eMedicine Specialties > Gastroenterology > Systemic Disease

WDHA Syndrome: Follow-up

Author: Sandeep Mukherjee, MB, BCh, MPH, FRCPC, Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Consulting Staff, Section of Gastroenterology and Hepatology, Veteran Affairs Medical Center
Coauthor(s): Randall E Brand, MD, Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Richard K Gilroy, MBBS, FRACP, Assistant Professor, Medical Director of Liver Transplantation and Hepatology, Department of Internal Medicine, Kansas University Medical Center; Daniel Schafer, Department of Surgery, University of Nebraska Medical Center
Contributor Information and Disclosures

Updated: Jun 4, 2008

Follow-up

Further Inpatient Care

  • Preliminary data suggest that octreotide administered postoperatively is associated with a lower rate of complications, such as the development of pancreatic fistula. As a result, many surgical units routinely administer octreotide until the abdominal drains have been removed and the patient is eating normally.
  • Histamine 2 (H2)–receptor antagonists or proton pump inhibitors should be administered postoperatively to prevent hyperacidity because rebound hyperchlorhydria is common in the postoperative period.

Inpatient & Outpatient Medications

  • Octreotide should be prescribed to treat the debilitating diarrhea frequently observed in these patients.
  • Antidiarrheals may be useful adjuncts for treating diarrhea in patients who are not responding to octreotide. Rebound diarrhea can be difficult to treat.

Deterrence/Prevention

  • No effective therapy is available for patients who are not cured surgically. Neither somatostatin nor interferon alfa has significant tumoricidal properties, although they may have a tumoristatic effect.

Complications

  • Dehydration
  • The most common cause of death in WDHA syndrome is acute renal failure associated with hypokalemia. The typical renal lesion observed in these patients is a vacuolar tubular nephropathy.
  • Non–anion gap metabolic acidosis
  • Perianal skin irritation from severe diarrhea
  • Poor quality of life from severe diarrhea

Prognosis

  • A proper assessment of the prognosis is limited because of the rarity of the condition.
  • Tumors that cannot be resected completely are associated with a 1-year survival rate of approximately 40%.
  • A paper from the Mayo Clinic, Jacksonville, reported 5- and 10-year survival rates for malignant tumors of 88% and 25%, respectively.
  • Soga and Yakuwa (1998) also observed encouraging long-term outcomes in an evaluation of 241 patients with WDHA syndrome.7 The 5-year survival rate was 94.4% in 46 patients without metastases and 59.6% in 43 patients with metastatic disease.7

Patient Education

  • Patients should be advised to seek treatment at a center with expertise in this field. Patients need to be aware that dehydration and acute renal failure are significant complications, and they must seek hospitalization if they are not doing well at home.

Miscellaneous

Medicolegal Pitfalls

  • Delay in diagnosis: This is not uncommon because of the rarity of the condition and because the initial presentation may masquerade as other disease processes.
  • Education: Once the diagnosis is established, patients and their families need to be educated about the disease and treatment approach.
  • Referrals: Patients should be advised to seek treatment at a major referral center that has expertise in this field. This is true particularly for the subset of patients with nonresectable disease who may be candidates for innovative, potentially life-saving treatment protocols.
  • Consultations: Because treatment of this condition can be complex, patients benefit from a multidisciplinary team of specialists starting at the time of the initial diagnosis.
  • Endoscopic ultrasound: This procedure should be performed only by trained physicians.
  • Pneumococcal vaccination: This should be administered to all patients who undergo distal pancreatectomy and splenectomy.
 


More on WDHA Syndrome

Overview: WDHA Syndrome
Differential Diagnoses & Workup: WDHA Syndrome
Treatment & Medication: WDHA Syndrome
Follow-up: WDHA Syndrome
References
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Further Reading

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Keywords

pancreatic cholera; Verner-Morrison syndrome; VIPoma syndrome; watery diarrhea, hypokalemia, and achlorhydria syndrome; vasoactive intestinal peptide; VIP; non–beta pancreatic islet cells; dehydration; hypokalemia; ganglioneuroma; ganglioneuroblastoma; hypercalcemia; hyperglycemia; hypochlorhydria; flushing; multiple endocrine neoplasia type 1 syndrome; MEN-1 syndrome; Werner syndrome; watery diarrhea, hypokalemia, and hypochlorhydria syndrome; WDHH syndrome; amine precursor uptake and decarboxylation tumors; APUD tumors; APUDomas

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Contributor Information and Disclosures

Author

Sandeep Mukherjee, MB, BCh, MPH, FRCPC, Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center; Consulting Staff, Section of Gastroenterology and Hepatology, Veteran Affairs Medical Center
Sandeep Mukherjee, MB, BCh, MPH, FRCPC is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Coauthor(s)

Randall E Brand, MD, Associate Professor, Department of Internal Medicine, Section of Gastroenterology and Hepatology, University of Nebraska Medical Center
Randall E Brand, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Pancreatic Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Richard K Gilroy, MBBS, FRACP, Assistant Professor, Medical Director of Liver Transplantation and Hepatology, Department of Internal Medicine, Kansas University Medical Center
Disclosure: Nothing to disclose.

Daniel Schafer, Department of Surgery, University of Nebraska Medical Center
Disclosure: Nothing to disclose.

Medical Editor

Manoop S Bhutani, MD, FACG, FACP, Professor, Department of Medicine, Division of Gastroenterology, Director, Center for Endoscopic Ultrasound, Co-Director, Center for Endoscopic Research, Training and Innovation, University of Texas Medical Branch at Galveston
Manoop S Bhutani, MD, FACG, FACP is a member of the following medical societies: American Association for the Advancement of Science, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Institute of Ultrasound in Medicine, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

BS Anand, MD, Department of Internal Medicine, Division of Gastroenterology, Professor, Baylor University College of Medicine
BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law Medicine and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

 
 
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