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The shoulder and pectoral girdle
Published in Ashley W. Blom, David Warwick, Michael R. Whitehouse, Apley and Solomon’s System of Orthopaedics and Trauma, 2017
Sprengel’s deformity Deformity is the cardinal symptom and it may be noticed at birth. The shoulder on the affected side is elevated; the scapula looks and feels abnormally high, smaller than usual and somewhat prominent; occasionally both scapulae are affected. The neck appears shorter than usual and there may be kyphosis or scoliosis of the upper thoracic spine. Shoulder movements are painless but abduction and elevation may be limited by the fixation of the scapula.
The use of Fat-Augmented Latissimus Dorsi (FALD) flap for male Poland Syndrome correction: a case report
Published in Case Reports in Plastic Surgery and Hand Surgery, 2022
Fabio Santanelli di Pompeo, Michail Sorotos, Guido Paolini, Gennaro D’Orsi, Guido Firmani
Poland Syndrome (PS) is a sporadic congenital disease with a wide spectrum of presentations [1,2]. Typical features include the absence of the sternum-costal head of the pectoralis major (PM) muscle, along with glandular and subcutaneous tissue hypoplasia and thin overlying skin. Sometimes the nipple-areola complex (NAC) appears underdeveloped or missing. Clinical presentation may include skeletal deformities, costal cartilage and rib defects, ipsilateral upper limb malformations or elevation and rotation of the scapula, also known as Sprengel deformity [3]. The causes for PS are unknown, although a disruption of the embryonic blood supply to the subclavian arteries seems to be the most accepted hypothesis [4]. The prevalence at birth of this syndrome is of about 1–3 in 100.000 individuals, with male predominance at a ratio of 2:1–3:1 [5].
Unexpected finding of thyroid hemiagenesis in a patient presenting with a right thyroid nodule and a history of Poland syndrome
Published in Journal of Endocrinology, Metabolism and Diabetes of South Africa, 2023
Cheikh Ahmedou Lame, Birame Loum, Peter Mazzaglia, Cheikhna Ndiaye, Thierno Boubacar Diallo, Abibou Ndiaye, Agnes Diouf, Aly Toure
Poland syndrome has been described in association with other congenital abnormalities including Sprengel deformity of the scapula, Klippel–Feil spinal abnormalities, as well as both Moebius and Adams–Oliver syndromes.5 Association of left-sided Poland syndrome and dextrocardia is not uncommon. The theory most cited as an explanation for this is that hemiagenesis of at least 2 ribs pushes the developing heart to the right.27 Most often in cases of Poland syndrome in women, it is the lack of development of breast tissue that is most troubling for the patient, and ultimately prompts a consultation for cosmetic surgical repair.1,5 However, in our patient, this was not the case, and her main aesthetic concern was the visible right-sided goitre.
Anaesthetic challenges in a patient with Klippel–Feil syndrome scheduled for panendoscopy and biopsy*
Published in Southern African Journal of Anaesthesia and Analgesia, 2018
The patient was a known case of Klippel–Feil syndrome with a Type II variant associated with Sprengel’s deformity. His other medical history included spina bifida affecting the thoracic spine, unilateral renal agenesis and previous corrective heart surgery as a child for congenital heart disease. He took amlodipine to control his hypertension.