Congenital hepatic fibrosis (CHF) is considered to be a rare autosomal recessive hereditary fibrocystic liver disease, mainly found in children. However, cases of adult CHF with autosomal dominant polycystic kidney disease (ADPKD) caused by PKD1 gene mutation are extremely rare. We report a 31-year-old female patient admitted for esophageal and gastric variceal bleeding. Physical examination revealed significant splenomegaly, biochemical tests showed a slight increase in liver enzymes, and a decrease in platelet count. Imaging examinations showed significant dilatation of the common bile duct and intrahepatic bile ducts, as well as multiple renal cysts. Liver biopsy revealed enlarged portal areas, bridging fibrosis, and numerous variably shaped small bile ducts. Genetic testing identified two unique mutations in the PKD1 gene, identified as biallelic mutations compound heterozygous mutations composed of a mutation inherited from the father (c.8296 T > C) and one from the mother (c.9653G > C). Based on multiple test results, the patient was diagnosed with the portal hypertension type CHF associated with ADPKD. During her initial hospital stay, the patient underwent endoscopic treatment for gastrointestinal bleeding. To date, the patient has recovered well. Moreover, a significant reduction in varices was observed in a gastroscopy examination 18 months later.

BACKGROUND: Disorders of sex development are anomalies in which the development of urogenital ridge is undifferentiated for the male and female child. Imaging plays a vital role in investigating the gross anatomy and associated anomalies. Ultrasonography, such as genitography and magnetic resonance, is the primary modality for demonstrating internal gonads and genitalia. Early multidisciplinary approach in the management of ambiguous genitalia including early surgical intervention is the predominant practice, with few current considerations on deferral of genital reconstruction until adolescent age.
METHODS: We report the rare case of a 24-year-old adult female from a majority ethnic group of the Volta region, Ghana who was diagnosed and raised as male, now requiring surgical restoration to the female gender. The surgical team decided to assign external genitalia to correspond with the already intact internal organs, thus constructing the vulva. Consent was given by the client and her family members for management and surgical intervention. The surgery was scheduled and duly performed with a successful outcome. Understanding and consent was sought from the patient for the purpose of using her images for teaching, scientific publication, and demonstrations.
CONCLUSIONS: The advantages of deferring surgical reconstruction with psychological counseling after early assessment need to be considered to prevent inappropriate gender assignment.

UNASSIGNED: Amyand\'s hernia is a rare type of inguinal hernia with an incidence of about 0.1% of all inguinal hernias with most in occurring in childhood. It is characterized by the presence of the vermiform appendix within the hernia sac.
METHODS: We report the case of 40-year-old female who underwent inguino-labial hernia repair with an incidental finding of a normal appendix within the sac; this was not predicted by the pre-operative ultrasound scan.
UNASSIGNED: We recommend that a detailed ultrasound scan be done for all patients with an inguinal hernia to help to manage the patient timeously and safely.
CONCLUSIONS: We present a rare condition in a 40-year-old female with a right inguinal hernia, an Amyand\'s hernia.

BACKGROUND: Among adult females, Hydrocele of Canal of Nuck (HCN) is a very rare condition. The majority of the reported cases of HCN were not conclusively diagnosed until surgery was performed on a suspected inguinal hernia. To the best of our knowledge, this is the first case of laparoscopically operated HCN in adult female in Middle East and North Africa Region, and the fourth such case worldwide.
METHODS: A 36-year-old female presented with a painful small swelling in the right groin of 3 months duration. The swelling extended to the right labia majora while standing, and disappeared when the patient was in prone position. There was no lymph node enlargement, no other masses in the abdomen, and no swelling in the contralateral side. Ultrasonography was undertaken and the patient was admitted electively for laparoscopic exploration and repair of non-complicated right inguinal hernia. She was known to have dyslipidemia on medications. Further history and physical examination were unremarkable. Intraoperatively it was diagnosed to be right-sided HCN which was excised and repaired by mesh fixation using transabdominal preperitoneal (TAPP) approach. Her follow up course was unremarkable.
CONCLUSIONS: This case expands the clinical and intraoperative potential differential diagnosis of adult female groin masses. Surgeons should consider such cases when they observe such swellings intraoperatively. Laparoscopic TAPP approach in such cases is a superior diagnostic and treatment modality.

BACKGROUND: Neuroleptic malignant syndrome is a rare but life-threatening idiosyncratic complication following the use of antipsychotic agents, anaesthesia and surgery. It is characterized by hyperthermia, muscle rigidity, autonomic disturbances and mental state alterations.
METHODS: A 31 year old female weighing 60kg received a depot preparation of Fluphenazine on account of depression with psychotic features observed two days prior to elective Cholecystectomy under general anaesthesia. Surgery and anaesthesia were essentially uneventful. Forty eight hours post-operatively, the patient developed features suggestive of neuroleptic malignant syndrome complicated by aspiration pneumonitis. This necessitated her admission into the intensive care unit. She had prolonged stay in the intensive care unit, where she was mechanically ventilated, developed deep venous thrombosis of the left upper limb and required tracheostomy on account of prolonged endotracheal intubation. Patient recovered fully following bromocriptine and dantrolene therapy. She was discharged home after 60 days on admission and has remained in good health.
CONCLUSIONS: Though rare, neuroleptic malignant syndrome can occur in young adult females following use of antipsychotics, anaesthesia and surgery. Its clinical course can be prolonged and distressing with the use of depot preparations. Early diagnosis and prompt supportive measures are essential to reduce morbidity and mortality.