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Case report: For facial swelling, look below the beltAn atypical presentation of a patient with a ruptured diverticulum highlights a concern of corticosteroid use—and demonstrates once again how in medicine, things aren’t always what they’re supposed to be.Kristopher M. Murphy, PA-C; Brent Savelli, MD; Karen Newell, PA-CKris Murphy works at the Georgia Sports Medicine and Orthopedic Clinic, Tifton, Ga. Brent Savelli works at Tift Regional Medical Center in Tifton. Karen Newell is affiliated with the PA program at Emory University School of Medicine, Atlanta, Ga. The authors have indicated no relationships to disclose relating to the content of this article.
In medicine, clinicians are constantly reminded to look for the horses, not the zebras. While that remains good advice, especially to those just beginning their careers, a mind that is open to the unusual remains important when developing a differential diagnosis. The following case illustrates how disease states may not always manifest as the textbooks say they should. In addition, it shows that practitioners must maintain a constant awareness of the potential side effects of the medications their patients may be taking, as well as understand how medicines may affect the already inconsistent presentation of disease states. This report, which presents a rare case, shows the impact and role of specific pharmacologic agents as the disease developed. The case also demonstrates the importance of history taking and how information that may appear trivial can lead to a diagnosis if the clinician is equipped with a firm foundation of medical knowledge. Case reportA 63-year-old white man presented with a chief complaint of right-sided facial swelling that had increased over the prior 24 hours. During the initial examination in the office, he denied any fever, chills, rhinorrhea, or facial pain or pressure. In addition, he reported no associated visual changes or recent history of trauma to the affected area. On physical examination, there was obvious right-sided facial and periorbital swelling that greatly limited the patient’s ability to open the right eye (see Figure 1). Subcutaneous crepitus was noted upon palpation of the involved area. The patient had an extensive medical history, but it appeared unrelated to his present problem. He had documented atherosclerotic coronary heart disease (CHD) with an MI in 1989 and triple coronary artery bypass graft surgery in 1991. He was taking aspirin, atenolol, diltiazem, and simvastatin for the CHD. In addition, over the past 2 years, he had suffered from degenerative disk disease with foraminal and central canal stenosis at C3-4 and C4-5. Most recently, the patient had developed lower extremity symptoms, including difficulty walking that was related to a spastic gait, low back pain, and right-sided sciatic pain. Two weeks before onset of the facial swelling, he had seen his neurosurgeon, who recommended a course of dexamethasone, 2 mg every 8 hours, and rofecoxib, 25 mg daily, for an exacerbation of radicular (sciatic) pain. In addition, the patient was taking omeprazole 20 mg daily. He was a nonsmoker and admitted to occasional alcohol consumption. Workup and treatmentBecause of the unusual symptoms, concern about the possibility of severe infection, and the recent history of corticosteroid usage, the patient was admitted to the local hospital for further evaluation. Upon admission, his vital signs were as follows: temperature, 98.1°F (36.7°C); heart rate, 64 beats per minute; BP, 146/78 mm Hg; respiratory rate, 16 breaths per minute; and oxygen saturation, 94% on room air. Initial laboratory studies included a CBC with differential, a complete metabolic panel, and blood cultures. These revealed leukocytosis (WBC count, 32,400/mm3) with 92% polymorphonuclear cells, stable hemoglobin and hematocrit, mild hyperkalemia (potassium, 5.3 mEq/L), and slightly elevated ALT level (54 U/L). All blood culture results were eventually reported to be negative. Differential diagnosisGiven the patient’s physical presentation as well as the leukocytosis, the differential diagnosis at this point included a necrotizing soft tissue infection versus infection originating from a sinus or Mucormycosis, or rhinocerebral zygomycosis, is a fungal infection that typically occurs among patients who have diabetes or are immunocompromised. The infection develops in the nasal sinuses, progresses through the ethmoid sinuses into the retroorbital space, and eventually manifests late as periorbital edema, painful extraocular movement, and proptosis. Zygomycetes thrive among the acidic environments produced in diabetic ketoacidosis and in the presence of neutropenia associated with immunosuppression. Imaging studies
Upright posteroanterior and lateral chest radiographs were then ordered, as was CT of the neck and thorax to localize the source of air. CT of the neck showed extensive emphysema, while the thorax study revealed extensive air in the mediastinum with no signs of pneumothorax, pneumonia, or pleural effusion (see Figure 3). The chest radiographs demonstrated the pneumomediastinum as well; more significantly, however, there was free air under the diaphragm (see Figure 4). CT of the abdomen and pelvis was then performed, revealing air in the left anterior perirenal space and adjacent to the lesser curvature of the stomach, pancreas, and vena cava. There was evidence of extensive air around the sigmoid colon, along with scattered diverticuli (see Figure 5). However, no thickening of the bowel wall was present to suggest diverticulitis. Diagnostic laparotomyA preliminary diagnosis of subcutaneous emphysema resulting from a ruptured diverticulum was made, and general surgery was consulted for management. On examination, the surgeon found the abdomen to be soft and nondistended, with only mild left lower quadrant tenderness and no peritoneal signs. Diagnostic laparotomy revealed diverticulitis with perforation of a sigmoid diverticulum, small bowel adhesions, and abdominal wall cellulitis. A sigmoid colectomy/Hartmann procedure and small bowel resection were required.
An atypical presentationOne of the most unique aspects of this case was the atypical manifestation of a ruptured diverticulum.1 In most cases of diverticulitis, before perforation the patient will develop symptoms such as fever, constipation or diarrhea, nausea, vomiting, and noticeable left lower quadrant pain.2 In the case of right-sided diverticuli or a redundant sigmoid colon, the pain may be localized to the suprapubic area or the right lower quadrant. Following perforation, the patient will typically manifest diffuse peritoneal symptoms and signs that may include significant abdominal pain with guarding and rebound tenderness. Unfortunately, with the exception of mild left lower quadrant tenderness to palpation, this patient’s abdominal examination remained relatively benign during the course of the developing infection. There have been reports of retroperitoneal gas passing through the diaphragmatic hiati and the mediastinum to track within soft tissue fascial planes and manifest as subcutaneous emphysema of the neck or face.2,3 However, air from the sigmoid rarely produces cervical emphysema because it is typically contained in the retroperitoneal/anterior pararenal compartment.2 As with this patient, retroperitoneal perforations are typically difficult to diagnose without the aid of CT, because of the relatively normal abdominal examination.4 The implications of corticosteroidsFollowing the diagnosis of subcutaneous emphysema originating from a ruptured diverticulum, the role that corticosteroids played in this case became more obvious. Glucocorticoid (GC) use is a risk factor for perforation of diverticuli, especially in patients older than 50 years.5,6 One source reports that up to 20% of patients with perforation had received prior GC therapy.1 A study of neurosurgical patients older than 50 found that 5 of 719 patients sustained colonic perforation while receiving a mean dose of 3,947 mg of methylprednisolone over at least 7 days.6 This is compared to 3,749 patients who remained incident free without GC therapy over the same time period.6 Of GI adverse effects from corticosteroids, upper GI perforation and peptic ulcer disease are up to 1.8 times more common than colonic perforation. The concomitant use of NSAIDs increases the rate of upper GI problems 12 times further.7 GCs increase gastric acid secretion, decrease mucus production, cause gastric and parietal cell hyperplasia, and delay the healing of existing or developing gastric ulcers.8 Although lower GI involvement is less common in patients who have taken GCs, when it does occur it is accompanied by increased mortality. GCs have a profound impact on soft tissue integrity as well as on wound healing. They have been shown to decrease protein synthesis by fibroblasts and the synthesis of type I and III collagen.8 This is especially significant because of the histologic makeup of the peritoneum and serosa with type III collagen. GCs decrease mucosal regeneration and repair in the GI tract and cause thinning of the bowel wall. It is common knowledge that GCs prevent or minimize inflammation; however, this in turn delays the inflammatory phase necessary for wound healing.9 GCs interrupt the release of various factors, including pro-inflammatory cytokines, growth factors, matrix proteases, and matrix proteins.8 Patients who experience perforation while receiving GC therapy will often present late in the disease process—a result of a lack of early warning signs and symptoms that would typically allow for earlier intervention. One source documents an average delay of 8 days before symptoms appear and the diagnosis of peritonitis is made, with a mortality rate of approximately 85%.5 The increased rate is attributable in part to a late presentation accompanied by more severe side effects, as well as to more severe infection accompanied by a decreased immune response. There is no evidence that infection rates are increased in those patients taking less than 10 mg of prednisolone daily. In this case, the patient’s dexamethasone dosage was the approximate equivalent of 40 mg of prednisolone daily. However, although minimal to moderate dosages may not increase the infection rates, they do increase the risks of opportunistic infections and complications—including active tuberculosis, oral candidiasis, and aspergillosis. One study utilizing a retrospective analysis found the risk of infection to be 1.6 times higher in patients taking GC therapy.8 ConclusionWhile this case included a rare presentation that most clinicians may never encounter, it is a valuable teaching tool in many respects. Clinicians are trained to look for the more common conditions and to use likelihood percentages when developing a differential diagnosis. In doing so, they will definitely be right more often than they are wrong. However, while most patients under these circumstances will receive a correct diagnosis, consider the difficulty of explaining to patients that the course of their disease has been adversely affected because it did not follow the norm. While keeping the most likely diagnosis in mind, providers must still remain prepared for the unexpected and always maintain an appropriate amount of doubt. Although clinicians cannot refrain from prescribing systemic corticosteroid therapy in cases such as this one, this patient serves as a critical reminder of the power entrusted to health care providers and how their decisions can have a profound impact on outcomes. It is crucial to maintain a firm understanding of the medications prescribed in order to be able to predict the possible consequences. Clinicians must also rely on their understanding of pharmacology when developing a differential diagnosis, which may lead to earlier disease identification and treatment. REFERENCES
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retropharyngeal location. The possibility was also considered, because of the crepitus and likely subcutaneous emphysema, that Clostridium perfringens had produced gas gangrene. Mucormycosal infection of the sinuses was also thought to be a possibility because of immunosuppression secondary to the corticosteroid usage. Tables 1 and 2 summarize the differential diagnosis of subcutaneous emphysema and acute facial swelling.
CT of the head and sinuses was obtained and revealed extensive subcutaneous emphysema over the right face and periorbital region (see Figure 2). There were no identifiable abscesses and only minimal mucosal thickening in the sinuses. In addition, flexible rhinolaryngoscopy was unremarkable, making mucormycosis unlikely.
Postoperatively the patient was transferred to the ICU, and treatment was initiated with IV piperacillin/tazobactam, clindamycin, and tapered hydrocortisone sodium succinate. He was discharged home 8 days following his initial presentation to the office. Following a lengthy recovery period that included spinal decompression and fusion surgery, the patient returned 10 months later for a subtotal colectomy with ileorectal anastomosis and colostomy closure.