Title: Tie Up Technique for Hemorrhageless Resection
of Diffuse Neurofibroma
Authors: Sachio Kouraba, MD, Taisuke Sakamoto, MD,
Cyu Kimura, MD, Akiteru Takeuchi, MD, Emi Funayama,
MD,
Yuhei Yamamoto, MD, Tomoko Yokoyama, MD,
Tamotsu Kamishima, MD, Masanobu Kumakiri, MD
Neurofibromatosis type 1 (NF1) is a common genetic disease
that affected one in every 2500 to 3000 live birth. NF1 is of
particular interest to the plastic surgeons, as it affects skin
with various clinical manifestations. Diffuse neurofibroma,
an uncommon but distinctive form, may produce gross
disfigurement such as elephantiasis neurofibromatosis.
Although the majority of patients require surgical procedure
for cosmetic and functional reasons, the associated vascular
malformations consist of excessive vascular fragility make most
surgeons hesitant to address them. In fact, surgical resection
of neurofibroma may cause life-threatening hemorrhage and
require massive transfusion.
To develop safe surgical procedure, we direct our attention
to the pathological characteristics of diffuse neurofibroma.
Clinicopathologically, the entire subcutis in diffuese
neurofibroma between superficial fascia and dermis is thickened
by firm, grayish tumor tissue. Despite its infiltrative growth,
it does not destroy the deep strauctures such as muscle. This
characteristics of diffuse neurofibroma means hand-grasped
pendulous tumor may not contain any of non-affected functional
structures.
First, these features were confirmed radiologically in a NF1
patient with elephantiasis neurofibromatosis on the trunk. The
patient, 28-year-old woman, has repeated history of massive
hemorrhage in surgical resection of elephantiasis
neurofibromatosis. She was admitted to hospital to treat
spontaneous massive hematoma within tumor on the back. On the
radiological examination of hematoma with computed tomography,
elephantiasis neurofibromatosis lesion next to hematoma was
grasped longitudinally with both hands by an inspector. The
grasped massive tumor with hands forms narrow pedicle without
muscle involvement.
On the basis of these findings, we developed “tie up” technique
for hemorrhageless resection of diffuse neurofibroma. The
details are as follows: (1) grasp up tumor to be excised, (2)
pierce through the base of grasped tumor with a needle and suture,
(3) tie up the base of tumor tightly 2 - 3 cm in width and repeat
it all over the tumor to be excised. After that, blood supply
to tumor is perfectly cut off and tumor resection may be completed
without bleeding. The wound is sutured tightly in subdermal
layer and closed with skin suture without drainage. All of
ligation sutures are removed after wound closure. Hemorrhage
in sutured wound after ligation suture removal may well controled
with simple bolus dressing.
We performed this procedure for four times in 3 NF1 patients
with a history of massive transfusion in resection of diffuse
neurofibroma. Counts of blood loss in each operations were 50g,
15g, 412g and 18g. No transfusion was needed in all patients.
Massive hemorrhage (412g) was experienced due to incomplete
ligation of tumor.