Clinical Vignette Symposium 2020
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The 2020 Clinical Vignette Symposium was held virtually Monday, May 11 to Friday, May 15. The Clinical Vignette Symposium is the OU-TU School of Community Medicine's annual showcase of clinical vignettes from fellows, residents, and students. The event was presented by the OU-TU School of Community Medicine and the Office for Research Development and Scholarly Activity.
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Item Open Access Duodenal Ulcer After Roux-en-Y Gastric Bypass Surgery: The Value of Laparoscopic Transgastric Endoscopy(2020-05) Hart, Thomas; Sahawneh, James; Chow, GeoffreyINTRODUCTION: While peptic ulcers can occur after Roux-en-Y gastric bypass surgeries, they are most commonly found as marginal ulcers at the gastrojejunal anastomosis and amenable to standard endoscopy. Peptic ulcers that occur in the gastric remnant and duodenum are less common and pose significant diagnostic and therapeutic challenges due to their inability to be accessed by esophagogastroduodenoscopy (EGD). We report one such case of a woman who presented with CT findings suggestive of a gastric remnant ulcer who underwent laparoscopic assisted endoscopy of gastric remnant and duodenum. CASE DESCRIPTION: A 53-year-old female who underwent a Roux-en-Y gastric bypass nine years prior presented to clinic with three weeks of sharp post-prandial abdominal pain radiating to the back. CT imaging of the abdomen was suspicious for inflammation and a possible ulcer in the anterior antrum of the gastric remnant without any free air. She was placed on a proton pump inhibitor by gastroenterology and then referred to surgery as this area was not accessible by EGD secondary to her gastric bypass. The patient subsequently underwent a laparoscopic lysis of adhesions, laparoscopic assisted endoscopy of gastric remnant and duodenum, and upper endoscopy of esophagus, gastric bypass, and distal extent of roux limb. She was discovered to have a duodenal ulcer. Biopsies taken from the gastric antrum and duodenum were negative for Helicobacter pylori or malignancy. DISCUSSION: Morbid obesity is a debilitating illness that has resulted in a growing volume of bariatric surgery. Sleeve gastrectomy and Roux-en-Y gastric bypass are the two most predominate weight loss surgeries performed currently in the United States. Proficiency in evaluating and treating complications of Roux-en-Y gastric bypass is imperative for management of bariatric patients, which includes evaluation for peptic ulcers and other pathology in the gastric remnant and duodenum. Endoscopy evaluation of the gastric remnant and duodenum cannot be performed trans-orally. This case demonstrates a laparoscopic assisted trans-gastric access for endoscopic evaluation of the duodenum and gastric remnant for peptic ulcer disease.Item Open Access An Interesting Case Highlighting Management of Pelvic Congestion Syndrome(2020-05) Hart, Thomas; Pandit, Viraj; Horst, Vernon; Kempe, Kelly; Kim, Hyein; Nelson, PeterINTRODUCTION: The development of valvular incompetence and/or obstruction in the pelvic and gonadal veins causing disabling symptoms comprises Pelvic Congestion Syndrome (PCS). It is a less known pathologic condition commonly occurring in women of child bearing age, and diagnosis can be challenging due to a variety of clinical presentations. Nutcracker syndrome (NCS) is a potential cause of PCS and involves compression of the left renal vein between the superior mesenteric artery and the aorta. We report one such case of a woman who presented with chronic pelvic pain. CASE DESCRIPTION: Our patient is a 31-year-old woman evaluated in our clinic with 6 years of unbearable back and suprapubic pain associated with dyspareunia and dysuria. She underwent evaluation by her gynecologist, and a transvaginal ultrasound demonstrated vaginal varicosities. Computed tomography angiogram (CTA) showed dilated left ovarian vein contiguous with left greater than right pelvic varicosities. We then performed a diagnostic venogram that demonstrated left pelvic venous plexus consistent with pelvic varicosities in the left ovarian vein and NCS anatomy. We decided to treat the PCS first. The patient underwent foam sclerotherapy of the pelvic venous plexus using 3cc of foam sclerosing agent (polidocanol) followed by coil embolization of the left ovarian vein. Post embolization venogram demonstrated radiographically successful treatment with no flow in the ovarian vein and preserved flow in the left renal vein. DISCUSSION: Chronic pelvic pain due to pelvic congestion is often associated with a constellation of symptoms including pelvic pain, dyspareunia, dysmenorrhea, and dysuria causing negative cognitive, behavioral, sexual and emotional consequences. There are three types of chronic pelvic pain, categorized by etiology. Type I is secondary to valvular incompetence of pelvic or ovarian veins, Type II is secondary to obstruction of outflow, and Type III is due to local compression. Venography is the gold standard for diagnosis. Treatment is directed at providing symptomatic relief with medical therapy, and treating the underlying cause by either endovascular (sclerotherapy and embolization, left renal venous stent placement) or open surgical techniques (including gonadal vein transposition, left renal vein transposition, or saphenous vein bypass).Item Open Access Thoracic Endovascular Repair of Descending Thoracic Aortic Thrombus(2020-05) Chitwood, Joshua; Horst, Vernon; Pandit, Viraj; Nelson, Peter; Kempe, Kelly; Kim, HyeinINTRODUCTION: Penetrating atherosclerotic ulcers (PAU) develop in the internal elastic lamina and indicate severe aortic atherosclerosis. PAU can have an associated intraluminal aortic thrombus, with associated risk of thromboembolism with subsequent mesenteric infarct and limb loss. We present a case of persistent descending thoracic aortic thrombus, with embolic renal infarcts, necessitating repair. CASE DESCRIPTION: A 80-year-old woman with history of bilateral renal infarcts on Eliquis and uncontrolled hypertension was referred to our department for extensive aortic atherosclerosis and 6mm ulceration proximal to the celiac artery, demonstrated on a CT. A follow up Computed Tomography Angiogram (CTA) demonstrated a persistent descending thoracic aortic thrombus and thoracic endovascular repair (TEVAR) was indicated. Preoperative imaging demonstrated >50% stenosis of the distal left common femoral artery (CFA). We accessed the proximal left CFA and subsequently placed stent grafts in the descending thoracic aorta, distally to proximally. Completion aortogram revealed complete occlusion in left external iliac artery. Doppler signals to the left foot were diminished on prompt exam. Current intraoperative imaging showed thromboembolism vs. flow-limiting dissection in the left CFA and external iliac arteries. Given these findings, an open endarterectomy of the iliac arteries was indicated, along with stent graft deployment in the left external iliac artery and bilateral stents in the common iliac arteries. A complete angiogram revealed a flow-limiting thromboembolism in the left superficial femoral artery (SFA). This was treated with covered stent placement, excluding the thrombus. Subsequent imaging demonstrated brisk flow into the left foot. On exam, pulses were palpable in the feet bilaterally and she moved all extremities. This patient had an uneventful hospital course and was discharge on postoperative day three. DISCUSSION: PAUs are a collective part of Acute Aortic Syndrome (AAS), seen in 2-8% of patients with AAS. PAUs develop from aortic mural thrombi, located most frequently in the abdominal aorta. A PAU > 4mm thick with ulceration is categorized as a complex plaque and warrants surgical repair. Anticoagulation is not sufficient for complex plaques. Endovascular repair (EVAR) is preferred to open since operating time is shorter, involves smaller incisions, and decreased hospital stay, with improved perioperative morbidity and all-cause mortality. Employing quality vascular access and wire manipulation can reduce endoleaks, access complications, and peripheral embolization. If complications arise, prompt imaging to access the stenotic vessels is necessary. This case demonstrates the potential morbidity of aortic thrombus embolization and the attention to detail required for successful treatment.Item Open Access Abdominal Pain of Unknown Origin(2020-05) Thompson, Gregory; Elvir, RobertoINTRODUCTION: Abdominal pain is a common presenting problem in both primary care settings and Emergency Departments. The underlying cause is not determined in about 1 in 3 patients. The following is a perplexing case of abdominal pain that presented to our University clinic. CASE DESCRIPTION: A 43-year-old female with a past medical history of diabetes and hypertension presented to the clinic with a chief complaint of generalized abdominal pain. The pain started about 6 months prior and occurred about every 4 weeks, and typically lasting for 3-4 days. The patient had tried many over-the-counter treatments including TUMS and Maalox. There was an improvement with bowel movements. She described the abdominal pain as a throbbing with no radiation. She denied any urinary symptoms, vaginal bleeding, vomiting, or hematochezia. She had approximately 45 pounds of unintentional weight loss during this time. On exam, the abdomen was soft, nontender to palpation, and nondistended. Bowel sounds were present in all four quadrants. There was no hepatosplenomegaly. Of note, patient had a history of diabetes mellitus type II and was currently taking metformin 1000mg BID and dulaglutide 1.5mg weekly. The patient had been on this regimen for approximately 11 months. The initial differential included IBS, IBD, iatrogenic, and anxiety. Lab work was obtained and included a CBC, ESR, and CRP. The CBC was remarkable for an eosinophil count of 1710; the ESR was elevated at 28. The CRP was unremarkable. A Stool Ova and Parasites was negative. Due to the elevated eosinophil count, the patient was referred to Gastroenterology who performed an abdominal ultrasound, EGD, and colonoscopy. The abdominal ultrasound was normal. The EGD inspection was normal. The biopsy showed some mild inflammation and was negative for H. pylori. The colonoscopy was only remarkable for diverticulosis. Given the negative workup, it was determined this patient was likely suffering from irritable bowel syndrome. DISCUSSION: Abdominal pain is a common complaint in primary care settings and emergency departments. The differential should include iatrogenic causes and abdominal migraines, along with the more common causes including IBS, IBD, H. pylori infection, and gastritis. This patient presented with a lengthy differential diagnosis given her comorbidities, medications, and eosinophilia. Given those findings, an EGD and Colonoscopy were warranted in order to exclude eosinophilic gastroenteritis, IBD, and Celiac Disease. IT is important to remember that Irritable Bowel Syndrome is a diagnosis of exclusion, and this case required an extensive workup to exclude it.Item Open Access Pseudohyponatremia Due to Severe Hypertriglyceridemia(2020-05) Harmon, Starr; Whitt, JanelleINTRODUCTION: Hyponatremia is defined as low sodium within the blood that can lead to headache, balance and gait issues, confusion, seizures, or even death in severe cases. However, in some instances a low sodium value on laboratory studies is not a true representation of sodium concentration. This is pseudohyponatremia. One known cause of pseudohyponatremia is extreme elevations in triglycerides in the blood. When lipids replace the water within the serum, a low sodium number will be recorded but will not be accurate. We describe a case of pseudohyponatremia in the setting of severe hypertriglyceridemia. CASE DESCRIPTION: extreme anxiety and confusion. She was recently prescribed 30 tablets of Ativan for her anxiety, a prescription that she regularly obtained, leading to a question of Ativan overdose. Her clinic labs from one week ago noted a sodium of 126. She was sent to the Emergency Department for further evaluation of confusion and agitation with concerns of overdose versus further worsening of hyponatremia. Though her triglycerides were elevated at 1,409 on recent labs, further assessment was warranted to confirm pseudohyponatremia. At the ED, her hyponatremia was worse than previously at 119. She was given IV saline and fluid restriction, and admitted to the hospital for further management of hyponatremia. A lipid panel was obtained which showed triglyceride level of 8,520, resulting in a diagnosis of pseudohyponatremia in the setting of severe hypertriglyceridemia. She was subsequently discharged the following day with instructions to follow up with her primary care physician for antihyperlipidemic medication and lifestyle measures. DISCUSSION: Without evidence of pancreatitis, she was not treated with plasmapheresis or discharged home with an antihyperlipidemic medication. The pseudohyponatremia-associated low sodium value left her delirium and agitation unresolved. She returned to clinic for discussion of her severe anxiety and was referred to psychiatry. Ativan was discontinued due to her risk of overuse. She was then lost to follow up as she cancelled her next four clinic appointments. Although hyponatremia is often a result of dehydration or other common causes including diuretic use, SIADH, and renal failure, pseudohyponatremia should be present in a differential. This case serves as a reminder to explore other possibilities when an abnormal lab result is obtained. A lipid panel is not always a routine part of a hyponatremia work up, but maybe should be when suspicion of common causes is low.Item Open Access Enterobacter Bacteremia Secondary to Pyelonephritis(2020-05) Brigance, James; Kern, ThomasINTRODUCTION: Pyelonephritis, an infection of the kidney, occurs in 16/10,000 women in the U.S. The rate in pregnancy is 2% versus 1% in the general population. Escherichia coli is the most common cause of pyelonephritis. Enterobacter is identified in <3% of cases. Fifteen percent of women with pyelonephritis develop bacteremia. We present a case of acute pyelonephritis in a postpartum patient who developed Enterobacter bacteremia and sepsis. CASE DESCRIPTION: Previously healthy 16-year-old female presented to ED with acutely worsening back pain and fever 3 days after vaginal birth to a healthy baby. She reported back pain for several weeks but contributed it to pregnancy. On arrival, she was tachycardic, febrile and initial labs showed WBC of 17,000 and UA suggestive of infection. CXR was normal. Blood and urine cultures were obtained. IV sulfamethoxazole/trimethoprim was started; a fluid bolus given. Over 24 hours, she became more ill and preliminary blood cultures were positive for gram-positive cocci. Antibiotic coverage was expanded to vancomycin and piperacillin/tazobactam. Kidney ultrasound showed possible pyelonephritis. Urine cultures grew Enterobacter with blood cultures positive for methicillin-sensitive Staphylococcus aureus and Enterobacter. Antibiotics were changed to cefazolin and piperacillin/tazobactam. After 48 hours, she remained tachycardic and febrile. Abdominal CT showed only kidney changes consistent with pyelonephritis. Infectious Disease was consulted and recommended meropenem and cefazolin. The next blood cultures were negative at 48 hours. Clinical improvement occurred over the next several days. She was discharged with PICC line for outpatient administration of antibiotic regimen for two more weeks. Follow-up with Infectious Disease and weekly CMP, CBC, ESR and CRP was scheduled. DISCUSSION: This case of postpartum pyelonephritis was complicated in many aspects. The patient’s recent pregnancy created delay in care as back pain and UTI symptoms were easily mistaken for pregnancy/postpartum-associated pain. The UTI developed into pyelonephritis and eventually bacteremia. The initial antibiotic regimen, targeted at UTI, proved ineffective against the pyelonephritis and bacteremia. Additionally, although only one culture grew MSSA, the antibiotic course should have covered it due to high virulence and associated morbidity. Many surrounding circumstances made this a difficult case. Although recommendations are to use antibiotics targeted at the likely infection, when treating sepsis, broad spectrum coverage should not be narrowed without ruling out blood stream infection. Due to her recent delivery and associated pain from pregnancy, this patient presented late with her condition, which allowed for a severe and life-threatening illness.Item Open Access Choking on Sand: Diffuse Calcification of the Lungs from Pulmonary Alveolar Microlithiasis(2020-05) Lepley, Summer; Eslam, Mohamed; Phillips, Kaitlin; Kiehl, Robin; Lesselroth, BlakeINTRODUCTION: Widespread pulmonary calcification is a relatively uncommon finding in patients presenting with dyspnea; differential diagnosis is limited, particularly when accompanied by a diffuse “sandstorm” appearance on plain radiographs. Pulmonary alveolar microlithiasis (PAM) is an extremely rare genetic disease characterized by deposition of calcium phosphate microliths choking the alveoli and small airways. CASE DESCRIPTION: A 33-year-old female immigrant from the Mediterranean region presented for acute on chronic dyspnea. Her 10-year history of exertional dyspnea was punctuated by two days of acute, unremitting, exertional and resting dyspnea with associated fever, pleurisy, and anxiety. Initial room air oximetry was 68%, increasing to 92% on 2L supplemental oxygen. Exam showed cachexia, tachypnea without accessory muscle usage, and diffuse bronchial breath sounds. Laboratory evaluation revealed polycythemia, and computed tomography showed essentially complete lung opacification with diffuse confluent sand-type calcific opacities with air bronchograms. Extensive inpatient workup for a precipitating infectious or rheumatologic etiology of acute decline was largely fruitless, including blood and sputum cultures, atypical respiratory panel, fungal cultures, and autoimmune workup. Quantiferon gold testing was positive. Bronchoalveolar lavage showed scattered calcified concretions. She was discharged on supplemental oxygen to follow up with an interstitial lung disease specialist and complete latent tuberculosis treatment. She was unfortunately lost to follow up after returning to her home country. DISCUSSION: Lung tissue calcification can be organized into three main categories: metastatic calcification (i.e., deposition in normal tissue due to systemic disease); dystrophic calcification (i.e., deposition due to trauma or previously abnormal tissue), and PAM – a rare genetic mutation producing an abnormal type IIb sodium phosphate cotransporter in alveolar type II cells, from mutation of the SLC34A2 gene1. The dysfunctional cotransporter fails to clear phosphate from degraded surfactant, resulting in microlith accumulation, chronic inflammation, tissue destruction, pulmonary fibrosis, and respiratory failure2,3. PAM has been diagnosed in 65 countries and is most common in Asia. There is a spectrum of disease severity; some patients present asymptomatically, discovered based on incidental abnormal radiographs, and some present in respiratory failure. CT chest plus bronchoscopy with bonchoalveolar lavage is typically used for diagnosis. Clinical course varies but most patients progress to respiratory failure and cor pulmonale. Currently the only definitive treatment is lung transplantation1. Widespread or multifocal calcification is rare. When diffuse alveolar calcific disease is present, the differential should include metastatic pulmonary calcification and pulmonary alveolar microlithiasis. In our patient, progression of her underlying lung pathology was suspected as etiology of progressively worsening dyspnea.Item Open Access Renal Artery Stenosis and Obstructive Uropathy: To Resect or to Stent?(2020-05) Kaiser, Daniel; Horst, Vernon; Pandit, Viraj; Kim, Hyein; Kempe, Kelly; Nelson, PeterINTRODUCTION: Atherosclerotic renal artery stenosis causes hypertension that is resistant to medical management and may eventually lead to loss of kidney function. The treatment options for uncontrolled hypertension include best medical therapy (BMT), renal artery stenting (RAS), or surgical intervention; however, the optimal modality remains uncertain. This is the case of a patient with renal artery stenosis complicated by concurrent obstructive uropathy. CASE DESCRIPTION: Our patient is a 66-year-old male with a long-standing history of hypertension uncontrolled with BMT (beta blocker, angiotensin II receptor blocker (ARB) and a thiazide diuretic) and progressive renal insufficiency which was presumed to be due to prostatic obstructive uropathy. Despite undergoing a transurethral prostatectomy, his renal function continued to decline. A pelvic magnetic resonance angiogram (MRA) was performed which showed bilateral renal artery stenosis with the left being worse than the right. Computed tomography angiogram (CTA) was then performed which confirmed significant bilateral renal artery stenosis of 80 percent and 60 percent of the left and right renal artery respectively. The patient then underwent balloon angioplasty followed by stenting of both vessels without complication. Post-stenting angiography showed less than 30 percent stenosis of either vessel. During hospitalization, he had some improvement in his blood pressure, and his renal function was improved from baseline. One month follow up showed no significant change in blood pressure from initial visit prior to stenting. DISCUSSION: In healthy subjects, reduced renal perfusion lowers the glomerular filtration rate (GFR), causing a response by the renin-angiotensin-aldosterone system (RAAS) which leads to a compensatory increase in blood pressure. Although the GFR is reduced, renal parenchyma is able to adapt to reduced blood flow if perfusion pressure is reduced by 40 percent. There are a number of ways to manage renal artery stenosis, including RAS versus BMT. Currently, the effects of stenting are controversial and not fully understood. Multiple randomized trails have shown confounding results. Some trails have shown benefit in RAS, while others have demonstrated increase adverse outcomes due to renal reperfusion. In this case, we decided to to proceed with RAS given the significant stenosis on imaging, the patient's clinical presentation of worsening renal function despite BMT, and having ruled out other causes of progressive renal failure. The decision to intervene in these patients is usually determined on a case-by-case basis after discussion between surgeon, nephrologist and patient and after educating the patient on the risks and benefits.Item Open Access Fusobacterium Nucleatum Brain Abscesses in an Immunocompetent Adolescent with Dental Braces(2020-05) Toor, Gavely; Martin, KimberlyINTRODUCTION: Fusobacterium nucleatum is an anaerobic, non-spore forming, gram-negative bacilli that is commonly found in soil, respiratory tracts of animals, and can be isolated from oropharyngeal specimens of healthy people. Fusobacterium spp. infections of dental plaque are most common in adolescents and young adults and may lead to periodontal disease. Fusobacterium spp. are capable of causing invasive disease commonly associated with otitis media, tonsillitis with Lemierre syndrome, gingivitis, and oropharyngeal trauma. CASE DESCRIPTION: A previously healthy 16-year-old, Hispanic male initially presented with a three day history of fevers, chest pain, and lower back pain and acutely developed headache, neck pain, and vomiting. Broad spectrum empiric antibiotics were promptly started. Cerebral spinal fluid (CSF) was concerning for bacterial meningitis with white blood cell (WBC) count of 18,440 with 91% segmented neutrophils, red blood cells (RBC) 1260, Glucose 34, and Protein 222. Pre-treated CSF Gram stain and culture were negative. MRI of the brain showed numerous ring-enhancing lesions within bilateral cerebral hemispheres and brainstem concerning for multiple cerebral abscesses versus Neurocysticercosis (NCC). Fungal studies and immune studies including HIV and TB testing were all negative. Neuroimaging was reviewed with national NCC experts who did not feel the imaging was consistent with NCC. Serum serology sent to CDC for Neurocysticercosis testing ultimately returned negative. CSF 16s PCR analysis for fungal and broad range bacterial pathogens resulted as Fusobacterium nucleatum. Triple antibiotic therapy with ceftriaxone, vancomycin, and metronidazole was continued for eight weeks. Repeat CSF analysis showed remarkable improvement with WBC 169, RBC 146, Glucose 29, and Protein 68. Repeat MRI of the brain after finishing antibiotic therapy showed ring-enhancing lesions remarkably decreased in size and number with no new lesions demonstrated. DISCUSSION: This case illustrates a very rare cause of brain abscesses in an immunocompetent adolescent with dental braces as the probable source of oropharyngeal infection. Further discussion with family and patient indicated that his oral hygiene routine was poor which likely lead to periodontal disease and ultimately dissemination of the organism to the brain. The diagnosis was ultimately established by 16s PCR analysis. The combination therapy with Metronidazole and Beta-lactams is recommended for an invasive disease. Because the patient was presumed to have meningitis, he received broad spectrum antibiotics which sterilized his CSF culture. This intriguing case of an uncommon cause of brain abscesses prompts further investigation of the role of Fusobacterium nucleatum causing disseminated disease in a healthy population.Item Open Access Right Supraclavicular Lymphadaenitis Caused by Kikuchi-Fujimoto Disease(2020-05) Toor, Gavely; Mather, Keith; Karikari, Ama; Kirkpatrick, GregoryINTRODUCTION: Kikuchi-Fujimoto disease is a rare and self-limiting condition with unknown etiology that is most commonly seen in young Asian women. It usually presents with low grade fever that varies from one week to one month, cervical lymphadenopathy between 2-7cm, and other non-specific symptoms including rash, arthralgias, fatigue, night sweats, and weight loss. Histopathology of the involved lymph node typically differentiates it from more serious, life-threatening conditions that mimic similar clinical features, such as lymphoma. CASE DESCRIPTION: We present the case of a previously healthy 15-year-old, African-American male, with a two week history of fatigue, low grade fever, and cervical lymphadenopathy with a prominent right supraclavicular lymph node. Lab work was significant for leukopenia, anemia of chronic disease, and mildly elevated ESR and LDH. Monospot testing was initially negative; however, EBV titers (both IgM and IgG) were found to be elevated. CT scan of the neck was consistent with bilateral cervical chain lymphadenopathy with a right supraclavicular lymph node measuring 2.5 x 1.6cm. Peripheral smear was negative for blasts and immunotyping by flow cytometry was negative for lymphoid population. Supraclavicular lymph node excisional biopsy showed focal areas containing lympho-histiocytic infiltrate and immunochemistry stain with CD68 of the lymph node was positive for histiocytes, consistent with proliferative phase of Kikuchi-Fujimoto lymphadenitis. DISCUSSION: Here is a very rare case of Kikuchi-Fujimoto disease preceded by EBV in a young African-American male. Etiology is unknown but preceding viral etiology has been proposed, such as EBV, HHV-6, HIV, Parvovirus, or Parainfluenza, or even bacterial etiology with Yersinia enterocolitica or Toxoplasma. Although it is a self-limiting condition, patients should be followed within one to four months for recurrence and increased risk of developing systemic lupus erythematosus. This intriguing case of an uncommon cause of right supraclavicular lymphadenitis prompts investigation and recognition by physicians and pathologists to avoid misdiagnosing it as lymphoma and initiating treatment with cytotoxic agents.Item Open Access Severe Coarctation of the Aorta: A Delayed Diagnosis(2020-05) Kaiser, Barrie; Couch, Taylor; Bielefeld, KyleINTRODUCTION: Coarctation of the aorta (CoA) is a congenital heart disease characterized by narrowing of the aorta near the aortic isthmus. The incidence of CoA is 4 out of every 10,000 births, accounting for 6–8% of all congenital heart disease. It is common for CoA to be accompanied by other cardiovascular abnormalities, such as bicuspid aortic valve, ventricular septal defects, and mitral valve stenosis. Early diagnosis and treatment of CoA is crucial to improving morbidity and mortality associated with this disease as the mean age of death in untreated coarctation is 34 years. CASE DESCRIPTION: A 10 year old male presented to the outpatient pediatric clinic for an acute illness. On examination, he was found to be hypertensive with a blood pressure of 126/69 accompanied by a systolic heart murmur. Medical history included the diagnosis of a heart murmur at a 2 year well child examination before the patient was lost to follow-up until age 9. Upon re-establishment he was noted to have a II/VI systolic heart murmur. He was seen multiple times during year 10 of life where this murmur was consistently documented. Referral to cardiology for further evaluation was made. At presentation to the cardiologist, patient was found to be well appearing with clear lung sounds and a III/VI systolic murmur heard best at the left upper sternal boarder with radiation throughout the chest. During examination absent femoral and lower extremity pulses with pale lower extremity nail beds were also noted. Echocardiogram demonstrated severe CoA, bicuspid aortic valve, and aortic root dilatation. He was started on metoprolol for aortic root dilatation. Referral for heart catheterization was made for planned stenting to relieve the coarctation. DISCUSSION: Heart murmurs are a common finding on pediatric exams. The primary care provider typically determines which heart murmurs are pathologic and require follow-up. In the setting of a heart murmur there are clinical signs that indicate when further follow-up with cardiology is needed, including absent distal pulses, hypertension, radiation of the murmur throughout the chest, and discrepancy between upper and lower extremity blood pressure. Early referral to cardiology is imperative for any murmur that is not innocent to prevent delayed diagnosis of pathologic lesions. This case demonstrates the importance of checking blood pressure and lower extremity pulses in the setting of an asymptomatic patient with a heart murmur.Item Open Access A Case of Congenital Syphilis Complicated by Severe Hepatocellular Dysfunction, Thrombocytopenia, and Atypical Skin Lesions(2020-05) Ifikhar, Jazeb; Robertson, Christopher; Watson, KimberlyINTRODUCTION: Congenital Syphilis (CS) is an infection caused by the spirochete Treponema Pallidum. It’s transmitted predominantly via the placenta, and only severe cases are clinically apparent at birth. Its incidence has risen sharply, with the CDC reporting 1306 cases in 2018, a 40% increase from 2017 nationally with a 92% increase in Oklahoma alone. CASE DESCRIPTION: A 28-year-old mom with no prenatal care and a positive admit maternal syphilis antibody underwent C-section and delivered a newborn female infant weighing at 3020 g with APGARS 6 and 9 at a gestational age of 38w5d. Maternal history was significant for illicit drug use and positive UDS for methamphetamine and marijuana prior to delivery. NB exam showed hypotonia, low-set ears, flattened nasal bridge, 2/6 holosystolic murmur, hepatomegaly, scattered petechiae on the back, an erythematous ring-shaped lesion on right palm, and another similar lesion on right foot. NB admission was complicated by hypoglycemia, thrombocytopenia and hyperbilirubinemia. Patient was admitted to NICU soon after delivery. Lumbar puncture with CSF analysis yielded RPR 1:64 and non-reactive VDRL. ECHO showed a small PFO. A diagnosis of CS was made anda 10-day course of IV Penicillin G was initiated. LFT showed transaminitis and TPN/SMOF was begun. Abdominal ultrasound confirmed hepatomegaly and HIDA scan results were consistent with severe hepatocellular dysfunction without exclusion of biliary atresia. The lowest direct bilirubin was 1.96 and lowest platelet level was 30. One platelet transfusion was administered and Ursodiol 10 mg/kgs was initiated. She was discharged with Ursodiol 15 mg/kgs and will be following up with the Audiology, Hepatology, Gastroenterology, Infectious Disease, and Opthalmology pending referral from PCP. DISCUSSION: This case illustrates the various clinical findings seen in classic CS as well as those unique to our patient. Interestingly, our patient had a healthy birthweight, whereas classic CS patients are four times more likely to have low birth weights. Distinctive features of this case include the ring-shaped skin lesions as large as 2 cm in diameter which were present at birth. The rash of CS usually appears later on in the disease course and consists of small brown/copper colored spots on the palm or soles. Another noteworthy finding in this case was the severe involvement of the liver requiring treatment with Ursodiol. Although early diagnosis and treatment is linked to better outcomes, our patient’s vast hepatocellular dysfunction may be an indicator of a poorer prognosis. The rising prevalence of CS may make these exam findings more common.Item Open Access Penile Injury in Infant Male: Accident or Abuse?(2020-05) Jones, Garrett; Conway, LaurenINTRODUCTION: Traumatic injury to the penis may occur from a wide variety of mechanisms in the context of accidental injury, or child physical or sexual abuse. Accidental penile injuries may include injuries occurring during toilet training in a closing toilet lid, with zippers, hair tourniquets, from straddle injuries, kicks, or more traumatic events such as bike wrecks or automobile accidents. Inflicted penile injury may occur in the context of physical or sexual abuse. The developmental ability, history provided by the caregiver, and other concurrent injuries play an important role in distinguishing between accidental or inflicted trauma. CASE DESCRIPTION: A 3 month old male presented to the emergency room due to redness on the glans and shaft of his penis that parents reportedly noticed after a bath. Coagulation studies and complete blood count were obtained which were unremarkable. Social work, Child Protective Services, and Law Enforcement were contacted. He was seen in follow-up the next morning at the Children’s Advocacy Center, where the Child Abuse Pediatrician ordered imaging studies to complete a work-up of possible child physical abuse, including a non-contrast head CT and a full skeletal survey radiography. Head CT was negative. The skeletal survey found bilateral distal medial metaphyseal corner fractures of the femurs, which raised additional concern for child physical abuse and prompted admission to the Children’s Hospital for further management. Pediatric orthopedics was consulted for management of the fractures; treatment included a Pavlik harness which was managed by orthopedics follow up. The patient’s father later admitted during interviews with law enforcement to have forcefully pinched the glans penis in an attempt to stop urination during the patient’s bath. The Child Abuse Pediatrician testified as an expert witness during the trial for this patient, which ultimately found the defendant guilty of child physical abuse. DISCUSSION: Classifying an injury as accidental, physical abuse, or sexual abuse depends on the setting in which it is reported as well as the intent behind the injury. Multiple accidental mechanisms of injury for the penis bruising were presented prior to the confession; no history was provided for the metaphyseal corner fractures. Child physical abuse was diagnosed due to the additional fractures and lack of history consistent with the injuries. The diagnosis was additionally ultimately supported by the partial confession. This case represents an uncommon presentation of child physical abuse that highlights the need for full appropriate medical evaluation and investigation by coordinating agencies.Item Open Access Disseminated Histoplasmosis in an Immunocompromised Pediatric Patient(2020-05) Ramirez-Cueva, Fatima Angelica; Jarshaw, Jane; Kuchmiy, Ella; Martin, KimberlyINTRODUCTION: Histoplasma capsulatum is a thermally dimorphic fungi that is highly endemic in the central and eastern United States. The fungus lives in soil contaminated by bird or bat droppings and is aerosolized and inhaled causing infection. Histoplasmosis is generally a self-limited disease. Most symptomatic patients have acute pulmonary histoplasmosis while immunocompromised patients can have severe pulmonary or disseminated infection. This case emphasizes the importance of considering disseminated histoplasmosis in patients with a fever of unknown etiology. CASE DESCRIPTION: A 10-year-old female with Juvenile Dermatomyositis, managed with mycophenolate and methotrexate, presented to the Pediatric Emergency Center with ten days of fever (T max of 103.7°F) and symptoms of myalgia, fatigue, and anorexia. She had no recent travel or significant exposures and immunizations were up to date. Labs were remarkable for a WBC of 2.7 with absolute neutrophil count of 1100, hemoglobin of 11.9 and platelets of 79. Peripheral smear was consistent with mild leukopenia and marked thrombocytopenia. CRP was elevated to 2.46 and ALT to 69. Chest x-ray was negative for infectious process and blood cultures showed no growth. Due to persistent fevers, infectious work-up was broadened to include testing for common respiratory and gastrointestinal pathogens, Cytomegalovirus, Epstein-Barr virus, Aspergillus, Bartonella, Brucella, Francisella tularensis, Mycoplasma, and tick-borne illnesses, with all results negative. Due to worsening pancytopenia, a bone marrow biopsy was performed which revealed non-caseating granulomas with fungal yeast forms consistent with Histoplasma capsulatum. Histoplasmosis urine and serum antigen tests were positive, as was bone marrow culture. She completed a two-week course of intravenous amphotericin B while inpatient, with continued oral itraconazole for 12 months. DISCUSSION: Disseminated histoplasmosis is acquired by inhaling fungal spores in endemic areas and presents with symptoms of prolonged fever, fatigue, anorexia and hepatosplenomegaly. Common laboratory findings include pancytopenia, transaminitis and hyperbilirubinemia. Typical radiologic findings include diffuse reticulonodular, interstitial, or military infiltrates, however 40–50% of immunocompromised pediatric patients with disseminated disease have negative chest x-rays. Tissue culture demonstrating typical fungal yeast forms is definitive for diagnosing histoplasmosis. Treatment with intravenous amphotericin B is given for a minimum of 2 weeks, contingent on clinical response. When clinical improvement is demonstrated, oral itraconazole is given for 12 months. Timely diagnosis of disseminated histoplasmosis can be challenging due to its heterogenous clinical presentation. This case illustrates disseminated histoplasmosis as an important differential diagnostic consideration especially in immunocompromised patients presenting with systemic illness.Item Open Access Shocking Results: A Case of Malignant Catatonia(2020-05) Kovelamudi, Asha; NcNaughton, Matt; McClanahan, Sarah; Manning, TessaINTRODUCTION: Malignant catatonia is a life threatening disease and can be difficult to distinguish from other disorders including delirium, neuroleptic malignant syndrome, or exacerbation of mood and psychotic disorders. Catatonia includes three or more of the following symptoms: stupor, catalepsy, waxy flexibility, mutism, negativism, posturing, mannerism, stereotypy, agitation, grimacing, echolalia and echopraxia. Malignant catatonia can include autonomic instability (fever, hypertension, tachycardia), severe muscle rigidity, and nonspecific laboratory findings of leukocytosis and elevated creatinine kinase. This syndrome can lead to severe morbidity and mortality if not promptly treated. CASE DESCRIPTION: An 18 year old African American male with no past psychiatric history and a family history of schizophrenia presented with new onset of mania and psychotic symptoms including bizarre, disorganized behavior and emotional outbursts. He initially responded to olanzapine for control of his symptoms, but over time stopped the medication due to side effects. As a result, he had a return of his psychotic symptoms along with waxy flexibility, sleeping for nearly 20 hours each day, restricted affect with minimal speech, and a refusal to eat or drink. The patient responded to a lorazepam challenge putting catatonia on the differential. The patient needed high doses of lorazepam to help with catatonia and severe symptoms of mania and psychosis, including hypersexual behavior, agitation, combativeness, and bizarre and disorganized thought content. With his symptoms, hypertension, and elevated creatine kinase, electroconvulsive therapy (ECT) was initiated for treatment of malignant catatonia. He responded well to six sessions with total resolution of symptoms. After discharge, he was admitted to our community assertiveness program and he continued to do well with low dose lorazepam, mood stabilizer and antipsychotic which were added for maintenance treatment of his mood and psychotic symptoms. Over several months, his outpatient psychiatrist slowly tapered the lorazepam and he continues to have good control of his mood and psychotic symptoms with no return of catatonia symptoms. DISCUSSION: First line treatment of catatonia is scheduled doses of lorazepam. However, in severe cases such as malignant catatonia, ECT should be initiated as soon as possible. The response rate to malignant catatonia is higher with ECT versus treatment with lorazepam alone. On review of case reports, it is beneficial to start ECT within five days of onset of symptoms to lower mortality rates. It is important to have catatonia on a differential diagnosis for patients presenting with mental status changes to ensure timely treatment is initiated.Item Open Access Birth Trauma Masquerading as Child Physical Abuse(2020-05) Beeson, Christine; Conway, LaurenINTRODUCTION: Birth trauma is a common cause of injury to newborn infants. In a minority of infants, birth trauma is mistaken for abusive injury. Suspected abuse requires additional diagnostic workup and involvement of child protective services and law enforcement. Neonates with concerning injuries present a diagnostic challenge for child abuse pediatricians and medical personnel. CASE DESCRIPTION: A 29-day-old male born large for gestation age at 40 weeks gestation with a NICU stay for pulmonary and cardiac problems presented to the hospital with a fever of 105°F. Medical history included propranolol for supraventricular tachycardia. Physical exam indicated boggy swelling of the right parietal area. Septic workup included urinalysis which showed hematuria, elevated white blood cells, leukocyte esterase, and growth of extended spectrum beta lactamase E. coli, confirming sepsis secondary to pyelonephritis. X-ray imaging found right ribs 5-8 with callus formation indicating subacute (>10 days old) rib fractures, resulting in a child abuse pediatrics consult. A skeletal survey showed right posteromedial 5-8 rib fractures. Head CT without contrast showed right occipital parietal soft tissue swelling. Bone mineralization appeared normal, and metabolic bone mineralization workup including osteogenesis imperfecta labs (COL1A1 and COL2A1) was negative. Investigating the birth history revealed good prenatal care but a difficult delivery complicated by six failed vacuum-assist attempts with a pop off, Robertson maneuver, attempted clavicle fracture, and an ultimately successful corkscrew maneuver. Physical abuse was ruled out and a diagnosis of residual birth trauma was made based on the subacute rib fracture callus formation, subgaleal hematoma, and birth history. He completed ten days of piperacillin/tazobactam with complete resolution of pyelonephritis. Repeat skeletal survey was negative for acute fractures. DISCUSSION: Posterior rib fractures in infants are highly specific and suspicious for abusive injury; however, other causes must be considered when rib fractures are discovered, including birth trauma. Neonatal fractures from routine deliveries are well-documented in the literature, especially in large neonates or with shoulder dystocia. In this case, difficult delivery including shoulder dystocia was pertinent history that aided in the diagnosis of birth trauma. It is imperative that physicians take a detailed history, including birth history, even when the presenting symptom appears not to be directly related. Labs investigating bone development disorders should be obtained when assessing neonatal fractures. The obstetric history in this case led to the retention of a family and spared them unnecessary involvement with law enforcement and child protective services.Item Open Access Enteric Duplication Cyst in a Patient with Suspected Milk-Protein Allergic Proctocolitis(2020-05) Larsen, Adam; Baab, Kelsey; McGhee, Jessica; Woslager, MeganINTRODUCTION: Gastrointestinal duplication cysts are rare congenital malformations that are typically discovered by routine prenatal ultrasound. These cysts can arise anywhere along the gastrointestinal tract but are most commonly found in the small intestine. Presenting symptoms vary, but often include: nausea, vomiting, abdominal pain or distention, obstruction, or a palpable mass. CASE DESCRIPTION: An exclusively breastfed eight-month-old female presented to the children’s hospital with severe microcytic anemia (hemoglobin concentration of 3.2 g/dL). Past medical history was significant for a six-month history of emesis and intermittent hematochezia. Around two months of age, she presented to the pediatrician due to intermittent loose, bloody stools, which were attributed to milk protein-induced proctocolitis. Dairy was removed from the mother’s diet, which reportedly provided some improvement. Iron deficiency anemia was diagnosed at four months of age when hemoglobin concentration was 8 g/dL and improved to 10.6 g/dL at six months after the initiation of iron therapy. Follow-up labs at eight months revealed severe microcytic anemia and prompted admission to the children’s hospital. Notably, medical history included a prenatal ultrasound concerning for bilateral ovarian cysts. Repeated postnatal images revealed decreased size of the cysts, and they were deemed insignificant. Upon admission, the patient was pale and tachycardic, but otherwise well appearing with a soft, non-tender, non-distended abdomen, normal bowel sounds, no palpable masses or hepatosplenomegaly. Ultrasound and CT of the abdomen showed bilateral abdominal mesenteric masses favoring mesenteric cysts. Exploratory laparotomy identified a ten cm bilobed retroperitoneal cystic mass and 80 cm of mesenteric duplication at the distal jejunum and proximal ileum closely adherent to native tissues with surrounding mucosal necrosis, ulceration, and hemorrhage. The native bowel could not be salvaged so resection and jejunoileostomy were performed. Hematochezia and anemia resolved after surgical recovery. DISCUSSION: This case demonstrates hematochezia, a relatively common pediatric complaint, which was suspected to be due to milk protein allergic proctocolitis and resulted in the missed diagnosis of a congenital enteric lesion. Unlike most cases of mesenteric duplication cysts, this patient had no palpable masses or abdominal distension. These lesions were detected by prenatal ultrasound but were dismissed postnatally. Severe microcytic anemia was the only sign of the severity of her illness and the need for prompt surgical management. Anatomic abnormalities discovered prenatally should be surveilled into infancy until they are resolved, corrected, or determined to be benign. Gastrointestinal duplication cyst(s) should be included in differential diagnoses for pediatric patients with unexplained hematochezia refractory to treatment.Item Open Access Multi-Drug Resistant Mycobacterium Abscessus Osteomyelitis Following an ATV Accident(2020-05) Guzman, Janitzio; Bolender, Emily; Sabet, Samie; Martin, KimberlyINTRODUCTION: Mycobacterium abscessus (MA) is a rapidly-growing nontuberculous mycobacterium (NTM) found in water and soil that causes pulmonary, skin/soft tissue, and bone infections. Treatment of MA infections is difficult as there is extensive antimicrobial resistance and no consensus among experts on one standard regimen. Agents such as amikacin, cefoxitin, clofazimine, and omadacycline have been used in the treatment of this infection. CASE DESCRIPTION: A previously healthy 15-year-old female presented for evaluation of an open midshaft fracture with visible contamination of the tibia and fibula after an ATV accident. She underwent surgical debridement and open fracture repair. Over the following two months, she had multiple readmissions for repeat debridement, and was placed on IV antibiotics for treatment of osteomyelitis due to numerous organisms. While these organisms were not recovered on subsequent intraoperative cultures, she did not have tissue healing as expected for her injury, prompting empiric expansion of therapy until acid-fast bacillus culture returned positive for M. abscessus sub. bolletii. Therapy was thus expanded to include clarithromycin and meropenem. Based on susceptibilities, the therapy was changed to amikacin, cefoxitin, and linezolid. Repeat debridements were undertaken, resulting in clearance of cultures five weeks after MA was isolated. All interim hardware was removed but an intermedullary rod was required given non-union of the tibia. Antibiotics were continued with excellent wound healing. Despite initial tolerance, after six months the patient experienced increasing serum creatinine and decreasing white blood cell count, concerning for potential amikacin and linezolid toxicities, respectively. In consultation with MA experts, the decision was made to transition to oral therapy with clofazamine, linezolid, and omadacycline. To date, laboratories are not suggestive of infection recurrence. DISCUSSION: MA is a rapidly-growing NTM uncommonly encountered in healthy children; however, MA resistance to multiple agents is common. Current data does not support a single standard therapeutic regimen. Rather, the infection usually requires a prolonged courses of antimicrobials particularly in presence of hardware. This patient tolerated her initial regimen for the majority of her course, but she began to demonstrate evidence of possible nephrotoxicity and myelosuppression. Due to these abnormalities and the absence of data suggesting a clear total duration of therapy, transition to an oral regimen of clofazamine, omadacycline, and linezolid was planned. While the literature supporting clofazimine use for this indication is scant, for omadacycline it is limited to a NTM pulmonary case. This case highlights the need for study and identification of safe and efficacious NTM susceptible antibiotics.Item Open Access Management of Endoleak After Abdominal Aortic Aneurysm Repair(2020-05) Pham, William; Horst, Vernon; Pandit, Viraj; Kempe, Kelly; Nelson, Peter; Kim, HyeinINTRODUCTION: Abdominal aortic aneurysm (AAA) represents a potentially life-threatening condition that requires proper surveillance and treatment. While AAA was historically treated by open surgical repair, technologic advances have resulted in Endovascular Aortic Aneurysm repair (EVAR) becoming the primary repair technique for AAA. Persistent endoleaks are a known complication of EVAR that often require additional intervention. We present a case highlighting the management of a Type 1B endoleak post-EVAR. CASE DESCRIPTION: Our patient is a 66-year-old male with history of EVAR at outside facility, and was evaluated in clinic with an enlarging iliac artery aneurysm. Computed tomography angiogram (CTA) demonstrated a 5cm aneurysmal degeneration of the right common iliac artery, most likely due to an endoleak in the distal portion of the stent (Type 1B). Due to the existing aortic endograft with a high flow divider, this required a combined axillary and femoral artery approach. The patient underwent placement of iliac branch endoprosthesis (IBE) at the right common iliac artery bifurcation with limb extension into both the external iliac and hypogastric arteries. Completion angiogram demonstrated successful extension of the endograft with exclusion of both the AAA and iliac aneurysm, preserved patency of the right hypogastric artery, and no endoleak. DISCUSSION: Endoleaks are a known complication of EVAR and frequently require intervention. There are multiple type of endoleak. Type 1 endoleaks denote leaks due to inadequate seal, and are divided further into 1A (proximal seal zone) and 1B (distal seal zone). Type II are due to branch vessel, type III endoleak are due to graft connection, and type IV due to graft porosity. The identification of location and type is frequently challenging and requires an angiogram. The management is based on the type of leak. Our patient had a type IB, due to leakage around the distal right common iliac limb. Relining the endograft with placement of an IBE device and extension of the stents into the external iliac and hypogastric arteries ensured development of adequate seal while preserving blood flow distally. With extensive pre-operative case planning and an intricate knowledge of the vascular anatomy, the modern vascular surgeon is equipped to provide advanced endovascular treatment options for complex vascular conditions.Item Open Access Hereditary Hemorrhagic Telangiectasia: Consequences of Delayed Diagnosis(2020-05) Kiehl, Robin Rainey Whitt, JanelleINTRODUCTION: Hereditary Hemorrhagic Telangiectasia (HHT), a rare autosomal dominant disorder also known as Osler-Weber-Rendu Disease, is often underdiagnosed. The initial symptom tends to be spontaneous and recurrent nose bleeds followed by the development of telangiectasias at multiple mucocutaneous locations. In HHT, patients develop arteriovenous malformations (AVMs) in the spine, lung, brain, and liver. This case examines the impact of delayed diagnosis of HHT. CASE DESCRIPTION: A 36-year-old male with history of alcoholic liver cirrhosis and neurologic deficits due to intraparenchymal hemorrhage (7 months prior) presented to the longitudinal free clinic to establish care and refill seizure medication. On further evaluation, the patient had history of an upper gastrointestinal bleed of unknown etiology in 2013, recurrent spontaneous epistaxis since middle school, telangiectasias on skin and roof of mouth, jaundice, and scleral icterus. Hemoglobin was decreased, but in proportion to his nosebleeds. CT angiogram two months prior showed cerebral AVMs. The diagnosis of HHT was confirmed with three of the four Curacao Criteria. Further evaluation was needed to assess for disease complications including gastrointestinal bleeding and pulmonary and hepatic AVMs. Transthoracic contrast echo with agitated saline, a screen for pulmonary AVMs, was positive. Abdominal ultrasounds showed an AVM in the left lobe of the liver. Due to a continual decrease in hemoglobin, an upper gastrointestinal study was ordered. Interventional radiology and neurovascular specialist were needed to evaluate possible embolizations of AVMs. Care would ideally be managed at a HHT center but due to the uninsured status of the patient this was challenging. Patient is currently stable and has not developed any new symptoms since establishing care. DISCUSSION: Many physicians who manage the complications of this disease are unfamiliar with the genetic condition; therefore, this disorder is often under-diagnosed. Diagnosis is made when three of four Curacao Criteria are met. The criteria are spontaneous and recurrent epistaxis, mucocutaneous telangiectasia, a first-degree relative with diagnosis, and multi-organ AVMs. An earlier diagnosis could have been made if his previous physicians were more aware of the disease, its associated signs and complications, and had conducted a more thorough history and physical exam. Unfortunately, the diagnosis of HHT was made months after the patient experienced a life-threatening hemorrhagic stroke caused by a cerebral AVM, resulting in neurologic deficits. Hopefully with the knowledge of his diagnosis, physicians will continually screen and evaluate him to prevent serious complications in the future.