Giant facial neurofibroma leads to disfigurement and functional and neurological deficits. Surgical resection is the mainstay of treatment and poses a great challenge to the surgeon with regard to the restoration of the defects arising from tumour resection. The cases of three male and three female patients diagnosed with giant facial neurofibroma, who underwent radical resection and reconstruction with soft tissue free flaps between 2008 and 2015, were analyzed retrospectively. Clinical data including patient sex, age, preoperative embolization of the nutrient artery, volume of blood loss, type and size of flaps used for reconstruction, and complications were recorded. Three of the six patients underwent preoperative embolization of the nutrient artery. The average volume of blood loss was 2850 ml. Reconstruction was performed with anterolateral thigh flaps in four patients and latissimus dorsi myocutaneous flaps in two patients. All free flap reconstructions were successful. Partial necrosis of the scalp and wound dehiscence occurred in one patient each. All complications were managed successfully. In conclusion, the soft tissue free flap is a good choice for the coverage of defects after giant facial neurofibroma resection. Multi-disciplinary treatment should be strengthened to minimize the risks of complications, as well as improving quality of life.
Neurofibromatosis type 1 (NF1) is a common autosomal domain disease and occurs in approximately 1 in 3000 live-births. The clinical manifestations of NF1 include the presence of numerous cutaneous café au lait spots, axillary freckling, and neurofibromas. These tumours usually involve multiple nerve fascicles or the branches of major nerves and often lead to soft tissue overgrowth, resulting in dysfunction, pain, and disfigurement.
The management of progressive symptomatic neurofibromas, especially giant facial neurofibromas, is very challenging. Surgical resection is still a mainstay of treatment, as these tumours are radio-resistant and respond poorly to chemotherapy. However, radical tumour resection usually results in large defects, which are very challenging to restore. NF1 tumours are highly vascularized and life-threatening haemorrhage can occur during surgery, thus reconstructive surgery is extremely challenging in these cases.
This article presents the cases of six patients with giant facial neurofibroma treated in the authors’ institution. Soft tissue free flaps were used to restore the defect after extensive tumour resection. The aim is to emphasize the importance of multidisciplinary cooperation in order to minimize the risks of the operation and in particular to avoid the severe complications caused by life-threatening haemorrhage.
Patients and methods
This was a retrospective study. The cases of six patients (three male and three female) who were diagnosed with giant facial neurofibroma and underwent reconstruction with soft tissue free flaps to restore the defect after tumour resection were reviewed. These patients were treated at the Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine between September 2008 and December 2015. The mean age of the patients was 26.7 years (range 24–29 years). These giant tumours involved multiple sites in the head and neck and the defects could not be closed after tumour resection. Two of the patients underwent an interventional embolization of the external carotid artery before surgery, while one patient underwent temporal artery embolization before surgery. Anterolateral thigh (ALT) flaps were used to reconstruct the defect in four patients and latissimus dorsi myocutaneous flaps were used in two patients. All of these patients were assessed by a multidisciplinary tumour board that included head and neck surgeons, plastic surgeons, otolaryngologists, ophthalmologists, anaesthetists, and neurosurgeons.
The following clinical data were recorded: patient sex, age, preoperative embolization of the nutrient artery, volume of blood loss, type and size of flaps used for reconstruction, and postoperative complications.
The clinical data recorded in this study are presented in Table 1 .
|Patient||Age, years/sex||Artery embolized||Blood loss, ml||Type of flap||Size of flap, cm||Complications|
|1||29/F||External carotid artery||5000||Latissimus dorsi flap||30 × 9||Partial necrosis of the scalp|
|2||28/M||No||3400||ALT flap||25 × 9||No|
|3||24/M||No||2300||ALT flap||15 × 8||Wound dehiscence|
|4||26/F||External carotid artery||2000||ALT flap||20 × 7||No|
|5||27/M||No||2600||Latissimus dorsi flap||25 × 10||No|
|6||26/F||Temporal artery||1800||ALT flap||16 × 10||No|
Four of the six patients had tumour involvement of the eyes, but visual acuity was not affected. Two of the six patients had involvement of the ears without evidence of hearing impairment. Four patients underwent ALT flap reconstruction (patients 2, 3, 4, and 6; Table 1 ), while two patients underwent latissimus dorsi myocutaneous flap reconstruction (patients 1 and 5; Table 1 ). Patient 1 also underwent scalp re-transplantation to cover a large defect. Preoperative embolization of the nutrient artery was performed in three patients: the external carotid artery in two patients (patients 1 and 4; Table 1 ) and the temporal artery in one patient (patient 6; Table 1 ). The average volume of blood loss was 2850 ml; blood loss ranged from 1800 ml to 5000 ml.
All six free flaps were successful. Partial necrosis of the grafted scalp occurred in patient 1, and debridement of the necrotic tissue was performed. In patient 3, wound dehiscence was found in the anterior part of the flap and a minor surgical procedure was performed to manage this complication. No tumour recurrence was recorded in the six patients and no patient died during routine follow-up (range 3–84 months).
Case 1 ( Figs 1–3 )
A 29-year-old female presented with a progressive mass involving the right face of 22-year duration. A biopsy had been performed at the local hospital and the pathological diagnosis was plexiform neurofibroma. Physical examination revealed a non-tender, firm swelling measuring approximately 25 cm × 18 cm located in the right orbitotemporal region ( Fig. 1 a–c). The patient was unable to open her right eye due to the overhanging folds affecting the right eyelid, and the eye was pulled inferiorly. However, her visual acuity was not affected. The right ear was also involved, without hearing impairment. Magnetic resonance imaging (MRI) showed a well-defined lesion with homogeneous signal intensity in the subcutaneous plane of the right side of the face ( Fig. 2 a and b).
The patient underwent preoperative angiography ( Fig. 2 c), and embolization of the right external carotid artery was performed before excision surgery to reduce intraoperative bleeding and facilitate tumour excision ( Fig. 2 d). Autologous blood storage was performed preoperatively (400 ml of blood was obtained) and an additional 3000 ml blood was also prepared.
Autologous blood transfusion and hypothermic anaesthesia were used to reduce neurological ischaemia intraoperatively. Incisions were made over the normal tissue as much as possible in order to minimize blood loss. Bleeding was controlled by pressure. Despite all measures taken, there was a 5000-ml blood loss. The patient received 4000 ml packed red blood cells and 2000 ml packed plasma. The resection of the large tumour included the involved right ear and resulted in a large soft tissue defect ( Fig. 3 a and b). A latissimus dorsi flap was used to repair the defect ( Fig. 3 d). The size of the flap was 30 cm × 9 cm and it was islanded into two parts with the same pedicle. Primary closure of the donor site was performed. Folding and rotation of the flaps resulted in partial closure of the defect, while the right side of the skull remained exposed. A 10 cm × 10 cm section of the remaining scalp obtained from the resected tumour was then mobilized; the residual tumour under it was shaved off ( Fig. 3 c). The scalp was closed and a compression dressing was placed ( Fig. 1 d). The resected right ear was not reconstructed. Suspension of the upper eyelid was performed by an ophthalmologist. There were no intraoperative complications. The patient was discharged to the ICU after an operation lasting 15 h.
The compression dressing was removed on day 15 postoperative. Most of the surgical wound had healed, but areas of necrosis were evident on the implanted scalp. Debridement of the necrotic tissue was performed behind the bed, which subsequently granulated ( Fig. 1 e–h).
The patient was satisfied with her appearance and made an uneventful recovery. She was discharged 3 weeks postoperative and was followed up in the clinic with clinical examinations.
Case 2 ( Figs 4 and 5 )
A 28-year-old male presented with a mass in the left orbitotemporal region that had gradually increased in size over a period of 20 years. The pathological diagnosis was neurofibroma. On physical examination, the mass was found to measure approximately 15 cm × 20 cm in size ( Fig. 4 a). The left eyelid was affected by the tumour, which had pulled the eyelid inferiorly, but the patient’s visual acuity was normal ( Fig. 4 b and c).