The Risk of Malignant Degeneration of Müllerian Derivatives in PMDS: A Review of the Literature.

Persistent Müllerian Duct Syndrome (PMDS) is a rare autosomal recessive disorder of sex development characterized by the presence of fallopian tubes, uterus and upper one-third of the vagina in individuals with XY genotype and normal male phenotype. The main complications of PMDS are infertility and the rare risk of malignant degeneration of both testicular and Müllerian derivatives. We report the case of a 49-year-old man who, during repair of an incisional hernia, was incidentally found to have a uterine-like structure posterior to the bladder. In the past at the age of 18 months, he had undergone bilateral orchidopexies for bilateral cryptorchidism. The intraoperative decision was to preserve the uterine-like structure and make a more accurate diagnosis postoperatively. Evaluation revealed an XY chromosome and imaging consistent with PMDS. The patient was informed about the risk of neoplastic transformation of the residual Müller ducts and was offered surgical treatment, which he declined. Subsequent follow-up imaging studies, including testicular and pelvic ultrasound, were negative for findings suggestive of malignant testicular and Mullerian derivative degeneration. A review of the international literature showed that, when a decision is taken to remove the Mullerian derivatives, laparoscopy and especially robotic surgery allow for the successful removal of Müllerian derivatives. Whenever the removal of these structures is not possible or the patient refuses to undergo surgery, it is necessary to inform the patient of the need for adequate follow-up. Patients should undergo regular pelvic imaging examination and MRI might be a better method for that purpose.

Severe rectal prolapse and solitary rectal ulcer in a young anorectic woman with myxoid degeneration of visceral adipose tissue.

Rectal prolapse is a disorder in which the rectum protrudes from the anal canal. Solitary rectal ulcer may coexist. Both conditions have been associated with chronic constipation and excessive straining during defecation. Rectal prolapse has been rarely reported in women suffering from anorexia nervosa. Lack of rectal support because of loss of ischiorectal fat has been proposed as one of the possible mechanisms in this condition, together with chronic constipation and abuse of laxative. We report the case of an anorexic woman with a severe rectal prolapse and bleeding requiring urgent Altmeier's procedure. Surgery was complicated by dehiscence of the anastomosis and volvulus, requiring ileostomy and laciniae debridement. Pathological analysis of all the surgical samples taken from different abdominal sites highlighted changes in the visceral adipose tissue consisting in nodular aggregates of small adipocytes dispersed in a myxoid matrix surrounding blood vessels within abundant fibrosis. The morphologic features resemble those observed in primordial fetal fat and are comparable to those observed in cancer associated cachexia. The diffuse myxoid degeneration of visceral adipose tissue may play a role in the pathogenesis of rectal prolapse in patients with anorexia nervosa. Besides starvation, the mechanism sustaining myxoid degeneration of the adipose tissue is not entirely clear. Whenever possible improving nutritional and clinical conditions should be ideal before any surgical approach.

Cardiovascular risk in chronic autoimmune thyroiditis and subclinical hypothyroidism patients. A cluster analysis.

BACKGROUND: Subclinical hypothyroidism (SCH) and chronic autoimmune thyroiditis (CAT) are linked to an increased risk of atherosclerosis and coronary heart disease (CHD). The aim of this study was to look for positive markers of CHD and correlations with thyroid blood tests in patients with SCH or CAT, but no symptoms of CHD, so as to identify CHD risk conditions that otherwise would likely be missed. METHODS: We measured a series of thyroid, clinical-metabolic and cardiovascular parameters in 30 consecutive endocrinology patients enrolled in our ambulatory endocrinological referral center of "Sapienza" University of Rome. (19 with CAT, 11 with SCH) from January 2015 to March 2015. 13 asymptomatic subjects were enrolled as controls. In each patient, we measured a series of 34 thyroid, clinical-metabolic and cardiovascular parameters. RESULTS: in the statistical analysis of collected data, the oblique principal components clustering procedure (OPC) revealed the presence of an interesting mixed cluster, composed of a thyroid parameter (TPO-Ab), a metabolic parameter (homocysteine level) and a cardiovascular parameter (MAPSE), in which we assessed the relationships between the single components. Our preliminary results indicate that in both groups of patients elevated TPO-Ab, when accompanied by reduced MAPSE and increased IMT and homocysteine values, may be taken to indicate the presence of clinically unrecognized CHD. CONCLUSIONS: Confirmation of these results in larger series of patients could justify hormone therapy for prevention of CHD in these thyroid patients versus placebo treatment.

Appendiceal abscess in a giant left-sided inguinoscrotal hernia: a rare case of Amyand hernia.

The hernia of Amyand is an inguinal hernia containing the appendix in the sac. It is a rare pathology often diagnosed only intra-operatively. We report a case even more rare of a giant left-sided inguinoscrotal Amyand hernia with appendiceal abscess without clinical findings of incarceration/strangulation, occlusion, perforation, or acute scrotum and with the presence in the sac of the caecum and other anatomical structures (last ileal loops, bladder and omentum). The 68-years-old man patient successfully underwent surgical treatment only through the hernia sac (meshless repair according to Postempski technique).