Cowden syndrome, a rare genetic disorder estimated to occur in 1 in 200,000 live births and inherited as an autosomal dominant mutation in PTEN gene, is part of the PTEN hamartoma tumor syndrome. These patients are at risk of breast cancer, as well as cancers of the digestive tract, thyroid, uterus and ovaries. Often identified by their dentist due to characteristic papillomatosis in the gingival mucosa, they have an estimated lifetime risk of up to 81% of developing breast cancer. This article describes a relatively uncommon procedure of bi-pedicle nipple-sparing mastectomy, a modified Letterman technique, used in the setting of immediate implant based reconstruction in a patient with Cowden syndrome.
Pectoralis major muscle was lifted to create a pocket for the implant using diathermy throughout to ensure hemostasis. TIGR Matrix mesh (Novus Scientific) was used to recreate the IMF and secure with interrupted 3-0 PDS suture. This synthetic absorbable mesh is easier to handle, produces less seroma with need for only one drain per side, compared to standard ADM. Temporary sizer was used to estimate implant size and confirm closure of skin wounds without tension. Pectoral muscle was sutured to TIGR mesh with interrupted 3-0 PDS once the implant was in a satisfactory position. A single no.15 low-suction Blake drain was placed under the skin flap.
The patient was discharged home 2 days after surgery. On week 3 the patient developed a minor wound infection at the edge of the left nipple. By week 5 patient returned to work.
Successful outcome in this case was possible due to coordinated teamwork in a multidisciplinary setting between the different specialists. Careful patient selection, detailed information with multiple discussions and close monitoring in the post-operative period.
Potential pitfalls of nipple loss or necrosis can be circumvented by careful patient selection, avoiding in smokers, diabetics, or older patients, careful handling of skin flaps and the pedicle to avoid traction injury and ensuring closure of all wounds without tension.