Acute abdomen due to an infected urachal cyst in a 5-year-old female: case report

The urachus is an embryonic tube that connects the upper portion of the bladder to the umbilicus, and obliterates normally during embryonic development stages forming the median umbilical ligament. Incomplete obliteration of this tube results in many anomalies such as congenital patent urachus, umbilical urachal sinus, vesicourachal diverticulum and urachal cyst. We report in this case a 5-year-old female presented to the Emergency, complaining of generalized abdominal pain, fever, vomiting, and constipation with no umbilical discharge. The clinical presentation accompanied by radiology investigations suggested a case of acute abdomen. We performed an exploratory laparotomy and found a mass above the bladder connected to the umbilicus; we excised the mass and sent a specimen to pathology that confirmed Urachal cyst. Urachal cyst is usually asymptomatic unless it is complicated; depending on our case, we recommend surgical management by complete excision for complicated urachal cyst.

Extraperitoneal radical trachelectomy with pelvic lymphadenectomy

Radical trachelectomy combined with pelvic lymphadenectomy has been used to treat patients with early-stage cervical cancer who wish to preserve their fertility. Vaginal, abdominal, laparoscopic, and robotic approaches have been employed during this procedure, but all cause peritoneal damage, which could result in periadnexal adhesion. As periadnexal adhesion can lead to female infertility due to restricted sweeping of the fimbria over the ovary, it is important to minimize peritoneal damage during the fertility-preserving surgery. Aiming to minimize peritoneal damage, we recently developed a new surgical approach. The techniques used are similar to those used for type III radical hysterectomy; however, all procedures are performed via the extraperitoneal approach.In this video article, we describe a step-by-step technique of this new fertility-preserving surgical procedure. Surgical procedures are as follows: (1) extraperitoneal pelvic lymphadenectomy, (2) excision of the vesicohypogastric fascia and median umbilical ligament, (3) bladder dissection from the peritoneum and identification of uterine cervix, (4) transection of the cardinal ligaments and vesicouterine ligaments, (5) transection of the vagina, (6) excision of the rectovaginal and uterosacral ligaments, (7) transection of the uterine cervix, (8) cervical cerclage and placement of a Foley catheter, (9) anastomosis of the uterine cervix, (10) suture of the median umbilical ligament and vesicohypogastric fascia. During these procedures, the uterine arteries, inferior hypogastric nerve, and pelvic splanchnic nerve were preserved. The advantages of this new surgical approach are first, peritoneal injuries can be completely avoided as the procedure is performed extraperitoneally, and second, it can be carried out using conventional low-cost instruments. In view of these features, we consider that this technique could be an ideal treatment option for selected women with early-stage cervical cancer. The oncological and reproductive outcomes of this new surgical approach need to be evaluated in future clinical studies.

Urachal remnant causing umbilical in-drawing during micturition

The urachus is a vestigial remnant of the allantois, which is normally obliterated during fetal life to become the median umbilical ligament, which runs between the urinary bladder and umbilicus in adults. Failure of obliteration leaves a tubular urachal remnant, which may present with disease. We report a unique case of a urachal remnant causing umbilical pain and in-drawing on micturition in a nine-year-old boy. There was no urine discharge from the umbilicus and in-drawing did not occur on defecation. His urinary stream was normal. High frequency ultrasonography revealed a thick band with a narrow, anechoic, fluid filled central channel. Exploration via an infraumbilical curvilinear incision identified a thick urachal band that could be traced to the dome of the bladder. This was excised flush with the bladder. The patient remains well at nine months following surgery with complete cessation of symptoms.

Costello Syndrome and Umbilical Ligament Rhabdomyosarcoma in Two Pediatric Patients: Case Reports and Review of the Literature

Costello syndrome is caused by heterozygous de novo missense mutations in the protooncogene HRAS with tumor predisposition, especially rhabdomyosarcoma. We here report two pediatric patients with Costello syndrome and umbilical ligament rhabdomyosarcoma. A review of the literature published in English in MEDLINE from January 1971 to June 2016 using the search terms “Costello syndrome” and “rhabdomyosarcoma” was performed, including two new cases that we describe. Twenty-six patients with Costello syndrome and rhabdomyosarcoma were recorded with mean age of diagnosis of 2 years and 8 months. The most common tumor location was the abdomen/pelvis, including four out of ten of those in the umbilical ligament. The most common histological subtype was embryonal rhabdomyosarcoma. Overall survival was 43%. A total of 17 rhabdomyosarcomas in pediatric patients arising in the umbilical ligament were recorded with mean age of diagnosis of 3 years and 4 months. Overall survival was 69%. Costello syndrome is a poorly known disorder in pediatric oncology but their predisposition to malignancies implies the need for a new perspective on early diagnosis and aggressive medical and surgical treatment.

A Rare Cause of Urachal Adenocarcinoma: Urachal Diverticle

Urachus is the remnant of the embryologic allantois and the fetal bladder, extending form the bladder roof to the umbilicus. It degenerates in the prenatal period into a tissue band known as the median umbilical ligament. Incomplete degeneration may lead to urachal diverticle development. It is difficult to diagnose unless it is considered in differential diagnosis and imaging modalities are employed. This paper describes a patient treated with partial cystectomy for urachal diverticle, and the pathologic examination revealed urachal adenocarcinoma.