Poland Syndrome and Pregnancy
Year 2018,
Volume: 19 Issue: 3, 265 - 267, 30.09.2018
Emre Zafer
,
Selda Demircan Sezer
,
Sümeyra Nergiz Avcıoğlu
,
Burcu Bıçakçı
Abstract
Poland syndrome is a rare congenital disorder presenting with absence of unilateral thoracic muscles. Upper extremity, rib and connective tissue deformities usually accompany muscle agenesis or hypoplasia on the same side. Although its etiology remains unknown, inadequate perfusion is suspected for related regional tissue hypoplasia/aplasia. There is scarce information about possible cardiopulmonary function involvement due to abnormal thoracic muscle and tissues. A slight change in this function may be worsened in pregnancy affecting maternal and fetal health. Here we present a 30 weeks pregnant patient with Poland syndrome who developed severe shortness of breath and eventually had to have iatrogenic preterm delivery. Rare syndromes compatible with life and reproduction can be a challenge for clinicians especially during pregnancy due to unpredictable effects on gestational physiologic adaptation processes. Therefore, case reports or series regarding pregnancy complications of these rare syndromes provide valuable information for possible complications and their management options.
References
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1. Baban A, Torre M, Bianca S, Buluggiu A, Rossello MI, Calevo MG, et al. Poland syndrome with bilateral features: case description with review of the literature. Am J Med Genet A 2009; 149: 1597-602.
-
2. Yiyit N, Işıtmangil T, Öksüz S. Clinical analysis of 113 patients with Poland syndrome. Ann Thorac Surg 2015; 99: 999-1004.
-
3. Sethuraman R, Kannan S, Bala I, Sharma RK. Anaesthesia in Poland syndrome. Can J Anaesth 1998; 45: 277-9.
-
4. Cingel V, Bohac M, Mestanova V, Zabojnikova L, Varga I. Poland syndrome: from embryological basis to plastic surgery. Surg Radiol Anat 2013; 35: 639-46.
-
5. Sparks DS, Adams BM, Wagels M. Poland's syndrome: an alternative to the vascular hypothesis. Surg Radiol Anat 2015; 37: 701-2.
-
6. Vaccari CM, Romanini MV, Musante I, Tassano E, Gimelli S, Divizia MT, et al. De novo deletion of chromosome 11q12.3 in monozygotic twins affected by Poland Syndrome. BMC Med Genet 2014; 15: 63.
-
7. OMIM Online Mendelian Inheritance of Man [http://omim.org/clinicalSynopsis/173800?highlight=poland](http://omim.org/clinicalSynopsis/173800?highlight=poland)
-
8. Sharma CM, Kumar S, Meghwani MK, Agrawal RP. Poland syndrome. Indian J Hum Genet 2014; 20: 82-4.
Year 2018,
Volume: 19 Issue: 3, 265 - 267, 30.09.2018
Emre Zafer
,
Selda Demircan Sezer
,
Sümeyra Nergiz Avcıoğlu
,
Burcu Bıçakçı
References
-
1. Baban A, Torre M, Bianca S, Buluggiu A, Rossello MI, Calevo MG, et al. Poland syndrome with bilateral features: case description with review of the literature. Am J Med Genet A 2009; 149: 1597-602.
-
2. Yiyit N, Işıtmangil T, Öksüz S. Clinical analysis of 113 patients with Poland syndrome. Ann Thorac Surg 2015; 99: 999-1004.
-
3. Sethuraman R, Kannan S, Bala I, Sharma RK. Anaesthesia in Poland syndrome. Can J Anaesth 1998; 45: 277-9.
-
4. Cingel V, Bohac M, Mestanova V, Zabojnikova L, Varga I. Poland syndrome: from embryological basis to plastic surgery. Surg Radiol Anat 2013; 35: 639-46.
-
5. Sparks DS, Adams BM, Wagels M. Poland's syndrome: an alternative to the vascular hypothesis. Surg Radiol Anat 2015; 37: 701-2.
-
6. Vaccari CM, Romanini MV, Musante I, Tassano E, Gimelli S, Divizia MT, et al. De novo deletion of chromosome 11q12.3 in monozygotic twins affected by Poland Syndrome. BMC Med Genet 2014; 15: 63.
-
7. OMIM Online Mendelian Inheritance of Man [http://omim.org/clinicalSynopsis/173800?highlight=poland](http://omim.org/clinicalSynopsis/173800?highlight=poland)
-
8. Sharma CM, Kumar S, Meghwani MK, Agrawal RP. Poland syndrome. Indian J Hum Genet 2014; 20: 82-4.