Disorders of sex development (DSD): not only babies with ambiguous genitalia. A practical guide for surgeons.

INTRODUCTION AND METHODS: In this review, we describe the common clinical scenarios that may be present to a paediatric surgeon when a patient has a disorder of sex development (DSD). Our aim was to prepare surgeons so that they can respond with correct approaches to diagnose and manage the given situations. RESULTS: DSD present in three distinct clinical situations: in the neonate with some abnormality of the external genitalia; in the child undergoing surgical treatment for inguinal hernia or during open or laparoscopic orchidopexy or during hypospadias correction; and at or after puberty, which may be precocious or delayed or in an adolescent girl with masculinisation at puberty. We describe the clinical features, likely diagnoses and the recommended management pathway in these scenarios.

Is the ovary in an inguinal hernia 'descended' like a testis or not?

BACKGROUND/AIM: How testes descend through the inguinal canal is well described, but how the ovaries appear inside an inguinal hernia remains controversial. We reviewed the literature to determine whether ovarian 'descent' has been described. METHODS: The English literature from 1950 to 2014 was reviewed via PubMed, Medline and Web of Science to identify papers describing an ovary in the hernia sac: reports were examined to find whether the ovary prolapsed or 'descended', and the anatomy of its supporting ligaments, cranial suspensory ligament (CSL) and round ligament (female gubernaculum, RL). RESULTS: In forty reports of >7140 inguinal herniotomies and/or imaging studies in females the hernia contains an ovary in 15-20%, often with the ipsilateral fallopian tube. The RL and ovary were aligned along the same path as testicular descent only rarely in Müllerian anomalies with an isolated uterine horn preceding the ovary into the sac. The ligament usually found inside the hernia sac was the CSL, not the RL. DISCUSSION: The high frequency of incarcerated ovary, along with the close proximity of the CSL to the internal ring in females is consistent with a sliding hernia pulling the CSL (and ovary) into the hernia sac, not ovarian 'descent' by traction on the RL.

Haemostatic management for aortic valve replacement in a patient with advanced liver disease.

Redo-sternotomy and aortic valve replacement in patients with advanced liver disease is rare and associated with a prohibitive morbidity and mortality. Refractory coagulopathy is common and a consequence of intense activation of the coagulation system that can be triggered by contact of blood with the cardiopulmonary bypass circuitry, bypass-induced fibrinolysis, platelet activation and dysfunction, haemodilution, surgical trauma, hepatic decompensation and hypothermia. Management can be further complicated by right heart dysfunction, porto-pulmonary hypertension, poor myocardial protection, and hepato-renal syndrome. Complex interactions between coagulation/fibrinolysis and systemic inflammatory response syndrome reactions like "post-perfusion-syndrome" also compound haemostatic failure. Given the limited information available for the specific management and prevention of cardiopulmonary bypass-induced haemostatic failure, this report serves to guide the anaesthesia and medical management of future cases of a similar kind. We discuss our multimodal management of haemostatic failure using pharmacological strategies, thromboelastography, continuous cerebral and liver oximetry, and continuous cardiac output monitoring.