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Alexis Collins, DO October 15, 2011. cc: Weakness bilateral lower extremities with multiple areas of skin necrosis of both lower extremities G.C. is a.

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Presentation on theme: "Alexis Collins, DO October 15, 2011. cc: Weakness bilateral lower extremities with multiple areas of skin necrosis of both lower extremities G.C. is a."— Presentation transcript:

1 Alexis Collins, DO October 15, 2011

2 cc: Weakness bilateral lower extremities with multiple areas of skin necrosis of both lower extremities G.C. is a 50 y/o WM with end stage renal disease and history of nephrotic syndrome of unknown etiology, who was changed from peritoneal to hemodialysis. G.C. gained a significant amount of weight while on peritoneal dialysis secondary to anasarca, especially of lower extremities G.C. has been experiencing leg weakness on standing up with unsteadiness of gait for last 3 months and is now unable to ambulate, requiring assistance with transferring from bed to chair and is now confined to a wheelchair

3 At first presentation of patient’s lower extremity weakness it was felt that symptoms were related to his history of painful diabetic peripheral neuropathy which was being managed with Neurontin and degenerative disk disease with mild bulging of the disk and mild to moderate spinal stenosis at L4 and L5 Evaluated by neurologist in Grundy, VA for the leg weakness who added Cymbalta, but symptoms did not improve 2 nd Consultation with another neurologist in Bristol, TN believed symptoms may be due to Neurontin toxicity, Neurontin was discontinued but still no improvement

4 To further investigate the leg weakness and pain, patient underwent muscle biopsy that was negative Consultation by 3 rd neurologist was concerned about muscular etiology such as mild myositis versus myopathy, with superimposed diabetic polyneuropathy Patient went through inpatient rehabilitation that resulted in only minimal improvement

5 On peritoneal dialysis G.C. Also developed severe hyperphosphatemia with serum phosphorous levels >11 due to poor compliance with diet, phosphate binder therapy, and lower efficiency of peritoneal dialysis Calcium phosphorous product significantly elevated and serum intact PTH >500 G.C. was converted from peritoneal dialysis to hemodialysis

6 In the days following G.C. developed areas of skin necrosis with eschar in the folds of the skin of lower extremities and an ulcerating lesion with secondary infection over medial aspect of his calf Patient developed hard edema of his lower extremities, especially medial aspects of his thighs and calves Feet were warm and not cyanotic Areas of skin necrosis with black eschar were noted in Right thigh skin folds and Left popliteal area Ulcerated area of skin necrosis medial aspect Left calf Area of erythema developing over the Right thigh medial aspect

7 PMH: ESRD, undefined etiology Assumed secondary to diabetic nephropathy &/or secondary to focal glomerular sclerosis due to morbid obesity Nephrotic range proteinuria as high as 11 g Recently converted from peritoneal dialysis to hemodialysis Secondary hyperparathyroidism Severe hyperphosphatemia and markedly increased calcium, phosphorous products while on peritoneal dialysis Arterial calcifications noted on radiographic examination of knees

8 PMH: Anemia of chronic renal failure Refractory to erythropoietin stimulating therapy Insulin-requiring type 2 DM, diagnosed in 1997 Peripheral neuropathy Nephrotic range proteinuria Hypertension, long-standing Dyslipidemia GERD Suspected OSA Bilateral lower extremity weakness believed to be due to severe myopathy, most likely diabetic

9 PMH: Febrile nonhemolytic transfusion reaction, February 2010 Streptococcal pharyngitis, April 2010 Morbid obesity with BMI greater than 40

10 PSH: Incision and drainage of abscess, Left groin Placement of tunneled hemodialysis catheter, Right internal jugular vein by Dr Ajkay in November 2010 Placement of peritoneal dialysis catheter by laparoscopic assistance, by Dr. Grady Stephens on October 14, 2009 Right quadriceps muscle biopsy by Dr Oon May 18, 2010 Placement of Tunneled hemodialysis catheter, June 2010

11 Allergies: None Known Meds: Amlodipine 10 mg daily Apidra 15 U SQ qac Lantus 40 U SQ at bedtime Atenolol 100 mg daily Colace 100 mg BID Diovan 320 mg daily Lipitor 80 mg bedtime Lortab 10/500 q6h PRN Nephro-Vite 1 tab daily PhosLo 667 mg 3 tablets with each meal Prostat 30 mL PO TID Protonix 40 mg daily Tekturna 300 mg daily

12 FH: HTN and heart disease in his parents. Father was also diabetic SH: Married and lives at home with his spouse, who is a LPN. He chews tobacco occasionally but does not smoke. No history of alcohol or drug abuse. He worked in the mines for ~24 years. He was a high school football player

13 ROS: Chronically overweight, increased weight while on peritoneal dialysis, however has decreased by over 50 lbs since the conversion to hemodialysis Chronic parasthesias in lower extremities distally, now significant weakness of his lower extremities with limited functioning, able to stand up only for few minutes Minimal urine output

14 PE: Morbidly obese, weight 300 lbs, Caucasian male with marked central adiposity Alert and oriented to person, place, and time T 98.5, HR 90, RR 20, BP 154/90 HEENT: NC, AT. Conjunctiva pale, sclerae anicteric. EMOI, oral mucous membrane are moist. Neck: Veins are flat. No thyromegaly or carotid bruits Heart: RRR Lungs: CTA bilaterally Abdomen: Soft, obese, nontender. Tenckhoff peritoneal catheter in situ Extremities: hard edema of his lower extremities, especially medial aspects of his thighs and calves. Areas of skin necrosis with black eschar are noted in right thigh skin folds and L popliteal area. Ulcerated area of skin necrosis medial aspect Left calf that is infected, area of erythema developing over Right thigh medial aspect





19 Labs: BMP: Sodium 140, potassium 4.0, Chloride 97, CO2 34, BUN 19, Creatinine 4.1, Glucose 114, Calcium 8.7, GFR 16.5 Phosphorous: 3.9 CBC: WBC 11.4, Hgb 8.9, Hct 27.4, Plt 239 Aerobic culture Wound L leg—Morganella morganii, PTH 333

20 Radiology: CXR: Resolution of CHF CT A&P: Cholelithiasis, Atheroscleortic vascular disease, mild periaortic adenopathy NM Bone Imaging: Foci of increased activity within the posterior and posterolateral left ribs as described likely representing old fractures. X-ray Right Knee: Mild osteoarthritic changes involving the lateral joint compartment of the knee. Extensive arterial calcification noted. X-ray Left knee: Mild osteoarthritic changes involving the lateral joint compartment of the knee. Extensive arterial calcification noted.

21 Assessment: Necrotizing skin lesion and hard edema of lower extremities secondary to Calciphylaxis Anemia of CRF, on Epogen Refractory to Epogen therapy, most likely due to chronic inflammation Secondary hyperparathyroidism Recent intact PTH 333 and serum phosphorous levels improved ESRD, on hemodialysis Type 2 DM, insulin requiring

22 Calciphylaxis Rare and serious disorder Systemic medial calcification of the arterioles that leads to ischemia and subcutaneous necrosis One of several types of extra-osseous calcification (which also include intimal, medial, and valvular calcifications) may occur in ESRD patients Passive mineralization of serum calcium and phosphate crystals Syndrome of vascular calcification, thrombosis, and skin necrosis Almost exclusively in patients with Stage 5 chronic kidney disease (occurs in 1 to 4% of affected patients)

23 Pathogenesis: Systemic anaphylactic reaction Pathogenesis is complex and yet to be determined Abnormalities in mineral metabolism are thought to be a passive precipitation that predispose ESRD patients to vascular and soft tissue calcifications Reduction in the arteriolar blood flow is a consequence of intimal fibrosis associated with the calcifications

24 Pathogenesis: Vascular endothelial injury and dysfunction result in cutaneous arterioler narrowing and hypercoagulable state producing frank tissue infarction Hyperparathyroidism, active vitamin D administration, hyperphosphatemia, and an elevated plasma calcium x phosphate product are implicated in the genesis of calciphylaxis

25 Patients at higher risk: Females undergoing dialysis have higher risk of developing calciphylaxis ESRD patients with obesity (BMI>30) may lead to excess stress on dermal and hypodermal arterioles, resulting in focally dystrophic calcification on arterioles Increase in phosphorous levels among those with ESRD Administration of certain medicines to ESRD patients including: warfarin, calcium based binders and vitamin D analogues, and systemic corticosteroids Presence of AI disorders in ESRD patients Non-uremic calciphylaxis is noted in patients with primary hyperthyroidism, breast cancer (treated with chemotherapy), liver cirrhosis (due to alcohol abuse), cholangiocarcinoma, Crohn's disease, rheumatoid arthritis (RA), and systemic lupus erythematosus (SLE).

26 Clinical Manifestations: Areas of excruciatingly painful ischemic necrosis that usually develop on areas with greatest adiposity including abdomen, buttock, and thigh Violaceous, painful, plaque-like subcutaneous nodules Initial purpuric plaques and nodules subsequently progress to ischemic/necrotic ulcers with eschars that often become superinfected Ischemic changes are reflection of medial arteriolar calcification and subintimal fibrosis leading to luminal narrowing, diminished blood flow, hypoxia, and procoagulant state When vascular thrombosis is advanced, the result is necrotic ulcer with eschar that is clinically evident

27 Laboratory and radiographic manifestations: No specific laboratory findings Some patients will have elevated PTH, phosphorous, calcium, and calcium-phosphorous product Plain radiographs, high resolution CT scans, and mammographic techniques can distinguish calcium from surrounding non-calcium containing radiolucent tissues

28 Evaluation and Diagnosis: Suspect in patients with skin lesions characterized by painful, nonulcerating subcutaneous nodules or plaques, nonhealing ulcers, and/or necrosis, which are most commonly present in the thigh and areas of increased adiposity Also may be increasing Ca X P product and PTH levels in ESRD patients especially in setting of medication noncompliance &/or concurrent warfarin use Sensitivity and specificity of biopsy has not been determined 3-phase bone scan may be useful in diagnosis: positive scan suggests presence of calcifications in subcutaneous nodules or non-ulcerating lesions in viable tissue

29 Prevention and Treatment: HIGH mortality No controlled prospective studies that compare different treatment strategies Plan should include: Aggressive program of wound care and adequate pain control Avoidance of local tissue trauma, including subcutaneous injections Correction of underlying abnormalities in plasma calcium & phosphorous concentrations, lowering Ca X P product below 55 mg2/dL2 in ESRD patients

30 Treatment: Mainly supportive 1 st step: normalize serum calcium and phosphate concentrations with dietary modification and phosphate and calcium binders If calcium and phosphate levels remain high, consider parathyroidectomy, especially if parathyroidism in present

31 Treatment: Increase sessions of renal replacement therapy in ESRD patients with inadequate clearance Role of surgical debridement controversial because removal of necrotic tissue exposes vital subcutaneous tissue to bacteria and increases risk of sepsis Sodium Thiosulfate: showed reduction of pain and inflammation and improved healing of ulcerative lesions

32 Prognosis: Lethal disease that carries high morbidity and mortality Sporadic so prospective studies regarding exact outcomes associated with calciphylaxis do not exist Response to any therapeutic regimen appears to be poor and there remains high mortality regardless of efforts to treat the underlying disordered mineral metabolism Infection primary cause of high mortality (up to 58% in one report) Ulceration has high mortality >80% Estimated one-year survival 45.8%

33 Resources: Wikipedia, Calciphylaxis Noah S. Scheinfield, MD, JD Skin Disorders in Older Adults: Cutaneous Ulcers, Part 1 Counsultant Vol. 51 No. 8 August 2011, Pages 565-570 Peter W Santos, DO, J Edward Hartle, II, MD, L Darryl Quarles, MD Calciphylaxis rce=search_result&search=calciphylaxis&selectedTitle =1%7E14 Peter W Santos, DO, J Edward Hartle, II, MD, L Darryl Quarles, MD rce=search_result&search=calciphylaxis&selectedTitle =1%7E14

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