A Case of Severe Hyperammonemic Encephalopathy Caused by Urinary Tract Infection in Obstructive Uropathy

Hyperammonemia is mainly caused by diseases related to liver failure. However, there are also non-hepatic causes of hyperammonemia, such as urinary tract infection (UTI) due to urease-producing organisms. Urease production by these bacteria induces a hydrolysis of urinary urea into ammonia that can cross the urothelial cell membrane and diffuse into blood vessels, leading to hyperammonemia. Delayed diagnosis and treatment of hyperammonemia can lead to lethal encephalopathy that can cause brain damage and life-threatening conditions. In the presence of obstructive uropathy, UTI by urease-producing bacteria can lead to more severe hyperammonemia due to enhanced resorption of ammonia into the systemic circulation. In this report, we present a case of acute severe hyperammonemic encephalopathy leading to brain death due to accumulation of ammonia in blood caused by Morganella morganii UTI in a 10-year-old girl with cloacal anomaly, causing obstructive uropathy even after multiple corrections.

A rare case of persistent urogenital sinus in an adult woman associated with unilateral rudimentary horn with ipsilateral renal agenesis and contralateral dermoid cyst

Persistent urogenital sinus (PUGS) is an uncommon developmental cloacal anomaly, with Incidence of 0.6 in 10000 female births. Herein we depict the case of a 22-year of age lady who presented with Infertility for 16 months with dyspareunia and was found to have Persistent urogenital sinus associated with other urogenital anomalies as unilateral rudimentary horn with ipsilateral renal agenesis and contralateral dermoid cyst. The patient was successfully treated with the excision of the sinus, the rudimentary horn and the dermoid cyst.

Prenatale echografische diagnose van een cloacale malformatie

Prenatal ultrasound diagnosis of a cloacal malformation Cloacal malformations are a spectrum of rare congenital disorders caused by the arrest of the cloacal division during different stages in early embryogenesis. They necessitate critical intervention at birth, posing important surgical challenges. The majority unfortunately remains undetected during prenatal screening. Knowledge about sonographic red flags, however, may improve antenatal detection and therefore postnatal outcome as a result of a timely referral to multidisciplinary tertiary centers. A 29-year-old patient presents at the hospital due to decreased fetal movements in the third trimester of pregnancy. The ultrasound reveals an anechoic bilobed cystic mass with a thick midline septum within the pelvis of a female fetus. The diagnosis of a cloacal anomaly with associated duplicated vaginas and uteri is made. Severe accumulation of urine lies at the base of bilateral hydrocolpos, peritoneal ascites and a urinary tract obstruction with an adverse influence on both pulmonary and renal development. The baby is prematurely delivered by an emergency caesarean section. Her condition is poor, requiring immediate respiratory resuscitation. An explorative laparotomy on the following day confirms the diagnosis. The first steps towards anatomical correction are taken. It may be concluded that the triad of bilateral hydronephrosis, a pelvic cystic mass and an absent normal bladder within a female fetus should always raise suspicion of a cloaca.

DIAGNOSTICS AND TREATMENT OF THE CLOACA POSTERIOR IN A 3-YEAR-OLD CHILD

The posterior cloaca (cloaca posterior) is the most rare type of cloacal anomaly representing one of the most complicated problems for the operative correction of the defect. A variety of options for the fusion of structures involved in the formation of urogenital sinus, vaginal and uterine anomalies cause the lack of a unified approach to surgical correction which would allow to obtain good anatomical and functional outcomes and a decent quality of life. The authors discuss a clinical case of successful treatment of a rare form of anorectal malformation - cloaca posterior in a 3-year old child.

Closure of a Recurrent Urethrovaginal Fistula in a Girl with Cloacal Anomaly Using Deflux Injection

In a girl born with cloaca, both hemivaginae and rectum were located above the bladder neck, and both ureters were connected to the hemivaginae. After diverting colostomy and cystovaginoscopy on the second day of life, the repair of cloaca was performed at 10 months of age by posterior sagittal anorecto vaginoplasty (PSARVP), including laparotomy and bilateral ureteric reimplantation. Eight months after the surgery, she developed a vesicovaginal fistula, which was repaired and closed by open surgery through the bladder. Three months after this procedure, a tiny urethrovaginal fistula was noticed, which was closed at the age of 2 years using hook diathermy to refresh the edges and was then closed by Deflux injection. The proper closure of the urethrovaginal fistula was confirmed by radiology and cystoscopy 3 months after the surgery. This report shows that injection of Deflux into a tiny urethrovaginal fistula following refreshing the edges may be a valid treatment option in selected cases.