Hereditary Hemorrhagic Telangiectasia A New
Dermoplasty Technique
G. Patrick
Bridger, FRCS, FRACS,
dermoplasty technique is described that allows both sides of the nasal septum to be covered with a single split-skin graft. This graft is designed with a narrow central dermal band that covers the septal dorsum and can be buried under the alar tissues. Exposure is obtained via a modified external rhinoplasty approach. The operation has been successfully performed on 11 patients, and in nine of them the graft take was complete. (Arch Otolaryngol Head Neck Surg. 1992;118:992-993) \s=b\ A new
is
incidence of hereditary hemorrhagic telangiectasia Thedifficult shows establish; however, surgeon collects the to
the
who
condition soon many patients. afflicted People by this disease are frequently treated for life-threatening nosebleeds. Nevertheless, it is a topic that is rarely featured at academic meetings and seldom appears in the literature. The epistaxes start without warning, can be profuse and bilateral, and occur several times each day. The destruction to quality of life is considerable. Hereditary hemorrhagic telangiectasia, or Rendu-OslerWeber disease, is a simple autosomal dominant trait that is manifested clinically by multiple telangiectases. The es¬ sential lesion is an abnormal subepidermal thin-walled ar¬ teriovenous fistula composed of a single layer of endothelial cells. Submucosal telangiectases are seen throughout the nasal mucosa, especially on the anterior septum, and it is these vessels that are easily traumatized and cause most nosebleeds. The most successful definitive operative procedure is the dermoplasty, which was first described by Saunders1 in 1960. In this operation, the diseased mucosa covering the anterior half of the septum is removed with a curette, leaving the vascular bed of perichondrium, onto which a split-skin graft is placed. Both sides of the septum are op¬ erated on at the same time, and usually all work is done through a speculum. The graft covering the septum and floor is only anchored anteriorly and is held in place with careful packing. When the telangiectases are widespread, a separate sleeve of skin graft is applied to the lateral na¬ sal surface. Many authors have testified to the success of this procedure. Most patients, however, will continue to bleed but not so frequently or severely. I have operated on seven patients using the classic Saunders' dermoplasty technique. The approach has proved to be difficult and the graft take unpredictable. The access provided by either a nasal speculum or a small alar-releasing incision is inadequate. General anesthesia supplemented by topical and intramucosal adrenalin selan
dorn controls the bleeding, which constantly obscures the operative field. The intranasal placement of the skin graft is imprecise and tenuous. Among the seven patients, there were two cases in which the graft failed to take and only a few cases in which the whole graft survived. As this is a familial condition, there are often several relatives who require treatment. An inadequate operation that does not rectify the bleeding problem will result in other family members deciding against surgery. Similarly, a successful procedure encourages other sufferers to seek a surgical solution. A new dermoplasty technique is described that facilitates graft placement and survival.
interest in
Accepted From the
for publication June 2, 1992. Department of Otolaryngology, The Prince of Wales
pital, Sydney,
Hos-
Australia. to Suite 1, 21 Kitchener Parade, Bankstown NSW 2200, Australia (Dr Bridger).
Reprint requests
DLO
SURGICAL
TECHNIQUE
With this technique, a single piece of split skin is used to drape both sides of the nasal septum, replacing all of the involved mucosa. The area to be grafted is measured and then liberally mapped out on the donor site, which is usually the thigh. Before the graft is taken, a central band, approximately 1 cm in width, is denuded of epider¬ mis. This dermal strip will be placed over the top of the septum, and because it has been deepithelialized, it can be buried under the alar nasal tissues. When the final measured graft is taken, it consists of two large skin segments joined by this dermal band (Fig 1). Excellent exposure of the nasal septum is obtained using a modification of the external rhinoplasty approach. A transcolumellar incision is made at the junction of the upper lip and then carried through into a transfixion exposure of the caudal septum. Bilateral alar base-releasing incisions complete the mobilization of the distal aspect of the nose (Fig 1). The upper lateral cartilages are divided from the septum, and the nose is degloved, providing direct exposure to all of the nasal septum below the nasal bones. The diseased septal mucosa is de¬ nuded with a scalpel, but the underlying perichondrium is pre¬ served. The split skin is draped over both sides of the nasal sep¬ tum, covering the septal dorsum with the narrow dermal strip. Superiorly, at the level of the nasal bones, the dermal strip is di¬ vided from the rest of the graft, which is placed beneath the nasal bones against the bony septum. The dermal strip passes via a subcutaneous tunnel above the nasal bony dorsum, to be fixed by a stitch in the area of the nasion (Fig 2). Anchoring stitches in the caudal septum ensures that the graft is evenly tensed anteriorly and superiorly (Fig 3). Careful intranasal packing facilitates the application of the graft to all of the involved septum. The mobi¬ lized alar cartilages and nasal skin are resutured. The dermal strip is buried under these tissues. There are select patients in whom, in addition to the septum, the disease diffusely involves the lateral vestibular and turbinate areas, making it imperative to treat these with split skin also. An appropriate, but similar, skin graft with a dermal strip is reversed and applied to both lateral nasal walls with the dermal band ly¬ ing on top of, parallel to, and in contact with the dermal part of the first graft (Fig 4).
COMMENT Over a period of 3 years, this operation has been performed on 11 patients, and in nine cases the graft take was complete. The improvement in quality of life has been
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/14/2015
Fig 1.—Drawings of split-skin graft with dermal strip and modified ex¬
Fig 2.—Intraoperative drawing
Fig 3.—The split-skin graft is in position and anchored with sutures. The
Fig 4.—An additional but similar graft is used to cover both lateral nasal
ternal rhinoplasty approach to the septum showing the transcolumellar and alar base incisions.
dermal strip extends subcutaneously
to
the nasion.
dramatic. In two patients the take was around 60%. Six patients have had their epistaxes completely arrested and now enjoy hemoglobin levels above 120 g/L, compared with preoperative levels of 50 to 100 g/L. Among the pa¬ tients were a grandmother and her two adult daughters, a father and son, and a 15-year-old boy. The dermal band is the only part of the skin graft that is buried under the dorsal skin. Once covered with viable tissues, however, the dermal elements will not proliferate. Clodius2 described dermal nasolabial flaps in the treatment of facial palsy. The external transcolumellar approach was first de¬ scribed by Rethi,3 and in recent years Anderson and Ries4 have shown that it can be used routinely in corrective rhi¬ noplasty. Kuriloff5 recommends the operation for expo¬ sure of the septum during the repair of septal perforations. I have also found the exposure to be excellent for extensive septal and other intranasal procedures. The dermoplasty operation is considered inadequate when both nasal passages are densely populated with vascular malformations. Bridger and Baldwin6 described a case of a 69-year-old man who had been hospitalized on
of exposed septum. The mucosa is removed, leaving perichondrium. The skin graft is draped over the sep¬ tum. Superiorly, the dermal strip is released, to pass subcutaneously above the nasal bony dorsum.
walls.
more
than 40 occasions for transfusions because of
nose¬
bleeds, chronic anemia, and secondary cardiac failure. This
and a similar patient have been satisfactorily treated by an extended total rhinotomy with excision of all intranasal mucosa and turbinâtes and resurfacing with a free radial forearm skin flap. In less advanced cases, however, the dermoplasty technique described herein is preferred. The author thanks Michael Baldwin, FRACS, for his assistance and Marcus Cremonese for preparing the photographs. References
Septal dermoplasty for control of nosebleeds caused by hereditary hemorrhagic telangiectasia. Trans Am Acad Ophthalmol Otolaryngol. 1960;64:500-506. 1. Saunders WH.
2. Clodius L. Reconstruction of the nasolabial fold. Plast Reconstr
1972;50:467-470.
Surg.
3. Rethi A. Operative treatment of ozoena.J LaryngolOtol. 1948;62:139-143. 4. Anderson JR, Ries WR. Incisions and skeletal exposure. In: Smith JD, ed. Rhinoplasty: Emphasizing the External Approach. New York, NY: Thieme\x=req-\ Stratton Inc; 1986:53-60. 5. Kuriloff DB. Nasal septal perforations and nasal obstruction. Otolaryngol Clin North Am. 1989;22:333-349. 6. Bridger GP, Baldwin M. Microvascular free flap in hereditary hemor-
rhagic telangiectasia. Arch Otolaryngol Head Neck Surg. 1989;116:85-87.
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/14/2015
Dermoplasty Technique
G. Patrick
Bridger, FRCS, FRACS,
dermoplasty technique is described that allows both sides of the nasal septum to be covered with a single split-skin graft. This graft is designed with a narrow central dermal band that covers the septal dorsum and can be buried under the alar tissues. Exposure is obtained via a modified external rhinoplasty approach. The operation has been successfully performed on 11 patients, and in nine of them the graft take was complete. (Arch Otolaryngol Head Neck Surg. 1992;118:992-993) \s=b\ A new
is
incidence of hereditary hemorrhagic telangiectasia Thedifficult shows establish; however, surgeon collects the to
the
who
condition soon many patients. afflicted People by this disease are frequently treated for life-threatening nosebleeds. Nevertheless, it is a topic that is rarely featured at academic meetings and seldom appears in the literature. The epistaxes start without warning, can be profuse and bilateral, and occur several times each day. The destruction to quality of life is considerable. Hereditary hemorrhagic telangiectasia, or Rendu-OslerWeber disease, is a simple autosomal dominant trait that is manifested clinically by multiple telangiectases. The es¬ sential lesion is an abnormal subepidermal thin-walled ar¬ teriovenous fistula composed of a single layer of endothelial cells. Submucosal telangiectases are seen throughout the nasal mucosa, especially on the anterior septum, and it is these vessels that are easily traumatized and cause most nosebleeds. The most successful definitive operative procedure is the dermoplasty, which was first described by Saunders1 in 1960. In this operation, the diseased mucosa covering the anterior half of the septum is removed with a curette, leaving the vascular bed of perichondrium, onto which a split-skin graft is placed. Both sides of the septum are op¬ erated on at the same time, and usually all work is done through a speculum. The graft covering the septum and floor is only anchored anteriorly and is held in place with careful packing. When the telangiectases are widespread, a separate sleeve of skin graft is applied to the lateral na¬ sal surface. Many authors have testified to the success of this procedure. Most patients, however, will continue to bleed but not so frequently or severely. I have operated on seven patients using the classic Saunders' dermoplasty technique. The approach has proved to be difficult and the graft take unpredictable. The access provided by either a nasal speculum or a small alar-releasing incision is inadequate. General anesthesia supplemented by topical and intramucosal adrenalin selan
dorn controls the bleeding, which constantly obscures the operative field. The intranasal placement of the skin graft is imprecise and tenuous. Among the seven patients, there were two cases in which the graft failed to take and only a few cases in which the whole graft survived. As this is a familial condition, there are often several relatives who require treatment. An inadequate operation that does not rectify the bleeding problem will result in other family members deciding against surgery. Similarly, a successful procedure encourages other sufferers to seek a surgical solution. A new dermoplasty technique is described that facilitates graft placement and survival.
interest in
Accepted From the
for publication June 2, 1992. Department of Otolaryngology, The Prince of Wales
pital, Sydney,
Hos-
Australia. to Suite 1, 21 Kitchener Parade, Bankstown NSW 2200, Australia (Dr Bridger).
Reprint requests
DLO
SURGICAL
TECHNIQUE
With this technique, a single piece of split skin is used to drape both sides of the nasal septum, replacing all of the involved mucosa. The area to be grafted is measured and then liberally mapped out on the donor site, which is usually the thigh. Before the graft is taken, a central band, approximately 1 cm in width, is denuded of epider¬ mis. This dermal strip will be placed over the top of the septum, and because it has been deepithelialized, it can be buried under the alar nasal tissues. When the final measured graft is taken, it consists of two large skin segments joined by this dermal band (Fig 1). Excellent exposure of the nasal septum is obtained using a modification of the external rhinoplasty approach. A transcolumellar incision is made at the junction of the upper lip and then carried through into a transfixion exposure of the caudal septum. Bilateral alar base-releasing incisions complete the mobilization of the distal aspect of the nose (Fig 1). The upper lateral cartilages are divided from the septum, and the nose is degloved, providing direct exposure to all of the nasal septum below the nasal bones. The diseased septal mucosa is de¬ nuded with a scalpel, but the underlying perichondrium is pre¬ served. The split skin is draped over both sides of the nasal sep¬ tum, covering the septal dorsum with the narrow dermal strip. Superiorly, at the level of the nasal bones, the dermal strip is di¬ vided from the rest of the graft, which is placed beneath the nasal bones against the bony septum. The dermal strip passes via a subcutaneous tunnel above the nasal bony dorsum, to be fixed by a stitch in the area of the nasion (Fig 2). Anchoring stitches in the caudal septum ensures that the graft is evenly tensed anteriorly and superiorly (Fig 3). Careful intranasal packing facilitates the application of the graft to all of the involved septum. The mobi¬ lized alar cartilages and nasal skin are resutured. The dermal strip is buried under these tissues. There are select patients in whom, in addition to the septum, the disease diffusely involves the lateral vestibular and turbinate areas, making it imperative to treat these with split skin also. An appropriate, but similar, skin graft with a dermal strip is reversed and applied to both lateral nasal walls with the dermal band ly¬ ing on top of, parallel to, and in contact with the dermal part of the first graft (Fig 4).
COMMENT Over a period of 3 years, this operation has been performed on 11 patients, and in nine cases the graft take was complete. The improvement in quality of life has been
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/14/2015
Fig 1.—Drawings of split-skin graft with dermal strip and modified ex¬
Fig 2.—Intraoperative drawing
Fig 3.—The split-skin graft is in position and anchored with sutures. The
Fig 4.—An additional but similar graft is used to cover both lateral nasal
ternal rhinoplasty approach to the septum showing the transcolumellar and alar base incisions.
dermal strip extends subcutaneously
to
the nasion.
dramatic. In two patients the take was around 60%. Six patients have had their epistaxes completely arrested and now enjoy hemoglobin levels above 120 g/L, compared with preoperative levels of 50 to 100 g/L. Among the pa¬ tients were a grandmother and her two adult daughters, a father and son, and a 15-year-old boy. The dermal band is the only part of the skin graft that is buried under the dorsal skin. Once covered with viable tissues, however, the dermal elements will not proliferate. Clodius2 described dermal nasolabial flaps in the treatment of facial palsy. The external transcolumellar approach was first de¬ scribed by Rethi,3 and in recent years Anderson and Ries4 have shown that it can be used routinely in corrective rhi¬ noplasty. Kuriloff5 recommends the operation for expo¬ sure of the septum during the repair of septal perforations. I have also found the exposure to be excellent for extensive septal and other intranasal procedures. The dermoplasty operation is considered inadequate when both nasal passages are densely populated with vascular malformations. Bridger and Baldwin6 described a case of a 69-year-old man who had been hospitalized on
of exposed septum. The mucosa is removed, leaving perichondrium. The skin graft is draped over the sep¬ tum. Superiorly, the dermal strip is released, to pass subcutaneously above the nasal bony dorsum.
walls.
more
than 40 occasions for transfusions because of
nose¬
bleeds, chronic anemia, and secondary cardiac failure. This
and a similar patient have been satisfactorily treated by an extended total rhinotomy with excision of all intranasal mucosa and turbinâtes and resurfacing with a free radial forearm skin flap. In less advanced cases, however, the dermoplasty technique described herein is preferred. The author thanks Michael Baldwin, FRACS, for his assistance and Marcus Cremonese for preparing the photographs. References
Septal dermoplasty for control of nosebleeds caused by hereditary hemorrhagic telangiectasia. Trans Am Acad Ophthalmol Otolaryngol. 1960;64:500-506. 1. Saunders WH.
2. Clodius L. Reconstruction of the nasolabial fold. Plast Reconstr
1972;50:467-470.
Surg.
3. Rethi A. Operative treatment of ozoena.J LaryngolOtol. 1948;62:139-143. 4. Anderson JR, Ries WR. Incisions and skeletal exposure. In: Smith JD, ed. Rhinoplasty: Emphasizing the External Approach. New York, NY: Thieme\x=req-\ Stratton Inc; 1986:53-60. 5. Kuriloff DB. Nasal septal perforations and nasal obstruction. Otolaryngol Clin North Am. 1989;22:333-349. 6. Bridger GP, Baldwin M. Microvascular free flap in hereditary hemor-
rhagic telangiectasia. Arch Otolaryngol Head Neck Surg. 1989;116:85-87.
Downloaded From: http://archotol.jamanetwork.com/ by a UQ Library User on 06/14/2015
