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Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism in which uterus and fallopian tubes are present in a genotypic and phenotypic male. Over 150 cases have been reported in the literature, mostly in adults.1,2 PMDS is likely to be encountered during surgery for undescended testis and inguinal hernia, and surgeons should be aware of this disorder and the options of surgical management. We report a case of PMDS and discuss the etiology, pathogenesis, anatomic types and surgical management. Case ReportA three-month-old Saudi male infant was referred to the Pediatric Surgery clinic for bilateral cryptorchidism. His parents were not related, and there was no family history of undescended testis. On examination, both testes were undescended and impalpable. The penis and scrotum were normal in appearance. A pelvic ultrasound (US) had shown absence of testes and no other abnormality. Human chorionic gonadotrophin stimulation (HCG) test confirmed the presence of testicular tissue.At seven months of age, there was a palpable left testis within an inguinal hernia. A month later at surgery, the uterus, fallopian tubes and both testes were unexpectedly found within the hernia sac (Figure 1). Testicular biopsies were performed and the testes, uterus and fallopian tubes returned into the pelvis, followed by herniorrhaphy. Postoperatively, chromosome analysis confirmed a normal male 46, XY karyotype, and a pelvic US showed a uterus. Histology of the testicular biopsies revealed immature testes consistent with cryptorchid testes.At the age of 15 months, the patient underwent bilateral proximal salpingectomies, leaving the fimbria with each epididymis, corporeal hysterectomy and pedicles of myometrium left with the vasa deferentia. The cervix was split longitudinally in the midline to achieve successful bilateral orchidopexy after mobilizing the internal spermatic vessels. Postoperative follow-up after one year showed both testes in the scrotum to be normal.

Pancreatoblastoma (PB) is a rare pancreatic neoplasm which arises when a group of pancreatic cells start to go through uncontrollable growth. The diagnosis of PB is challenging due to its vague symptoms. The initial diagnosis is made by imaging, afterwards the management is usually by resection of the tumor with or without chemotherapy which depends on the size and grade of the tumor. We report a case of a nine-year-old girl who was diagnosed with pancreotoblastoma and underwent complete resection with chemotherapy.