Pérez DMÁ, Ferrer MR, Montero AA

Language: Spanish
References: 15
Page: 432-442
PDF size: 405.36 Kb.

ABSTRACT

Introduction: Poland syndrome is a rare congenital disorder characterized by the unilateral absence or hypoplasia of the pectoralis major muscle associated with malformations of the upper limb and ipsilateral breast tissue. Clinical manifestations in newborns are only described in the literature, therefore, many aspects of their presentation in nurseries are ignored.
Presentation of the case: we present the case of a newborn who manifests paradoxical respiration from the moment of birth. This type of clinical finding is loosely described in Poland Syndrome and even less in the neonatal period.
Discussion: this syndrome can be related to diseases, such as paralysis of pairs VI and VII, which leads to a greater number of complex associations.9 It arises in association with dextrocardia, which according to some authors is secondary to the thoracic deformity caused by malformations ribs and their effect on cardiac formation in the embryonic period. No mental deficiencies or genetic transmission of the disease have been observed. An exceptional family association has been described. This could correspond to an autosomal dominant inheritance with reduced penetration.
Conclusions: in this patient, Poland syndrome was characterized by hypoplasia of the left pectoralis major, associated with skeletal malformations of the hand on the same side.

REFERENCES

1. Ruiz Botero F, Quintana Peña V, Pachajoa CH. Secuencia de Poland, de las bases embriológicas a la práctica clínica. Rev Cubana Ped. 2015; 87(2): 241-46.

2. Melo Victores MR, Alvaré Alvaré L, Lobato Pastrana MD, González Concepción I. Síndrome de Poland atípico. Invest Medicoquir. 2016; 8(2): 288-93.

3. Reshma S, Vijai R, Chakarvarthy N. Poland’s syndrome: a rare case report. Int Surg J. 2017; 4(10): 3526-28.

4. Terán CG, Antezana AN. Síndrome de Poland: descripción de un caso en la sala de partos. Gac Med Bol. 2018; 41(1): 64-6.

5. Torres Aja L, Caballero Aja N. Síndrome de Poland. Presentación de un caso y revisión de la literatura. Rev Finlay. 2016; 6(4): 329-33.

6. Ramírez Lluch N, Acevedo Echevarría JM. Deformidad de la caja torácica. Rev Chil Cir. 2018; 70(4): 373-81.

7. Ruiz Botero F, Quintana Peña V, Pachajoa H. Secuencia de Poland, de las bases embriológicas a la práctica clínica. Rev Cub Ped. 2015; 87(2): 241-46.

8. Vázquez Albornoz JH, Aguirre Zavala JE, García Ordoñez GF, Aguirre Zavala MF. Serie de Casos: Síndrome de Poland Completo vs. Incompleto. Resolución Quirúrgica. Rev. méd. Hosp. José Carrasco Arteaga; 2018; 10(1): 61-8.

9. Meléndez Negrette F, Monroy S, Serrano Montes S, Lizcano EN. Características por imagen del síndrome de poland: presentación de un caso. Rev Col Radiol. 2015; 26(2): 4220-2.

10. Bansal A, Reddy K, Dinsmore K, Gonzalez Estrada A. Poland syndrome: a case report. BMJ [Internet]. 2017 [citado 3/9/2019]. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5624099/

11. Agranovich OE, Komolkin IA, Dimitrieva AJ. Poland’s síndrome. Pediatric Traumatol Orthop Reconstructive Surg. 2017; 5(1): 63-70.

12. Giri D, Patil P, Hart R, Didi M, Senniappan S. Congenital hyperinsulinism and Poland syndrome in association with 10p13-14 duplication. [Internet]. 2017 [citado 3/9/2019]. Disponible en: https://edm.bioscientifica.com/view/journals/edm/2017/1/EDM16-0125.xml

13. Petleshkova P, Krasteva M, Gencheva D, Anesteva Ivanova N, Grozdanova L, Parahuleva N, et al. Poland syndrome: Two cases of new-borns with left-sided chest defect and extrocardia. Biomedical Research. 2019; 30(2): 362-65.

14. Konuk S. Poland Syndrome Case Study. Arch Clin Med Case Rep. 2019; 3(4): 135-39.

15. Ozkok S, Erol N, Onal C, Yikilmaz A. Left-sided Poland’s syndrome associated with dextrocardia. North Clin Istanb. 2019; 6(2): 192–95.