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DISCUSSION

Poland’s syndrome is a congenital anomaly with varying presentations. This case is an addition to the very few existing reported cases in females, since the disorder is noticed mostly in males. Our patient probably represents a sporadic case, as no member of her family had a similar abnormality—although variable expressivity and reduced penetrance is usually present. This patient appears to have one of the mild forms of the syndrome in terms of structural defects, since there is some element of breast tissue developing; in addition, there were no obvious skeletal limb or chest deformities. The importance of detailed examination of the chest wall and cardiovascular system cannot be overemphasized, since some of these abnormalities could be asymptomatic. Occasionally, such patients with rib cage anomalies may exhibit paradoxical respiratory movement leading to inadequate ventilation, especially during general anesthesia. Chest radiograph may reveal an area of hyperlucency on the affected side that mimics a radical mastectomy. Computerized tomogram (CT) more clearly depicts the absence of the greater pectoralis muscle and allows greater appreciation of other nearby associated abnormalities. Our patient demonstrates many characteristics in consonance with known literature and previously reported cases: unilateral involvement and on the right side, which is the most frequently involved. There have been case reports of Poland’s syndrome associated with unusual defects, such as dextrocardia; genitor-urinary and spinal malformations; and malignancies, such as leukemia and non-Hodgkin’s lymphoma. One such case report described by Kabra et al. consisted of myriad abnormalities not interrelated, in which the contralateral side was affected, in addition to the involvement of both feet. They hypothesized a possible link with the human homologue of the disorganization (Ds) mutation in laboratory mice, with Poland anomaly being part of the Ds spectrum. Recently, Guion et al. described a 13-year-old boy with Poland anomaly associated with fronto-nasal dysplasia and contralateral hypoplasia of the lower limbs. Furthermore, Mossoni and Kamath reported a case of bilateral congenital deficiency of the pectoralis major muscles in a 72-year-old female cadaver. They also noticed the absence of the lateral pectoral nerves, suggesting that the deficiencies of the muscles were congenital malformations resulting from a developmental failure of the embryonic muscles, rather than a sequel of poliomyelitis.

Similarly, Poland’s syndrome has been associated with harmartomas and acquired perforating dermatosis. The association with bleeding disorder, as in the case of our patient, is rare. Spontaneous bleeding may occur when the platelet count falls to or bellow 50 x lOVliter. Our patient might have suffered a combined effect of thrombocytopenia and defective platelet aggregation/adhesion, to have experienced such episodes even at a platelet count level of 75xl0/litre. It is difficult to rule out localized vascular and other connective tissue abnormalities, which could explain why such spontaneous bleeding episodes occurred in a particular region at all times. Regrettably, it was not possible to undertake detailed assessment (such as Antithrombin III, protein C, D-Dimer, platelet function tests, etc.) at the time of presentation. Fedorov and Rozanova reported their observations on hematological disorders in 14 women with Poland’s syndrome. The patients had thrombocytopenia and reduced platelet function (adhesion and aggregation). They noted that the hemostatic disorder depended on the degree of platelet deformity and observed good response to treatment, resulting in only minimal hemorrhage from soft tissues during operative procedures after medical treatment. The reason for cessation of further bleeding episodes in our patient after medical treatment, however, remains to be determined. In some occasions, the syndrome may pass undiagnosed, as it may pose no obvious structural or functional deformities. Phaltanker et al. recently described three such cases of undiagnosed Poland’s syndrome in patients who passed routine medical examination as well as successfully completed basic training in the army. However, physical therapy, as is being given to our patient, helps to develop muscles of the shoulder girdle, thereby improving function. Several reconstructive procedures are available to correct the functional and structural deformities associated with this syndrome. For the chest wall deformity, customized silicone prosthesis has been found to be very simple and safe. Transposition of latissimus dorsi muscle for such soft-tissue reconstruction has been used by many authors with satisfactory aesthetic and functional results. Our choice of this latter method for our patient was informed both by our past experience and the wish of the patient and her parents. Follow-up is mandatory in these patients even after reconstruction in order to detect pathologies that may arise later. Although only a few cases of carcinoma of the breast have been reported in patients with Poland’s syndrome, the disease could progress rapidly to involve neighboring structures due to the hypoplastic nature of the breast. This is akin to what obtains when a male breast is involved in such a malignancy, and treatment modalities follow known standards. In conclusion, Poland’s syndrome is a rare congenital disorder that needs to be borne in mind, as appropriate treatment can yield gratifying results. Its association with hematological/bleeding disorders needs further research.  Get the medication you need. Buy celecoxib 200 online

ACKNOWLEDGEMENT

The support rendered by all staff of the department of Hematology, Usmanu Danfodiyo University Teaching Hospital, Sokoto, in the management of this patient, is well appreciated. Atomoxetine hcl