IntroductionPoland＇s syndrome is a rare congenital anomaly, characterized by abnormalities of the chest wall, breast, spine and upper limb. The incidence of this syndrome has been estimated to be 1：30000. The pathog...IntroductionPoland＇s syndrome is a rare congenital anomaly, characterized by abnormalities of the chest wall, breast, spine and upper limb. The incidence of this syndrome has been estimated to be 1：30000. The pathogenesis is still unclear.展开更多
Zinner syndrome is a rare congenital malformation related to an abnormality in the development of the Wolffian duct, the clinical picture of which consists of a triad of unilateral renal agenesis, ipsilateral seminal ...Zinner syndrome is a rare congenital malformation related to an abnormality in the development of the Wolffian duct, the clinical picture of which consists of a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and obstruction of the ejaculatory duct. Chronic perineoscrotal pain may be an indication of the diagnosis of Z<span style="white-space:nowrap;">ï</span>nner syndrome to some extent. We report the observation of a 23-year-old patient, married and father of one child, who presented with chronic perineo-scrotal pain revealing on morphological assessment (ultrasound, uroscanner, prostatic MRI) a cystic formation of the seminal vesicle, left renal agenesis with an empty left renal compartment. Given the patient’s refusal of any surgical procedure, treatment consisted of echo-guided puncture of the seminal vesicle cyst bringing back 30 cc of a seroviscous liquid whose analysis revealed spermatozoa, without atypical cells, compatible with a cyst. The clinical evolution was marked by a progressive remission of the scrotal pain with a delay of several months.展开更多
Background Since its first description in 1841, numerous variations and treatments of Poland＇s syndrome （congenital deficiency of the pectoralis major muscle associated with brachysyndactyly） have been reported. No...Background Since its first description in 1841, numerous variations and treatments of Poland＇s syndrome （congenital deficiency of the pectoralis major muscle associated with brachysyndactyly） have been reported. None of the reports, however, involved female Chinese patients. Methods A retrospective study of 24 female patients was conducted to guide the selection of methods of surgical reconstruction. The patients were divided into three groups according to the degree of thoracic tissue development. Type I （mild）： Limited tissue loss which can be treated with simple filling with autologous fat and/or an artificial breast implant. Type II （moderate）： Moderate thoracic tissue hypoplasia where the breast parenchyma can still offer adequate implant coverage. Mammoplasty using a latissimus dorsi muscular flap with an implant was performed in this group. The flap was used to fill the infraclavicular hollow, and the implant was placed in the dual-plane pocket. Type Ⅲ （severe）： Severe thoracic tissue hypoplasia, without sufficient parenchyma to offer implant coverage. A latissimus dorsi muscular flap was used to form a total submuscular pocket in which an implant was placed. Results The numbers of Type Ⅰ, Ⅱ, and Ⅲ patients were 15, 3, and 6, respectively. All of the flaps and injected fat demonstrated good survival. Satisfactory cosmetic results were exhibited during the follow-up period of 1 to 9 years. Conclusions Although this group of patients showed varied conditions, they can be roughly divided into three types according to the degree of thoracic tissue development. In our experience, this classification is simple and useful in choosing the breast reconstruction options.展开更多
文摘IntroductionPoland＇s syndrome is a rare congenital anomaly, characterized by abnormalities of the chest wall, breast, spine and upper limb. The incidence of this syndrome has been estimated to be 1：30000. The pathogenesis is still unclear.
文摘Zinner syndrome is a rare congenital malformation related to an abnormality in the development of the Wolffian duct, the clinical picture of which consists of a triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst, and obstruction of the ejaculatory duct. Chronic perineoscrotal pain may be an indication of the diagnosis of Z<span style="white-space:nowrap;">ï</span>nner syndrome to some extent. We report the observation of a 23-year-old patient, married and father of one child, who presented with chronic perineo-scrotal pain revealing on morphological assessment (ultrasound, uroscanner, prostatic MRI) a cystic formation of the seminal vesicle, left renal agenesis with an empty left renal compartment. Given the patient’s refusal of any surgical procedure, treatment consisted of echo-guided puncture of the seminal vesicle cyst bringing back 30 cc of a seroviscous liquid whose analysis revealed spermatozoa, without atypical cells, compatible with a cyst. The clinical evolution was marked by a progressive remission of the scrotal pain with a delay of several months.
文摘Background Since its first description in 1841, numerous variations and treatments of Poland＇s syndrome （congenital deficiency of the pectoralis major muscle associated with brachysyndactyly） have been reported. None of the reports, however, involved female Chinese patients. Methods A retrospective study of 24 female patients was conducted to guide the selection of methods of surgical reconstruction. The patients were divided into three groups according to the degree of thoracic tissue development. Type I （mild）： Limited tissue loss which can be treated with simple filling with autologous fat and/or an artificial breast implant. Type II （moderate）： Moderate thoracic tissue hypoplasia where the breast parenchyma can still offer adequate implant coverage. Mammoplasty using a latissimus dorsi muscular flap with an implant was performed in this group. The flap was used to fill the infraclavicular hollow, and the implant was placed in the dual-plane pocket. Type Ⅲ （severe）： Severe thoracic tissue hypoplasia, without sufficient parenchyma to offer implant coverage. A latissimus dorsi muscular flap was used to form a total submuscular pocket in which an implant was placed. Results The numbers of Type Ⅰ, Ⅱ, and Ⅲ patients were 15, 3, and 6, respectively. All of the flaps and injected fat demonstrated good survival. Satisfactory cosmetic results were exhibited during the follow-up period of 1 to 9 years. Conclusions Although this group of patients showed varied conditions, they can be roughly divided into three types according to the degree of thoracic tissue development. In our experience, this classification is simple and useful in choosing the breast reconstruction options.
