eMedicine Specialties > Pulmonology > Idiopathic Lung Disorders

Milroy Disease: Follow-up

Author: Raphael J Kiel, MD, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Infectious Diseases Division, William Beaumont Hospital
Contributor Information and Disclosures

Updated: Oct 16, 2008

Follow-up

Further Outpatient Care

  • See patients monthly to teach the parents proper noninvasive methods of controlling swelling of the extremity.
  • Instruct parents to bring the child in for evaluation if fever associated with redness and further swelling of the extremity occurs.

Transfer

  • Generally, treatment of the patient can occur in a general hospital setting. If difficulty with proper intravenous access occurs, consider transfer to a pediatric hospital.

Deterrence/Prevention

  • Milroy disease is a genetically transferred illness. Watch for the development of complications of the disease (eg, cellulitis, lymphangiosarcoma).
  • Prenatal screening for the presence of an abnormal gene sequence may provide parents with additional information on the possibility of their child developing Milroy disease.

Complications

  • Recurrent cellulitis
  • Bacteremia
  • Lymphangiosarcoma
  • Sarcoma
  • Protein-losing enteropathy
  • Chylothorax
  • Chylous ascites

Prognosis

  • Long-term prognosis is excellent.

Patient Education

  • Milroy disease is a genetically transmitted swelling of an extremity that is present at birth and is usually not progressive. Patients develop lymphedema and can experience recurrent cellulitis, but the disease does not decrease their lifespans.

Miscellaneous

Medicolegal Pitfalls

  • Physicians should be aware of Milroy disease and recognize the condition's associated familial pattern. Extensive searching for a secondary cause of this condition could result in undue morbidity for the patient.
  • Physicians should be aware of the possibility of recurrent cellulitis associated with this disease, and they should establish a treatment plan with the parents that protects the child from potential complications.
 


More on Milroy Disease

Overview: Milroy Disease
Differential Diagnoses & Workup: Milroy Disease
Treatment & Medication: Milroy Disease
Follow-up: Milroy Disease
References
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References

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Further Reading

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Keywords

Milroy disease, congenital lymphedema, lymphedema congenita, noninfectious hereditary elephantiasis, autosomal dominant lymphedema, lymphatic obstruction, fibrosis, cellulitis, Meige disease, lymphedema tarda, lymphedema praecox

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Contributor Information and Disclosures

Author

Raphael J Kiel, MD, Associate Professor of Medicine, Wayne State University School of Medicine; Consulting Staff, Infectious Diseases Division, William Beaumont Hospital
Raphael J Kiel, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Geriatrics Society
Disclosure: Nothing to disclose.

Medical Editor

Sat Sharma, MD, FRCPC, Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St. Boniface General Hospital
Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, and World Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

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

Managing Editor

Gregg T Anders, DO, Medical Director, Great Plains Regional Medical Command , Brook Army Medical Center; Clinical Associate Professor, Department of Internal Medicine, Division of Pulmonary Disease, University of Texas Health Science Center at San Antonio
Gregg T Anders, DO is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society
Disclosure: Nothing to disclose.

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint Louis University School of Medicine
Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians
Disclosure: Nothing to disclose.

Chief Editor

Zab Mosenifar, MD, Director, Division of Pulmonary and Critical Care Medicine, Director, Women's Guild Pulmonary Disease Institute, Executive Vice Chair, Department of Medicine, Cedars Sinai Medical Center; Professor of Medicine, David Geffen School of Medicine at UCLA
Zab Mosenifar, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Federation for Medical Research, and American Thoracic Society
Disclosure: Nothing to disclose.

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