Approach Considerations
No current guidelines for the treatment of lipofibromatous hamartoma (LFH) are available. The surgeon and the patient must weigh the potential risks and benefits of surgery against those of conservative management. Overall, treatment is based on the symptoms of the condition, considered on a case-by-case basis. Although some patients with LFH experience no neurologic or functional complications, others do. Medical management has no role, and surgery is reserved for those with neurologic deficits.
Because of the tumor’s intimate involvement with the nerve, LFH is often accompanied by a degree of neurologic morbidity. If the risk of nerve damage is low and nerve involvement is minimal, surgical debulking for cosmetic reasons can be undertaken. However, in the face of advanced nerve involvement, indications for intervention are progressive and unrelenting neurologic deficits.
Surgical Care
Surgical considerations
Several problems may complicate the case-by-case surgical approach to the treatment of LFH. One such problem involves the treatment of the symptoms of nerve compression, typically carpal tunnel syndrome (CTS). Although it is generally held that carpal tunnel decompression should be undertaken for relief of paresthesias, some authors strongly advocate waiting for the tumor to become symptomatic before surgery, mostly in younger children. [6, 36, 37, 38]
Another problem involves the management of cases associated with macrodactyly. The following options are available:
-
Staged debulking
-
Epiphysiodesis/epiphysectomy
-
No treatment
With regard to debulking, a review of eight cases found that three patients reported a decrease in mass size from 1-year to 3-year follow-up, four noted no change from 2 to 7 years out, and one reported an increase in tumor mass after surgery. [7]
In many cases, both neurolysis and excision of the main trunk of the median nerve can lead to abnormal two-point tactile discrimination and a loss of sensory distribution after operation in adult patients. [33, 39] Furthermore, microdissection of the median nerve has led to ischemic complications in one reported case and has been unsuccessful in others. [32, 33, 40] One case of successful median nerve excision in the presence of a Martin-Gruber anastomosis has been reported. [41, 42]
Amadio et al reported on significant debulking of tissue followed by distal digital nerve excision. [6] Because of the considerable cutaneous overlap in the hand, the authors suggested debulking followed by rotation of the excess skin from the dorsal aspect to the volar aspect of the affected digits to restore sensation. [6] Although this does not fully restore the dermatomal distribution of sensation, it does offer a degree of sensation that would have been lost either with no treatment or with nerve excision alone.
Perioperative management
Preoperative management, surgical planning, and postoperative management are all similar to those of CTS surgery. As with CTS surgery, follow-up can be performed on an outpatient basis. Patients can be seen at 1 week, by which time their pain should have subsided considerably. Restoration of nerve function and resolution of paresthesias vary from case to case.
Complications
Reports on the efficacy of nerve excision tumor debulking and microsurgical intraneural dissection have been mixed. [43] Two studies described a complete loss of neurologic function after complete excision, whereas nerve excision and grafting have yielded favorable results in children younger than 5 years. [6, 36, 37] Children may have a capacity for reeducation of nerve sensibility that adults lack. [6, 36, 37]
-
Intraoperative photograph shows lipofibromatous hamartoma and typical gross appearance. Multiple soft, gray-yellow lobulated masses (arrows) are present within epineural sheath of radial digital nerve of right index finger. Epineurium of digital nerve displays extensive perineural fibrosis. Image reprinted with permission from American Journal of Roentgenology.
-
Axial T1-weighted image of 40-year-old woman who experienced several years of paresthesias in her median nerve distribution and mass in her wrist. Note pathognomonic coaxial-cable appearance of involved nerve.
-
Coronal T1-weighted image of 40-year-old woman who experienced several years of paresthesias in her median nerve distribution. On coronal sectioning, lipofibromatous hamartoma is described as resembling spaghetti.
