| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
J Thorac Cardiovasc Surg 2005;129:1457-1458
© 2005 The American Association for Thoracic Surgery
Brief Communication |
a Department of Surgery Hospital of the University of Pennsylvania, Philadelphia, Pa
b Department of Hematology, Hospital of the University of Pennsylvania, Philadelphia, Pa
c Easton Hospital, Easton, Pa
Received for publication September 29, 2004; revisions received October 30, 2004; accepted for publication November 4, 2004. * Address for reprints: Samantha Hendren, MD, Mount Sinai Hospital, Department of Surgery, 600 University Ave, Suite 451, Toronto, Ontario M5G 2J8, Canada (Email: shendren{at}mtsinai.on.ca).
Osteopetrosis is a rare genetic disorder characterized by a failure of osteoclast-mediated bone resorption and by hematologic abnormalities. A patient with osteopetrosis presenting with a distal esophagus cancer and secondary hypersplenism was surgically treated.
Clinical Summary
The patient is a 57-year-old man with osteopetrosis since childhood, complicated by multiple orthopedic fractures (Figure 1), osteomyelitis, splenomegaly, thrombocytopenia, and chronic anemia requiring routine blood transfusions. Several months before diagnosis, he was noted to have an increasing transfusion requirement. The hemoglobin fell to 2.6 g/dL, and upper endoscopy demonstrated bleeding from an adenocarcinoma at the gastroesophageal junction (Figure 2).
|
|
The patient tolerated the procedure well. His postoperative course was complicated by a pulmonary embolism, treated with a vena cava filter. He was discharged on postoperative day 10 eating a regular diet, with a dramatically reduced requirement for blood and platelet transfusion. The total utilization of blood products was 13 units of packed red blood cells, 13 units of platelets, and 2 units of fresh frozen plasma.
After discharge from the hospital, the patient’s quality of life was substantially improved owing to resolution of symptoms, as well as a durable elimination of his transfusion requirement. Approximately 18 months after his operation, weight loss and functional decline ensued due to liver metastases; 22 months after his operation, he died of progressive metastatic disease.
Comment
Although all patients with osteopetrosis have dense, fragile bones and obliteration of the marrow space, other manifestations of the disease vary. Osteopetrosis has traditionally been separated into 2 forms: infantile "malignant" and adult "benign."1 Clinically, the infantile form manifests with rickets-like bony changes, hepatosplenomegaly, progressive pancytopenia, cranial neuropathies, and susceptibility to infection.2 This disease is lethal in infancy or early childhood, except in some children treated with human leukocyte antigen-matched bone marrow transplantation.
The adult, benign form of osteopetrosis may be asymptomatic, or pathologic fractures and osteomyelitis may occur. Severe hematologic disturbances and hepatosplenomegaly are usually absent.3 The patient described here does not fit easily into the infantile or adult form of osteopetrosis and probably had an attenuated form of the infantile disease or an intermediate type of osteopetrosis.
Splenectomy has been used as a palliative procedure for patients with infantile osteopetrosis and hypersplenism since the 1960s.4 The primary concern with a splenectomy is the removal of a major site of extramedullary hematopoiesis in patients with little functioning bone marrow. To predict which patients will benefit from a splenectomy, one may use hematologic and radionuclide (bone marrow scintigraphy, red blood cell survival) studies to determine the relative contributions of decreased red cell production and increased destruction to a patient’s anemia and thrombocytopenia.2,4,5
Although this patient’s comorbidities substantially increased his risk for perioperative complications, we felt that surgery was the best treatment option because we believed that the patient’s severe bleeding and hypersplenism could be most effectively addressed with surgery. We also believe that surgery provides the best chance for cure in locally advanced esophageal cancer, while avoiding potential bone marrow toxicity from nonoperative cancer treatments. In this case, we decided on a left thoracoabdominal approach because we believed that the patient’s bleeding diathesis placed him at risk for excessive bleeding with blunt esophageal dissection. In addition, the thoracoabdominal approach provides superior exposure to the enlarged spleen compared with an anterior abdominal incision.
Additional considerations when operating on a patient with osteopetrosis include prevention of iatrogenic fractures and dental injuries, to which these patients are extremely susceptible, and perioperative correction of anemia and thrombocytopenia. Collaboration with a hematologist is essential. Adjuvant therapy was not prescribed because it was thought that the risk of hematologic suppression in this patient was too great to justify the potential benefit.
In summary, this is a report of esophagectomy and splenectomy performed for esophageal adenocarcinoma and secondary hypersplenism in a patient with osteopetrosis. With proper preparation and precautions, this high-risk patient underwent a major operation with favorable early outcome. Suggestions for perioperative management of the patient with osteopetrosis are made, and the palliative potential of splenectomy in osteopetrosis is highlighted.
References
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
| ANN THORAC SURG | ASIAN CARDIOVASC THORAC ANN | EUR J CARDIOTHORAC SURG |
| J THORAC CARDIOVASC SURG | ICVTS | ALL CTSNet JOURNALS |
