jiafm, 2010-32(2) april-june. - forensic medicine

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ISSN 0971-0973 J Indian Acad Forensic Med, 32(2)Case ReportA Rare Case Report: Poland‟s Syndrome with Unilateral Amastia inan alleged victim of Rape**Dr. Kalpasree Bhowmik, **Dr. Nibedita Shyam, *Dr. K.C. Das (MD)Abstract:We report a rare case on Poland‟s syndrome with unilateral Amastia in an alleged victim of rape who wasbrought to our department escorted by the police for Medical examination. Congenital absence of breast is anunusual anomaly of the breast. As such, anomalies of breast are rare. Amastia is the total absence of breast tissue.Poland‟s syndrome is one such rare syndrome associated with Amastia. Poland‟s syndrome is not a single entity butis a constellation of anomalies.In this syndrome, the predominant defect varies depending upon the extent of involvement of differentparts. All children with Poland‟s syndrome have apalsia or hypoplasia of the sternocostal portion of the pectoralismajor muscle and at least one other associated lesion. Other than Amastia, abnormalities of hand, nipples and othermuscles may be associated with Poland‟s syndrome. Such congenital anomalies may be associated with depressionand this in turn may lead to these patients falling easy prey of criminal minds.Key Words: Poland‟s syndrome, Amastia, Anomaly, BreastIntroduction:Amastia is a rare congenital developmentalanomaly of the breast. In Amastia, there is absence ofbreast tissue, areola and nipple.[1] Amastia isdescribed in The Holy Bible as:“We have a little sister and she hath nobreast.” (Song of Solomon VIII: 8). Amastia isfrequently not the only problem but it is associatedwith other anomalies. Poland‟s Syndrome is one suchrare condition associated with Amastia.Brief case history: (This case was registeredunder section 376/493 IPC in one of thePolice Station of the Kamrup district of Assam)‣ An alleged victim of Rape of age about thirteenyears, escorted by police, hailing from KamrupDistrict of Assam, presented to the Dept. ofForensic Medicine, Gauhati Medical College,Assam, for medical examination on 29thOctober, 2009.‣ As per the victim girl, she was in love with a boywho forced her to have sex with him in lieu offalse assurance of lawful marriage and thenconsequently she became pregnant.Corresponding Author:** PG Teacher, Dr. Kalpasree BhowmikDept. of Forensic and State Medicine,Gauhati Medical College, NARKASUR Hill Top(College building, 5 th floor), Bhangagarh, Guwahati,Assam. PIN-781032,E mail: kalpasree@yahoo.com** PG Teacher,*Associate Prof.No family history of absence of breast was found.‣ Birth history: She was born by normal vaginaldelivery at home.‣ Menstrual history: She has attained menarche atthe age of about twelve years.‣ No significant history of medical or surgicaltreatment.‣ Socio-economic status-poorOn examination:• Thin built• Height-155cm, weight-39kg• Chest girth-79cm• Abdomen- Girth-77cm. Fundal height at 32weeks. Fetal parts felt.• Teeth: Total number-twenty six. Twentyfour permanent; two temporary molars(second temporary molar in both lowerquadrants). Space for third molar notdeveloped.• Genitalia- healthy and well developed• Pubic and axillary hair- present and scantyin distribution.• Systemic examination: no other abnormalitydetected clinicallyOn local examination of the chest wall oninspection:‣ Absence of breast with absence of nipple andareola found on the left side of the chest wall‣ The sternocostal part of the Pectoralis majormuscle on the left side was also found to bedeficit and deformity seen on the part of thechest wall.‣ A furuncle present on the mid-sternum area176
J Indian Acad Forensic Med, 32(2) ISSN 0971-0973‣ The other breast on the right side was presentand borne features of changes during pregnancy.‣ Both the upper limbs and lower limbs are normalDiscussion:Amastia:Definition: [1]Amastia is the total absence of breast tissue. Atheliais the absence of nipple. In Hypomastia there isunderdevelopment of breast tissue and the nipple isusually present.Developmental cause of Amastia:Lack of formation or obliteration of milkline.Amastia can also be due to: [1] [2] Teratogen exposure [1] [2] Injuries sustained during – [1]• Thoracotomy• Chest tube placement• Inappropriate biopsy ofthe breast bud• Radiotherapy• Severe burnsAmastia may be associated with CongenitalEctodermal Dysplasia and Poland‟s Syndrome.[1] [2]Poland‟s Syndrome: [1] [3]The initial description of Poland‟s Syndrome firstappeared in the English literature in 1841 written byAlfred Poland (1822-1872), who was a medicalstudent at the Guy‟s Hospital, London, althoughGerman and French cases had been described earlier.Definition: By definition, all children with Poland‟sSyndrome have aplasia or hypoplasia of thesternocostal portion of the Pectoralis major muscleand at least one other associated lesion. [1]Incidence: [1] [3]It has a sporadic occurrence estimated at1:30,000 to 32,000 live births. [1] [3]It is rarely familial [1] [3]More commonly involves right side. [3]One out of six patients with Amastia orHypomastia have Poland‟s syndrome [3]Developmental causes of Poland‟s syndromesuggested are: [1] [3]Abnormal migration of the embryonictissues forming the pectoral musclesHypoplasia of the subclavian arteryIn utero injuries from attempted abortionNone of these theories have been uniformlyaccepted.Clinical features: [1] [3] Poland‟s syndrome is a constellation ofanomalies. [3] Children demonstrate remarkable diversity.[1] The predominant defect varies, dependingon the extent of involvement of differentparts.[1] The degree of abnormality of the hand,breast or chest wall varies. [3] Pectoralis major may be partially absent ortotally missing. Generally, the sternocostalcomponent of the Pectoralis major is themissing portion. [1] [3] [4] Pectoralis minor, Serratus anterior,Lattisimus Dorsi, Rectus Abdominis may bedeficit or absent. [3] Abnormalities of breast may includeAmastia, Hypomastia or Athelia. [3] Nipple, if present, may be lightly pigmentedand higher on the chest than the normalcontralateral nipple.[1] Syndactyly and brachydactyly may be seenin the hands. [1] [3] Aplasia of two to three ribs (second to fifthribs mostly) may be present. [1] Chest wall deformity so severe that it mayrequire surgery. [1] Scanty subcutaneous fat. [1] [3] Absent axillary hair. [1] [3]Investigations: [1]CT scan has been helpful in assessing:a) The chest wall configurationb) Extent of involvementTreatment: [2] [3]Surgical repair is rarely required except in certaincases. [2]Because of rarity of this condition, optimal treatmenthas not been adequately investigated. Some of thereasonable options are:a) Use of Transversus- Rectus AbdominisMuscle (TRAM) flap for reconstruction ofbreasts. [2]b) Tissue expansion and implant placement. [2]c) Reconstruction of aplastic ribs withautologous rib grafts. [3]Conclusion:Congenital anomalies are important data inIdentification but these have got other dimensionstoo. In a country like India, birth of a female child ishighly unwelcome amongst many communities. In agirl child with anomaly (especially of the breast),underlying depression resulting from social stigmadue to such deformity and the grim prospect ofmarriage, may be the cause behind such victimsbecoming easy prey of criminals who take advantageof their vulnerable minds by giving them falseassurance of marriage.177
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jiafm, 2010-32(2) april-june. - forensic medicine

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