==== Front Surg Neurol IntSurg Neurol IntSNISurgical Neurology International2229-50972152-7806Medknow Publications & Media Pvt Ltd India SNI-9-14310.4103/sni.sni_34_18Unique Case Observations: Case ReportDorsal accessory ectopic breast with polythelia – A marker of occult spinal dysraphism Gandhoke Charandeep S. charandeepsingh2008@gmail.com*Syal Simran K. simransyal100@gmail.com2Singh Hukum drhsingh2008@hotmail.comSingh Daljit drdaljit@hotmail.comSaran Ravindra K. ravindraksaran@hotmail.com1Department of Neurosurgery, Maulana Azad Medical College, Lok Nayak Jai Prakash Narayan Hospital, Guru Nanak Eye Centre and G. B. Pant Institute of Postgraduate Medical Education and Research (G.I.P.M.E.R.), New Delhi, India1 Department of Pathology, Maulana Azad Medical College, Lok Nayak Jai Prakash Narayan Hospital, Guru Nanak Eye Centre and G. B. Pant Institute of Postgraduate Medical Education and Research (G.I.P.M.E.R.), New Delhi, India2 Department of Paediatrics, Sri Guru Ram Das Institute of Medical Sciences and Research, Amritsar, Punjab, India* Corresponding author 2018 24 7 2018 9 14311 2 2018 05 6 2018 Copyright: © 2018 Surgical Neurology International2018This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.Background: Accessory breast, also known as supernumerary breasts, polymastia, or mammae erraticae, is a clinical condition of having an additional breast. Accessory breasts are usually seen along the embryonic milk line, with the majority located in the axilla. Polythelia is the presence of an additional nipple. We report a rare case of dorsal accessory ectopic breast with three nipples (two well formed and one rudimentary) occurring along with lipomeningomyelocele and diastematomyelia. Case Description: We report the case of an 18-year-old female who presented with chief complaints of swelling over the upper back since birth and spastic weakness of bilateral lower limbs with inability to walk since 2 years. Three-dimensional computed tomography scan of the dorsal spine was suggestive of a wide bony defect in the posterior spinal elements from D3 to D9 vertebrae. Diastematomyelia was also seen. Magnetic resonance imaging of the dorsal spine was suggestive of a complex spinal dysraphism with lipomeningomyelocele and diastematomyelia. During surgery, the patient's accessory breast was removed, lipomatous tissue and bony septum were excised, and dural repair was done. Histopathological examination was consistent with accessory ectopic breast with lipomeningomyelocele. Conclusion: Dorsal accessory breast, although a rare entity, whenever present should alert the clinician regarding the possibility of an underlying occult spinal dysraphism (OSD). Therefore, dorsal accessory breast can also be considered as a marker of OSD. Dorsalectopic breastlipomeningomyeloceleoccult spinal dysraphismpolythelia ==== Body INTRODUCTION Accessory breast, also known as supernumerary breasts, polymastia, or mammae erraticae, is a clinical condition of having an additional breast. Accessory breasts are usually seen along the embryonic milk line, with the majority located in the axilla. Polythelia is the presence of an additional nipple. We report a rare case of dorsal accessory ectopic breast with three nipples (two well formed and one rudimentary) occurring along with lipomeningomyelocele and diastematomyelia. The association of dorsal accessory breast with meningomyelocele and diastematomyelia has been reported only once in the world literature, however, the association of dorsal accessory breast with polythelia with lipomeningomyelocele and diastematomyelia has never been reported.[4] CASE DESCRIPTION We report the case of an 18-year-old female who presented with chief complaints of swelling over the upper back since birth and spastic weakness of bilateral lower limbs with an inability to walk since 2 years. On examination, the swelling was soft-to-firm, nontender, midline in location, which started increasing in size at puberty and progressed since then to the present size of 14 cm × 12 cm [Figure 1]. Three-dimensional (3D) computed tomography scan of the dorsal spine was suggestive of a wide bony defect in the posterior spinal elements from D3 to D9 vertebrae. Diastematomyelia was also seen from D3 to D7 vertebrae [Figure 2]. Magnetic resonance imaging (MRI) of the dorsal spine was suggestive of a complex spinal dysraphism with lipomeningomyelocele and diastematomyelia [Figure 3]. At surgery, the patient's accessory breast was removed, lipomatous tissue and bony septum were excised, and dural repair was done. An elliptical vertical skin incision was taken incorporating the reductant skin along with the three nipples. The entry of the fibrolipomatous stalk through the fascial defect was identified. A large lipomatous mass along with the accessory breast was amputated at the level of the stalk to help in the visualization of the spinal cord–lipoma interface. Two hemicords with a dorsal type lipomeningomyelocele was identified [Figure 4]. The penetrating intradural lipomatous tract was identified all around. The osseocartilaginous median septum was drilled. Normal dura of both the hemicords was opened and the openings were carried caudally towards the penetrating tract. Circumferential dissection around the lipomatous tract was continued until the dura was separated completely. The dural opening was then continued caudally. The fatty mass was resected with the help of cavitron ultrasonic surgical aspirator (CUSA). Neurulation of the neural placode was then done. Water tight dural closure (duraplasty) was done for both the hemicords separately with the help of a graft. Histopathological examination was consistent with accessory ectopic breast with lipomeningomyelocele [Figure 5]. Figure 1 (a) Clinical examination of the patient showing a midline dorsal accessory breast with 3 nipples (2 well formed and 1 rudimentary). (b) Close up view Figure 2 (a and b) 3D Computed Tomography (CT) scan of the dorsal spine showing a wide bony defect in the posterior spinal elements from D3 to D9 vertebrae. (c) Diastematomyelia is also seen Figure 3 (a) Magnetic Resonance Imaging (MRI) of the dorsal spine suggestive of a complex spinal dysraphism with lipomeningomyelocele. (b) Diastematomyelia is also seen Figure 4 Schematic diagram showing the relationship of occult spinal dysraphism (OSD) with dorsal accessory ectopic breast Figure 5 (a) Hematoxylin and eosin stained section (×40) showing normal skin with appendages (single arrow); Subepithelial tissue shows lactiferous duct (double arrow). (b) Hematoxylin and eosin stained section (×80) showing lactiferous duct with secretion inside (single arrow) along with terminal duct lobular units of breast (double arrow). (c and d) Hematoxylin and eosin stained section (×20 and ×40 respectively) showing lipomatous component composed of sheets of mature adipocytes with adjacent dural fibroblasts The postoperative period was uneventful. Spasticity and power in the lower limbs improved and the patient was able to walk with support at the time of discharge. DISCUSSION Multiple theories have been proposed to explain the occurrence of accessory breast. These include Darwin's theory which stated that “traits which have disappeared generations before, can reappear,” Pfeifer's theory of metaplasia of sweat glands or modified sweat glands, Hughes's theory of random migration of primordial breast cells away from the mammary crest, and Schultz's theory of displacement of milk lines, laterally or caudally.[2567] Studies have shown that an ectopic breast usually increases in size after hormonal stimulation during puberty, pregnancy, or lactation.[1] In our case also, the swelling started increasing in size after the girl attained puberty. Ectopic breasts, similar to normal breasts, are prone to the risk of developing various types of breast diseases such as abscess, mastitis, fibroadenoma, fibrocystic disease, or breast cancer.[8] Occult spinal dysraphism (OSD) is characterized by skin-covered lesions without exposed neural tissue. Cutaneous markers of OSD include lipoma, fibroma pendulum, human or faun tail, dermal sinus, atypical dimple (larger than 5 mm and more than 2.5 cm from the anus), hamartoma, aplasia cutis congenita, deviation of the gluteal furrow, port wine stain, hemangioma, hypertrichosis, and acrochordon (skin tag).[3] A combination of two or more congenital cutaneous lesions constitutes the strongest marker of OSD.[3] CONCLUSION Dorsal accessory breast, although a rare entity, whenever present should alert the clinician regarding the possibility of an underlying OSD. Therefore, dorsal accessory breast can also be considered as a marker of OSD. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. http://surgicalneurologyint.com/Dorsal-accessory-ectopic-breast-with-polythelia-–-A-marker-of-occult-spinal-dysraphism/ ==== Refs REFERENCES 1 Burdick AE Thomas KA Welsh E Powell J Elgart GW Axillary polymastia J Am Acad Dermatol 2003 49 1154 6 14639406 2 Darwin C The descent of man and selection in relation to sex 1892 New York Appleton and Co 36 7 3 Guggisberg D Hadj-Rabia S Viney C Bodemer C Brunelle F Zerah M Skin markers of occult spinal dysraphism in children: A review of 54 cases Arch Dermatol 2004 140 1109 15 15381552 4 Gupta VK Kapoor I Punia RS Attri AK Dorsal ectopic breast in a case of spinal dysraphism: A rare entity Neurol India 2015 63 392 4 26053812 5 Hughes ES The development of the mammary gland: Arris and Gale Lecture, delivered at the Royal College of Surgeons of England on 25 th October, 1949 Ann R Coll Surg Engl 1950 6 99 119 19309885 6 Pfeifer JD Barr RJ Wick MR Ectopic breast tissue and breast-like sweat gland metaplasias: An overlapping spectrum of lesions J Cutan Pathol 1999 26 190 6 10335896 7 Schultz A Pathologische anatomic der brustdruse In: Handbuch der speziellen Pathologischen Anatomie and Histologie 1933 Berlin Julius Springer 8 Shin SJ Sheikh FS Allenby PA Rosen PP Invasive secretory (juvenile) carcinoma arising in ectopic breast tissue of the axilla Arch Pathol Lab Med 2001 125 1372 4 11570920