit. At noon, blood pressure decreased to 77/47 mmHg and heart rate was 102 bpm. The rapid response team was called. Aggressive fluid resuscitation and pressors were initiated as the patient was in sepsis with septic shock.

Clinical presentation: days 6–11

Multiple abdominal abscesses were drained by interventional radiology and drains left in place; IV antibiotics were continued. On Day 10, repeat ultrasound showed “interval decrease in size of the suprapubic fluid collection, with persistent complicated right lower quadrant collection approximately 3.0 × 2.6 × 4.2 cm. Partially visualized percutaneous drainage catheter within the pelvis. Persistent complicated right lower quadrant fluid collection.” The transrectal drain was removed.

At discharge, Alice was afebrile but still complaining of some abdominal pain with mild distention and diarrhea. Oral intake was improving. Serous output from LLQ drains had decreased. The drains were removed, and Alice was discharged on a 14-day course of oral antibiotics.

Clinical presentation: day 3 post-discharge

Alice complained of persistent abdominal pain and presented to the surgery clinic. Abdominal X-ray showed: “Similar dilated loops of small bowel with multiple air-fluid levels, may represent ileus however developing obstruction is not excluded. No evidence of free intraperitoneal air.” There was no change in treatment.

Clinical presentation: day 6 post-discharge

Alice presented for follow up to the surgery clinic complaining of persistent abdominal pain.

Repeat ultrasound showed ascites and several complex fluid collections in the right lower quadrant. The largest pocket now measured 5.1 × 7.3 cm in the transverse plane (was 4.2 × 3.0 seven days earlier). WBC was 18,500 cells/microliter. CT of the abdomen/pelvis revealed multiloculated fluid collection in the lower abdomen, measuring approximately 9.2 × 6.3 × 4.7 cm. She was hospitalized for IV antibiotics and abscess drainage for an additional 8 days. A delayed appendectomy with lysis of adhesions was performed three months later.

Discussion: why do we keep missing appendicitis?

Alice’s father is CNN anchor Jake Tapper. In 2023, CNN broadcast her story to raise awareness, as Jake and Jennifer Tapper put it, that “doctors are not sufficiently aware of how often appendicitis does not present in a standard way.” Recent studies agree with them. In Chartier’s 2021 review of 1.5 million Ontario ED visits, the missed appendicitis rate approached 10 % [2]. Mahajan et al. (N-155,000 patients) found 6 % of adult and 4.4 % of pediatric cases of appendicitis were missed [3]. Appendicitis afflicts 7–8% of the population in their lifetime. Ruptured appendicitis has a 1.7–3% mortality rate (15 % in the elderly), and consistently ranks among the top 10 causes of medical malpractice lawsuits in adults and children [4], 5].

Understanding and teaching the protean natural history of appendicitis is key for decreasing misdiagnosis. Historically, clinicians – as in this case – have over-relied on McBurney’s point tenderness as the sine qua non of the diagnosis. One of the best roadmaps to the twists and turns of appendicitis is Cope’s Early Diagnosis of the Acute Abdomen [6]. Following are two of Cope’s insights as they pertain to Alice’s clinical course:

RE: Initial diagnosis of “severe gastroenteritis”: “…in my experience, the diagnosis of gastroenteritis is so often incorrect as to raise a serious question whenever the emergency physician comes to this conclusion. The diagnosis is usually made in a patient with abdominal pain, nausea, diarrhea or any combination of these that cannot be attributed to a more definite condition.” (p. 88).

RE: Alice’s clinical course: “the perforated pelvic appendix is one of the most easily overlooked and therefore one of the most dangerous conditions that may occur in the abdomen…rigidity of the lower abdominal wall is frequently absent even when a pelvic abscess is present….When rupture occurs, the epigastric pain diminishes and local pelvic peritonitis results…at the bottom of the pelvic pouch. This is usually unaccompanied by rigidity of the lower abdominal muscles, and since the pain of appendicular distention has ceased and the pain due to pelvic peritonitis at this stage is frequently very insignificant, the patient may seem better, and examination of the abdomen may give little indication of the trouble in the pelvis. Sooner or later – usually within three or four days…. (it will) give rise to generalized peritonitis.” (p. 81).

Cope also explains why the late onset (48 h in) of diarrhea can be due to colonic irritation from an abscess, rather than the reassuring “confirmation” of gastroenteritis.

Cope’s multidimensional description of appendicitis contrasts with McBurney’s oversimplification:

“(In appendicitis) …the seat of greatest pain, determined by the pressure of one finger has been very exactly between an inch and a half and two inches from the anterior spinous process of the ilium on a straight line…to the umbilicus.” [7].

Despite this description and the challenges with diagnosing appendicitis, we continue to teach McBurney, not Cope. Becker et al. (N-755, median age 11.9) tallied 10 “atypical” signs and symptoms in 272 patients with confirmed appendicitis and found 32 % had absence of pain in the RLQ; 52 % no rebound tenderness; 47 % no guarding; 16 % had diarrhea. Somewhat alarmingly, 44 % had six or more atypical features. [8].

Cope’s most critical observation – that ruptured appendicitis can transiently stabilize as Alice did – is worth repeating:

“(With perforated pelvic appendix) …the patient may seem better, and examination of the abdomen may give little indication of the trouble in the pelvis. Sooner or later – usually within three or four days…. (it will) give rise to generalized peritonitis.”

Scoring systems are a modern approach to evaluation of patients at risk for appendicitis. However, Jennifer Tapper later remarked that had the ER or the admitting teams applied any of the current appendicitis scoring systems, Alice would have met criteria for imaging. Applying the widely used Alvarado score, Alice’s would have been 4: 1 point each for nausea/vomiting (though that had abated), one for fever and two for a WBC of 20,000/μL. Presence of anorexia was not queried. The other three criteria, migratory right lower quadrant pain, tenderness in RLQ, rebound in RLQ, were deemed absent.

Inter-observer variability is a potentially confounding factor. The Pediatric Appendicitis Score, for instance, allots two points for “pain with cough, percussion or hopping.” Inter-observer variability was evident when Alice was repeatedly asked to hop, which the providers documented as “able to jump twice,” while her father (as noted above while still in the ED) wrote in his notebook she still had pain with walking.

As for the utility of scoring systems, a 2021 comparison of the Alvarado score vs. the Pediatric Appendicitis Score (PAS) showed sensitivity of 85 vs. 93.8 %, specificity 70 vs. 70 % and diagnostic accuracy of 84 vs. 91.6 % [9] These scores are still imperfect – highlighting why Cope remains a useful adjunct to standard teaching and current scoring systems. A deep baseline knowledge of the varied presentations of appendicitis can help clinicians navigate with patients through this challenging diagnostic journey.

A remaining important question in Alice’s case was why successive clinicians declined to order an ultrasound despite its immediate availability, and repeated parent requests. Sensitivity for diagnosing appendicitis in a fourteen-year-old with a slim body habitus probably exceeds 90 % [10] Did the parents’ entreaties – prompted by their pediatrician – trigger a subconscious reflex of “who’s the doctor here?” Then again, the provider team might have reasoned that a non-diagnostic ultrasound would mandate a CT abdomen/pelvis with its attendant radiation.

To close, Dr. Cope writes:

“It is not facetious or cynical to say that the diagnosis of gastritis or gastroenteritis is usually made in the emergency ward by a young physician who is ‘not impressed’ by a patient’s abdominal pain or physical findings.” (p. 87).

His opus, like appendicitis itself, cannot be reduced to a single point. We must master it whole. A brave young woman and her tenacious family – and all our future patients – deserve nothing less.