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SURGERY GRAND ROUNDS: CASE PRESENTATION

Maria B Albuja-Cruz, MD HISTORY PRESENT ILLNESS

 04/14/09: Mr. W, 43 y/o M  Few weeks of vague complaints: fatigue, malaise, , cough and loss of appetite  Symptoms gradually worsen  L sided 4/10 crampy  Abdominal distention, intermittent BRBR, + , weight loss (10 pounds)  CXR large amount of free air PAST MEDICAL HISTORY

 Non-ischemic cardiomyopathy 2/2 cardiac sarcoidosis  Refractory paroxysmal V-tach  Complete AV block  OSA on positive airway pressure  Pulmonary sarcoidosis  Amiodarone induced lung toxicity  HTN  PAST SURGICAL HISTORY

 Cardiac ablation

 Placement AICD PAST SOCIAL HISTORY/PAST FAMILY HISTORY/ ALLERGIES

 Do not smoke

 Drinks alcohol socially

 Denies

 NKDA MEDICATIONS  On chronic immunosuppressants:  Cellcept 1500mg BID  Prednisone 40 mg daily  On chronic anticoagulation : Coumadin 5mg daily  ASA 81mg daily  Lipitor 10mg PO daily  Cozaar 25mg PO daily  Coreg 50 mg PO BID  Lasix 20 mg PO every other day  Amiloride/HCTZ 5/50mg PO daily  Dapsone  Fosamax  Citalopram PHYSICAL EXAM

 36.5 107/68 87’ 18’ 90% RA  NAD  Cushingoid  : no scars, distended, +BS, soft, very minimal TTP. No rebound. No guarding. No . LABORATORY

 WBC 11.2 (Seg 87%) Hct 36.5 Plt 182  Cr 1.5 (1.1-1.3)  Bilirubin 3.9  INR 3.1  ABG 7.42/ 35 / 82 / 23 / 90% / -0.9  CRP 6.2

DIFERENTIAL DIAGNOSIS

 Perforated viscus?:

 Perforated ulcer?

 Perforated ?

 Perforated ?  Chilaiditi’s sign/syndrome?5,6 MANAGEMENT

 Broad spectrum IV antibiotics (Zosyn)  IVF  Transfused 2 units FFP & Vitamin K IV  OR PROCEDURE

 EXPLORATORY LAPAROTOMY  FINDINGS:

 NO EVIDENCE OF FREE FLUID OR

Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn Courtesy of Dr. Christopher Raeburn PERITONEAL FLUID CULTURES: Negative for any microorganism

Courtesy of Dr. Christopher Raeburn HISTOPATHOLOGY REPORT  INTESTINALIS

Courtesy of the Department of Pathology UCH HOSPITAL COURSE

 Uneventful

 Discharge POD# 6

 Tolerating regular diet

 Good bowel function PNEUMATOSIS CYSTOIDES INTESTINALIS

 First described in 1730 by Du Vernoi8  Multiple gas-filled within the wall1,7  Rare disease8  Mostly benign  Affects men more commnly7  Peak incidence 30-50 years of age7 PNEUMATOSIS CYSTOIDES INTESTINALIS

 33% mortality rate7  75% mortality necrotic bowel8  Primary 15% or secondary 85%8,9  Significance of PI depends on the nature and severity of the underlying condition1 St. Peter, S. D. et al. Arch Surg 2003;138:68-75 ETIOLOGIC FACTORS

St. Peter, S. D. et al. Arch Surg 2003;138:68-75 CLINICAL MANIFESTATION

 Most common symptoms1:

 Diarrhea

 Bloody stools

 Abdominal pain

 Weight loss

 Tenesmus DIAGNOSIS

 CT  technique of choice9  CT most sensitive9  Distinguish PI from intraluminal air or submucosal fat1,9 Soyer, P. J Radiol. 2008 Dec;89(12):1907-20 Clin Gastroenterol Hepatol. Aug 2009;7(8):A32 DIAGNOSIS

 Differentiation of benign from fulminant PCI10

 Portal or mesenteric venous gas

 PI + PVG have high fatality rate

 70% PI + PVG have bowel ischemia13 St. Peter, S. D. et al. Arch Surg 2003;138:68-75 DIAGNOSIS

 Differentiation of benign from fulminant10,16

 Portal or mesenteric venous gas

 Focal or diffusely fluid-filled bowel segments

 Thickening of the bowel wall

 Thrombosis SMA

 Absence or intense mucosal enhancement

 Free fluid Pneumatosis Cystoides Intestinalis: Radiographic and Endoscopic Features. Clin Gastroenterol Hepatol. Aug 2009;7(8):A32 MANAGMENT

 Associated illness inciting PI1  Management: level II-3/III clinical evidence13  Oxygen therapy1,2,3,10  Bowel rest10,11  TPN10,11  Antibiotics - Metronidazole10,11,14 Management and Outcome of Pneumatosis Intestinalis12

 Morris, Department of Surgery-OHSU  Retrospective review (2000-2007)  97 pts 3 management strategies:  52% non-operative (NGT/bowel rest)  Mortality rate= 6%  33% operative.  Mortality rate= 16%  13% nontherapeutic exploratory laparotomies  15% futile care. Mortality rate= 87%  Overall mortality rate= 22%  Patients admitted to GS service lower mortality rate  Approx 50% of PI can be managed non-operatively.  PI + portal venous gas may confer a higher mortality rate

The American Journal of Surgery, 2008, Volume 195, Issue 5, pp 679-683 in Adults: Management, Surgical, Indications, and Risk Factors for Mortality13  Greenstein, Department of Surgery-MSMC  Retrospective review (1996–2006)  Aim of study: identify factors significant for surgery/death  40 pts  Factors independently associated with surgical mngt  ≥ 60 years (p = 0.03)  Emesis (p = 0.01)  WBC > 12 c/mm3 (p = 0.03)  Sepsis highest risk of death  High mortality risk associated lactate ≥ 2 mmol/15

J Gastrointest Surg. 2007 Oct;11(10):1268-74 CONCLUSIONS

 Free air is not always an indication for surgery  Overall clinical picture of patient mandates the need for further evaluation/management  Presence of PI and PVG should rise high concern  Need for level I evidence base information regarding management REFERENCES

1. St. Peter SD, Abbas MA, Kelly KA. The spectrum of pneumatosis intestinalis. Arch Surg 2003; 138: 68–75 2. Gruenberg JC, Batra SK, Priest RJ. Treatment of pneumatosis cystoides intestinalis with oxygen. Arch Surg 1977; 112: 62–64 3. Simon NM, Nyman KE, Divertie MB, Rovelstad RA, King JE. Pneumatosis cystoides intestinalis: treatment with oxygen via close-fitting mask. JAMA. 1975;231:1354-1356 4. Rowe NM, Kahn FB, Acinapura AJ, Cunningham JN Jr. Nonsurgical pneumoperitoneum: a case report and a review. Am Surg. 1998;64:313- 322 5. Wong,V. Chilaiditi’s syndrome: A nonemergent cause of “free gas under diaphragm” The American Journal of Surgery, Volume 198, Issue 2, Pages e25-e26 6. Kamiyoshihara, M. Chilaiditi's Sign Mimicking a Traumatic Diaphragmatic . The Annals of Thoracic Surgery, 2009, Volume 87, Issue 3, pp. 959-961 7. Sakurai, Y. Pneumatosis cystoides intestinalis associated with massive free air mimicking perforated diffuse peritonitis. World J Gastroenterol 2008 November 21; 14(43): 6753-6756 REFERENCES 8. Hwang, J. Pneumatosis Cystoides Intestinalis with Free Intraperitoneal Air: A Case Report. American Surgeon; Apr2003, Vol. 69 Issue 4, p346-49 9. Soyer, P. Linear or bubbly: a pictorial review of CT features of intestinal pneumatosis in adults J Radiol. 2008 Dec;89(12):1907-20 10.Koop, A. Pneumatosis cystoides intestinalis with pneumoperitoneum and pneumoretroperitoneum following chemotherapy Abdom Imaging (1997) 22:395–397 11.Galm, O. Pneumatosis intestinalis following Cytotoxic or Immunosuppressive Treatment Digestion 2001;64:128-132 12.Morris, M. Management and outcome of pneumatosis intestinalis. The American Journal of Surgery, Volume 195, Issue 5, pp 679-683 13.Greenstein, J. Pneumatosis Intestinalis in Adults: Management, Surgical Indications, and Risk Factors for Mortality. J Gastrointest Surg. 2007 Oct;11(10):1268-74 14.Ellis BW, Symptomatic treatment of primary pneumatosis coli with metronidazole. Br Med J. 1980 Mar 15;280(6216):763-4 15.Hawn, T. Serum lactic acid determines the outcomes of CT diagnosis of pneumatosis of the Am Surg. 2004 Jan;70(1):19-23 16.Ho, L. Peumatosis Intestinalis in the Adult: Benign to Life-Threatening Cases. AJR, 2007;188:1604-1613 BIBLIOGRAPHY

 Attar, A. Pneumatosis Cystoides Intestinalis in Primary Intestinal Pseudo- obstruction: A nonsurgical cause of Pneumoperitoneum. Clinical gastroenterology and Hepatology, 2005;3:xxi  Braumann, C. Pneumatosis intesyinalis- A Pitfall for Surgeons? Scandinavian Journal of Surgery 2005, 94:47-50  Brill, S. Conservative Management of Pneumatosis Intestinalis and Massive Pneumoperitoneum in the : A Case Report. Ann R Coll Surg Engl. 2008 March; 90(2): 103  Cammarota, G. Free Peritoneal Gas Accumulation Caused by Pneumatosis Coli After Diagnostic Gastrointestinal Endoscopy. Clinical Gastroenterology and Hepatology. August 2009 (Vol. 7, Issue 8, Page A18)  Davila, M. Neutropenic : Current Issues in Diagnosis and Management. Current Infectious Disease Reports. 2007, 9:116-120  Davila, M. Neutropenic Enterocolitis. Current Treatment Options in Gastroenterology. 2006, 9: 249-55.  Hokama, A. Pneumatosis Cystoides Intestinalis: Radiographic and Endoscopic Features. Clin Gastroenterol Hepatol. Aug 2009;7(8):A32  Hoover, E "Avoiding laparotomy in nonsurgical pneumoperitoneum". The American journal of surgery, 1992, 164 (2), p. 99 BIBLIOGRAPHY

 Ito M, Horiguchi A, Miyakawa S. Pneumatosis intestinalis and hepatic portal venous gas. J Hepatobiliary Pancreat Surg 2008; 15: 334-337  Schulenburg, S. Pneumocystis cystoides intestinalis with pneumoperitoneum and pneumoretroperitoneum in a patient with extensive chronic graftversus- host disease. Bone Marrow Transplantation, (1999) 24, 331–333  Togawa, S. Evaluation of HBO2 therapy in pneumatosis cystoides intestinalis. Undersea Hyperb Med. 2004;31(4):387-93  Wu, B et al. An avoidable abdominal surgery: pneumatosis coli. The American Journal of Emergency Medicine, 2008, Volume 26, Issue 4, Pages 517.e1-517.e2 THANK YOU

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