Innovative surgical management of idiopathic retroperitoneal fibrosis

Idiopathic retroperitoneal fibrosis is a rare entity usually treated with exploratory laparotomy, deep biopsies of the fibrotic process and uretrolysis. Innovative surgical management occasionally is required for ureteral obstruction. We report the use of dismembered pyeloplasty, autorenal transplantation and bilateral psoas hitch ureteral reimplantation for the management of ureteral obstruction associated with idiopathic retroperitoneal fibrosis.     

Current approach to diagnosis and management of retroperitoneal fibrosis

Retroperitoneal fibrosis is characterized by fibrotic lesions around the abdominal aorta and common ileac artery causing ureteral obstruction. Secondary retroperitoneal fibrosis is associated with malignant disease, drugs, exposure to radiation and surgery. In contrast, the majority of retroperitoneal fibrosis is classified into idiopathic retroperitoneal fibrosis, for which immunological etiology has been suggested. Recently, idiopathic retroperitoneal fibrosis has been considered to be a spectrum of immunoglobulin G4-related disease, a systemic inflammatory disease, the concept of which has been developed during the past decade. In the management of retroperitoneal fibrosis, assessment of systemic lesions associated with immunoglobulin G4-related disease and the exclusion of secondary retroperitoneal fibrosis is mandatory. Histological examination of retroperitoneal lesions is desired for accurate diagnosis and management. Laparoscopic or open biopsy is often beneficial, although it is more invasive than needle biopsy. Treatment for idiopathic retroperitoneal fibrosis consists of meticulous glucocorticoid therapy based on that for immunoglobulin G4-related disease, which is expected to be highly effective. Ureteral obstruction is usually managed with conservative procedures, such as ureteral stenting or percutaneous nephrostomy. The goal of treatment for retroperitoneal fibrosis should be freedom from the stent/nephrostomy with withdrawal of the glucocorticoid in addition to salvage of renal function; however, conservative management does not always provide favorable outcomes. In contrast, aggressive surgical treatment, such as ureterolysis, can achieve the goal; however, the procedure is associated with high morbidity. Establishment of a consensus about treatment for idiopathic retroperitoneal fibrosis, including the optimal indications for the invasive surgical procedure and conservative management, is desired.     

Non-operative management of haemobilia

BACKGROUND: The aim was to evaluate a non-operative approach to the management of haemobilia. METHODS: This was a retrospective analysis of patients presenting over 10 years with haemobilia. All patients had upper gastrointestinal endoscopy, abdominal ultrasonography and digital subtraction angiography. Superselective coil and/or Gelfoam embolization was done as close as possible to the bleeding site. Completion angiography was performed routinely to confirm adequate embolization. RESULTS: There were 23 patients with liver trauma and six with inflammatory conditions. All patients required resuscitation with fluids and blood transfusion, and had the haemobilia controlled successfully by angiographic embolization. There was one death from fulminant hepatic sepsis. CONCLUSION: This series attests to the efficacy of a non-operative approach to haemobilia using radiological diagnosis and intervention.     

Idiopathic retroperitoneal fibrosis

We treated a patient with idiopathic retroperitoneal fibrosis accompanied by right ureteral constriction. Pyelography, ureterography, and abdominal CT scan were pertinent diagnostics. Close collaboration between the surgeon and the urologist is required when attempting to treat such patients.     

Familial retroperitoneal fibrosis

Retroperitoneal fibrosis is an unusual cause of obstructive uropathy in the pediatric population. The etiology is unknown although there are laboratory and clinical associations with various autoimmune diseases. Familial associations have not been reported in children. A family is reported where two siblings have idiopathic retroperitoneal fibrosis. In addition, these sisters and the father manifest clinical laboratory evidence for systemic immunologic diseases. These young girls represent the first patients to illustrate both autoimmune and familial characteristics of this disease. These cases support the concept that retroperitoneal fibrosis is a local manifestation of a systemic immune disease.     

Idiopathic retroperitoneal fibrosis

We treated a patient with idiopathic retroperitoneal fibrosis accompanied by right ureteral constriction. Pyelography, ureterography, and abdominal CT scan were pertinent diagnostics. Close collaboration between the surgeon and the urologist is required when attempting to treat such patients.     

Non-operative management of liver trauma

Aim-Background

Non-operative management (NOM) of liver trauma is well-established and accepted in current clinical practice. Initially considered as appropriate for lower grades of injury, NOM is now widely preferred for moderate and severe liver injuries too.

Methods

This review is based on a search of the English literature in Pubmed, using terms such as “liver trauma” and “non-operative”. Reconsideration of currently used indications and selection criteria for the non-operative management of liver trauma, as well as therapeutic strategies, complications and patient outcome, are discussed.

Results

Appropriate selection of patients according to the following criteria is essential for the successful outcome of NOM: haemodynamic stability (on arrival or after initial resuscitation), the absence of associated injuries to intra- or retro-peritoneal organs necessitating operative intervention, and the availability of a multidisciplinary team including an experienced radiologist, an experienced liver (preferably) surgeon and intensive care facilities. The volume of haemoperitoneum and the grade of injury are not considered factors that preclude NOM. Of paramount importance is the monitoring of signs of peritonitis, related to a possible unrecognised hollow viscus injury, which is appropriately managed operatively. Computed tomography is also important for early diagnosis of ongoing bleeding, in order to intervene either angiographically (embolization) or operatively (haemostasis). Mortality during NOM varies between 0.4% (liver-related) up to 13% (associated injuries).

Conclusions

In conclusion, the main selection criterion of patients with liver trauma for NOM is haemodynamic stability, independent of the grade of injury and the volume of haemoperitoneum. Accurate interpretation of liver CT scanning is of paramount importance, in order to exclude or diagnose any associated injuries requiring operative intervention or active bleeding that can be treated with angiography and selective embolization. The prospect of haemodynamic instability not improving with resuscitation, the increased need for liver-related blood transfusions (> 4 pRBCs), and failure of conservative treatment to deal with complications, necessitate prompt operative intervention to avoid the consequences of delayed management.