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Breasts and/or nipples, aplasia or hypoplasia of, 2(BNAH2)

MedGen UID:
863355
Concept ID:
C4014918
Congenital Abnormality
Synonym: BNAH2
 
Gene (location): PTPRF (1p34.2)
 
Monarch Initiative: MONDO:0014450
OMIM®: 616001

Definition

Congenital aplastic deformities of the breast include amastia (total absence of breasts and nipple), athelia (absence of the nipple), and amazia (absence of the mammary gland). Most common is amastia. Bilateral absence of the breasts may occur as an isolated anomaly or may be associated with a syndrome or a cluster of other anomalies, including anhidrotic ectodermal dysplasia (305100) or Poland syndrome (173800) (summary by Papadimitriou et al., 2009). For a discussion of genetic heterogeneity of aplasia or hypoplasia of the breasts and/or nipples, see 113700. [from OMIM]

Clinical features

From HPO
Cryptorchidism
MedGen UID:
8192
Concept ID:
C0010417
Congenital Abnormality
Cryptorchidism, or failure of testicular descent, is a common human congenital abnormality with a multifactorial etiology that likely reflects the involvement of endocrine, environmental, and hereditary factors. Cryptorchidism can result in infertility and increases risk for testicular tumors. Testicular descent from abdomen to scrotum occurs in 2 distinct phases: the transabdominal phase and the inguinoscrotal phase (summary by Gorlov et al., 2002).
Male urethral meatus stenosis
MedGen UID:
1694020
Concept ID:
C4760707
Disease or Syndrome
An abnormal narrowing of the urethral opening (meatus) of the penis.
Bilateral single transverse palmar creases
MedGen UID:
354661
Concept ID:
C1862095
Finding
The distal and proximal transverse palmar creases are merged into a single transverse palmar crease on both hands.
Small earlobe
MedGen UID:
334587
Concept ID:
C1842680
Finding
Reduced volume of the earlobe.
Broad nasal tip
MedGen UID:
98424
Concept ID:
C0426429
Finding
Increase in width of the nasal tip.
Smooth philtrum
MedGen UID:
222980
Concept ID:
C1142533
Finding
Flat skin surface, with no ridge formation in the central region of the upper lip between the nasal base and upper vermilion border.
Anteverted nares
MedGen UID:
326648
Concept ID:
C1840077
Finding
Anteriorly-facing nostrils viewed with the head in the Frankfurt horizontal and the eyes of the observer level with the eyes of the subject. This gives the appearance of an upturned nose (upturned nasal tip).
Widely spaced teeth
MedGen UID:
337093
Concept ID:
C1844813
Finding
Increased spaces (diastemata) between most of the teeth in the same dental arch.
Highly arched eyebrow
MedGen UID:
358357
Concept ID:
C1868571
Finding
Increased height of the central portion of the eyebrow, forming a crescent, semicircular, or inverted U shape.
Absent nipple
MedGen UID:
64223
Concept ID:
C0175755
Congenital Abnormality
Congenital failure to develop, and absence of, the nipple.
Hypoplastic nipples
MedGen UID:
98156
Concept ID:
C0432355
Congenital Abnormality
Underdevelopment of the nipple.
Hypoplastic areola
MedGen UID:
477663
Concept ID:
C3276032
Finding
Underdevelopment of the areola, the circular area of pigmented skin surrounding the nipple.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  

Professional guidelines

PubMed

Obstet Gynecol 2023 Jun 1;141(6):1262-1288. doi: 10.1097/AOG.0000000000005202. PMID: 37486661
Northrup H, Aronow ME, Bebin EM, Bissler J, Darling TN, de Vries PJ, Frost MD, Fuchs Z, Gosnell ES, Gupta N, Jansen AC, Jóźwiak S, Kingswood JC, Knilans TK, McCormack FX, Pounders A, Roberds SL, Rodriguez-Buritica DF, Roth J, Sampson JR, Sparagana S, Thiele EA, Weiner HL, Wheless JW, Towbin AJ, Krueger DA; International Tuberous Sclerosis Complex Consensus Group
Pediatr Neurol 2021 Oct;123:50-66. Epub 2021 Jul 24 doi: 10.1016/j.pediatrneurol.2021.07.011. PMID: 34399110
Messner AH, Walsh J, Rosenfeld RM, Schwartz SR, Ishman SL, Baldassari C, Brietzke SE, Darrow DH, Goldstein N, Levi J, Meyer AK, Parikh S, Simons JP, Wohl DL, Lambie E, Satterfield L
Otolaryngol Head Neck Surg 2020 May;162(5):597-611. Epub 2020 Apr 14 doi: 10.1177/0194599820915457. PMID: 32283998

Recent clinical studies

Etiology

Kim E, Lewin AA
Radiol Clin North Am 2024 Jul;62(4):593-605. Epub 2024 Mar 7 doi: 10.1016/j.rcl.2023.12.007. PMID: 38777536
Hussein H, Abbas E, Keshavarzi S, Fazelzad R, Bukhanov K, Kulkarni S, Au F, Ghai S, Alabousi A, Freitas V
Radiology 2023 Mar;306(3):e221785. Epub 2023 Jan 31 doi: 10.1148/radiol.221785. PMID: 36719288
Douglas P
Womens Health (Lond) 2022 Jan-Dec;18:17455057221087865. doi: 10.1177/17455057221087865. PMID: 35343816Free PMC Article
Hammond DC, Loffredo M
Plast Reconstr Surg 2012 May;129(5):829e-839e. doi: 10.1097/PRS.0b013e31824a2efc. PMID: 22544113
Dafydd H, Roehl KR, Phillips LG, Dancey A, Peart F, Shokrollahi K
J Plast Reconstr Aesthet Surg 2011 Feb;64(2):160-3. Epub 2010 Jun 8 doi: 10.1016/j.bjps.2010.04.043. PMID: 20965141

Diagnosis

Leung AKC, Lam JM, Hon KL
Curr Pediatr Rev 2024;20(4):500-509. doi: 10.2174/1573396320666230726110658. PMID: 37496240
Coffey K, Jochelson MS
Eur J Radiol 2022 Nov;156:110513. Epub 2022 Sep 10 doi: 10.1016/j.ejrad.2022.110513. PMID: 36108478Free PMC Article
Douglas P
Womens Health (Lond) 2022 Jan-Dec;18:17455057221087865. doi: 10.1177/17455057221087865. PMID: 35343816Free PMC Article
Faguy K
Radiol Technol 2022 Jan;93(3):303M-315M. PMID: 35017277
McGhee DE, Steele JR
Physiology (Bethesda) 2020 Mar 1;35(2):144-156. doi: 10.1152/physiol.00024.2019. PMID: 32027563

Therapy

Hussein H, Abbas E, Keshavarzi S, Fazelzad R, Bukhanov K, Kulkarni S, Au F, Ghai S, Alabousi A, Freitas V
Radiology 2023 Mar;306(3):e221785. Epub 2023 Jan 31 doi: 10.1148/radiol.221785. PMID: 36719288
Sisti A
Medicina (Kaunas) 2020 Jun 16;56(6) doi: 10.3390/medicina56060296. PMID: 32560062Free PMC Article
Fiorica JV
Clin Obstet Gynecol 2016 Dec;59(4):688-709. doi: 10.1097/GRF.0000000000000246. PMID: 27741212
Hammond DC, Loffredo M
Plast Reconstr Surg 2012 May;129(5):829e-839e. doi: 10.1097/PRS.0b013e31824a2efc. PMID: 22544113
Whelan TJ, Pignol JP, Levine MN, Julian JA, MacKenzie R, Parpia S, Shelley W, Grimard L, Bowen J, Lukka H, Perera F, Fyles A, Schneider K, Gulavita S, Freeman C
N Engl J Med 2010 Feb 11;362(6):513-20. doi: 10.1056/NEJMoa0906260. PMID: 20147717

Prognosis

Waldman RA, Finch J, Grant-Kels JM, Stevenson C, Whitaker-Worth D
J Am Acad Dermatol 2019 Jun;80(6):1467-1481. Epub 2018 Nov 16 doi: 10.1016/j.jaad.2018.08.066. PMID: 30452954
Qureshi AA, Myckatyn TM, Tenenbaum MM
Aesthet Surg J 2018 Mar 14;38(4):374-384. doi: 10.1093/asj/sjx181. PMID: 29365038
Chasan PE
Aesthetic Plast Surg 2018 Jun;42(3):662-671. Epub 2017 Nov 9 doi: 10.1007/s00266-017-1010-0. PMID: 29124375Free PMC Article
Peters MS, Lehman JS, Comfere NI
Am J Dermatopathol 2013 May;35(3):289-304; quiz 305-7. doi: 10.1097/DAD.0b013e318267caa3. PMID: 23598853
Bhatia S, Holla RG
Indian Pediatr 2005 Apr;42(4):373-5. PMID: 15876600

Clinical prediction guides

Pacifico MD, Goddard NV, Harris PA
Aesthet Surg J 2024 Sep 16;44(10):1032-1042. doi: 10.1093/asj/sjae084. PMID: 38621023
Liu D, Ba Z, Gao Y, Wang L
BMC Med Imaging 2023 Nov 10;23(1):182. doi: 10.1186/s12880-023-01144-w. PMID: 37950164Free PMC Article
Mastrosimini MG, Eccher A, Nottegar A, Montin U, Scarpa A, Pantanowitz L, Girolami I
Pathol Res Pract 2022 Dec;240:154191. Epub 2022 Oct 28 doi: 10.1016/j.prp.2022.154191. PMID: 36356336
Ferre R, Kuzmiak CM
Breast Dis 2022;41(1):205-214. doi: 10.3233/BD-210045. PMID: 35094984
Peters MS, Lehman JS, Comfere NI
Am J Dermatopathol 2013 May;35(3):289-304; quiz 305-7. doi: 10.1097/DAD.0b013e318267caa3. PMID: 23598853

Recent systematic reviews

Benitez Fuentes JD, Morgan E, de Luna Aguilar A, Mafra A, Shah R, Giusti F, Vignat J, Znaor A, Musetti C, Yip CH, Van Eycken L, Jedy-Agba E, Piñeros M, Soerjomataram I
JAMA Oncol 2024 Jan 1;10(1):71-78. doi: 10.1001/jamaoncol.2023.4837. PMID: 37943547Free PMC Article
Bodewes FTH, van Asselt AA, Dorrius MD, Greuter MJW, de Bock GH
Breast 2022 Dec;66:62-68. Epub 2022 Sep 26 doi: 10.1016/j.breast.2022.09.007. PMID: 36183671Free PMC Article
Ren W, Chen M, Qiao Y, Zhao F
Breast 2022 Aug;64:85-99. Epub 2022 Apr 19 doi: 10.1016/j.breast.2022.04.003. PMID: 35636342Free PMC Article
Lissidini G, Mulè A, Santoro A, Papa G, Nicosia L, Cassano E, Ashoor AA, Veronesi P, Pantanowitz L, Hornick JL, Rossi ED
Pathologica 2022 Apr;114(2):111-120. Epub 2022 Apr 13 doi: 10.32074/1591-951X-754. PMID: 35414723Free PMC Article
Boutas I, Kontogeorgi A, Dimitrakakis C, Kalantaridou SN
In Vivo 2022 Mar-Apr;36(2):556-562. doi: 10.21873/invivo.12737. PMID: 35241506Free PMC Article

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