Surgical Management of Spontaneous Ruptured Hepatocellular Adenoma

AIMS

Spontaneous ruptured hepatocellular adenoma (SRHA) is a rare life-threatening condition that may require surgical treatment to control hemorrhaging and also stabilize the patient. We report a series of emergency surgeries performed at our institution for this condition.

METHODS

We reviewed medical records and radiology files of 28 patients (from 1989 to 2006) with a proven diagnosis of hepatocellular adenoma (HA). Three (10.7%) of 28 patients had spontaneous ruptured hepatocellular adenoma, two of which were associated with intrahepatic hemorrhage while one had intraperitoneal bleeding. Two patients were female and one was male. Both female patients had a background history of oral contraceptive use. Sudden abdominal pain associated with hemodynamic instability occurred in all patients who suffered from spontaneous ruptured hepatocellular adenoma. The mean age was 41.6 years old. The preoperative assessment included liver function tests, ultrasonography and computed tomography.

RESULTS

The surgical approaches were as follows: right hemihepatectomy for controlling intraperitoneal bleeding, and right extended hepatectomy and non-anatomic resection of the liver for intrahepatic hemorrhage. There were no deaths, and the postoperative complications were bile leakage and wound infection (re-operation), as well as intraperitoneal abscess (re-operation) and pleural effusion.

CONCLUSION

Spontaneous ruptured hepatocellular adenoma may be treated by surgery for controlling hemorrhages and stabilizing the patient, and the decision to operate depends upon both the patient’s condition and the expertise of the surgical team.     

Biallelic loss‐of‐function WNT5A mutations in an infant with severe and atypical manifestations of Robinow syndrome

Robinow syndrome (RS) is a well‐recognized Mendelian disorder known to demonstrate both autosomal dominant and autosomal recessive inheritance. Typical manifestations include short stature, characteristic facies, and skeletal anomalies. Recessive inheritance has been associated with mutations in ROR2 while dominant inheritance has been observed for mutations in WNT5A, DVL1, and DVL3. Through trio whole genome sequencing, we identified a homozygous frameshifting single nucleotide deletion in WNT5A in a previously reported, deceased infant with a unique constellation of features comprising a 46,XY disorder of sex development with multiple congenital malformations including congenital diaphragmatic hernia, ambiguous genitalia, dysmorphic facies, shortened long bones, adactyly, and ventricular septal defect. The parents, who are both heterozygous for the deletion, appear clinically unaffected. In conjunction with published observations of Wnt5a double knockout mice, we provide evidence for the possibility of autosomal recessive inheritance in association with WNT5A loss‐of‐function mutations in RS.     

Potential Effect of Using ABO-Compatible Living-Donor Liver Transplantation

Liver transplantation increased 1.84-fold from 1988 to 2004. However, the number of patients on the waiting list for a liver increased 2.71-fold, from 553 to 1500. We used a mathematical equation to analyze the potential effect of using ABO-compatible living-donor liver transplantation (LDLT) on both our liver transplantation program and the waiting list. We calculated the prevalence distribution of blood groups (O, A, B, and AB) in the population and the probability of having a compatible parent or sibling for LDLT. The incidence of ABO compatibility in the overall population was as follows: A, 0.31; B, 0.133; O, 0.512; and AB, 0.04. The ABO compatibility for parent donors was blood group A, 0.174; B, 0.06; O, 0.152; and AB, 0.03; and for sibling donors was A, 0.121; B, 0.05; O, 0.354; and AB, 0.03. Use of LDLT can reduce the pressure on our liver transplantation waiting list by decreasing its size by at least 16.5% at 20 years after its introduction. Such a program could save an estimated 3600 lives over the same period.     

Restoration of the Occlusal Vertical Dimension with an Overlay Removable Partial Denture: A Clinical Report

The process of tooth loss throughout life associated with severe occlusal wear may pose a challenge in the rehabilitation of partially edentulous arches. In these cases, many therapeutic procedures are necessary because each tooth must be restored to obtain the correct anatomical contour and recover the occlusal vertical dimension (OVD). A removable partial denture (RPD) with occlusal/incisal coverage, also known as an overlay RPD, is an alternative treatment option with fewer interventions, and, consequently, lower cost. This clinical report reviews the principles involved in the clinical indication for an overlay RPD, as well as the necessary planning and execution, to discuss the feasibility and clinical effectiveness of this treatment, identifying the indications, advantages, and disadvantages of this procedure through the presentation of a clinical case. The overlay RPD can be an alternative treatment for special situations involving partially edentulous arches in patients who need reestablishment of the OVD and/or realignment of the occlusal plane, and it can be used as a temporary or definitive treatment. The main advantages of this type of treatment are its simplicity, reversibility, and relatively low cost; however, further studies are needed to ensure the efficacy of this treatment option.