Abstract
BackgroundA large plexiform neurofibroma in patients with neurofibromatosis type I can be life threatening due to possible massive bleeding within the lesion. Although the literature includes many reports that describe the plexiform neurofibroma size and weight or strategies for their surgical treatment, few have discussed their possible physical or mental benefits, such as reducing cardiac stress. In addition, resection of these large tumors can result in impaired wound healing, partly due to massive blood loss during surgery.Case presentationA 24-year-old man was diagnosed with neurofibromatosis type I and burdened with a large plexiform neurofibroma on the buttocks and upper posterior thighs. The patient was 159 cm in height and 70.0 kg in weight at the first visit. Cardiac overload was indicated by an echocardiography before surgery. His cardiac output was 5.2 L/min with mild tricuspid regurgitation. After embolism of the arteries feeding the tumor, the patient underwent surgery to remove the neurofibroma, followed by skin grafting. Follow-up echocardiography, performed 6 months after the final surgery, indicated a decreased cardiac output (3.6 L/min) with improvement of tricuspid regurgitation. Because the blood loss during the first surgery was over 3.8 L, malnutrition with albuminemia was induced and half of the skin graft did not attach. Nutritional support to improve the albuminemia produced better results following a second surgery to repair the skin wound.ConclusionCardiac overload may be latent in patients with neurofibromatosis type I with large plexiform neurofibromas. As in pregnancy, the body may compensate for this burden. In these patients, one stage total excision may improve quality of life and reduce cardiac overload. In addition, nutritional support is likely needed following a major surgery that results in either an extensive skin wound or excessive blood loss during treatment.
Highlights
A large plexiform neurofibroma in patients with neurofibromatosis type I can be life threatening due to possible massive bleeding within the lesion
Cardiac overload may be latent in patients with neurofibromatosis type I with large plexiform neurofibromas
We suggest that removal of a large plexiform neurofibroma can be beneficial by reducing cardiac overload, as well as for preventing malignant change within the tumor, avoiding accidental massive bleeding in the tumor that can cause hemorrhagic shock, and improving a patient’s quality of life [9]
Summary
Cardiac overload may be latent in patients with large size plexiform neurofibromas, and patients may compensate for increased cardiac output, as observed in pregnancy. One stage total excision is presumed to be beneficial for improved quality of life and for reduction in any cardiac overload. The data before surgical treatments were taken one month before surgery, and post operation data were obtained 8 months after the resection surgery. These data indicate latent cardiac overload before surgery that has been improved by total resection of the plexiform neurofibroma. Pre op: before operation, Post op: after surgical treatments, IVSd: Interventricular septal end diastolic dimension, IVSs: Interventricular septal end systolic dimension, AoD: Aortic root diameter, LVDd: Left ventricular end diastolic dimension, LVDs: Left ventricular end systolic dimension, LAD: Left atrial dimension, LVPWd: Left ventricular end diastolic posterior wall dimension, LVPWs: Left ventricular end systolic posterior wall dimension, A valve: Aortic valve, M valve: Mitral valve, T valve: Tricuspid valve, P valve: Pulmonary valve, MR: Mitral regurgitation, TR: Tricuspid regurgitation, PR: Pulmonary regurgitation produces an extensive wound or when excessive blood loss has occurred during surgery
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