Pulmonary hypertension associated with portal hypertension. Apropos of 2 cases

The authors report the cases of two patients with pulmonary hypertension associated with portal hypertension. This is a rare association with a reported prevalence ranging from 0.25 to 0.73%. The diagnosis of portal hypertension preceded that of pulmonary hypertension by several years. The physiopathological mechanism of the latter is not well known although several hypotheses have been proposed. Treatment is only symptomatic. The prognosis is usually poor, the causes of death being related to complications of liver failure and/or portal hypertension or to those of pulmonary hypertension.     

Treatment of portal hypertension

Abnormalities of carbohydrate metabolism in hyperthyroid patients have been long noted. Elevated proinsulin level is considered as an early marker of B-cell impairment. Proinsulin levels in hyperthyroid patients decreased after antithyroid drug therapy. However, proinsulin in hypothyroid patients was only rarely reported, and the difference was only demonstrated after glucose stimulation-there was a greater response of proinsulin secretion after thyroxine therapy-and the basal fasting proinsulin level was not different after therapy. One of the reasons might be that the assay was not sensitive enough to detect the change of basal proinsulin levels in patients with hypothyroidism after therapy. A newly developed immunochemiluminometric assay of proinsulin was used to demonstrate that the suppressed proinsulin level increased after thyroxine therapy in hypothyroid patients (4.2 +/- 2.4 vs. 10.0 +/- 5.6 pmol/L, p < 0.05; n = 7). On the other hand, our study also confirmed that the proinsulin levels decreased in hyperthyroid patients after antithyroid therapy by methimazole (27.8 +/- 26.0 vs. 15.8 +/- 15.7 pmol/L, p < 0.05; n = 12). In conclusion, proinsulin increased in hypothyroid patients after thyroxine therapy and decreased in hyperthyroid patients after methimazole therapy. The results demonstrated there is a high correlation between thyroid function and B-cell function in hypothyroid as well as hyperthyroid patients.     

Occipital encephalocele associated with mediastinal enteric duplication cyst

We report a new case of tracheobronchopathia osteoplastica revealed by bronchopulmonary infections. The review of the literature enables us to summarize the clinical, endoscopic, morphological features, as well as pathogenic hypotheses.     

Isolated mediastinal mass in primary amyloidosis

A previously healthy man with lytic bone lesion of the left talus was found to have a large middle mediastinal mass on routine admission chest roentgenogram. A large amyloid tumor of the mediastinum, representing the sole intrathoracic manifestation of primary amyloidosis, was resected. This presentation of intrathoracic amyloidosis has not previously been reported.     

Surgical management of portal hypertension

The surgical management of portal hypertension depends on the location of the obstruction. Suprahepatic obstruction is usually optimally treated by a surgical portacaval shunt. In extrahepatic obstruction the treatment should be sclerotherapy. For intrahepatic obstruction in emergency situations, sclerotherapy is the first choice, with portacaval systemic shunts or transjugular intrahepatic portal systemic stent shunt the second option. Liver transplantation in other situations should, if possible, be considered ahead of a portal diversion.     

Transposition of the spleen in rats with portal hypertension

Portal hypertension was induced in rats by progressive occlusion of the portal vein or by dimethylnitrosamine (DMNA) cirrhosis. Portasystemic venous connections did not develop in relation to the spleen when this organ was intraperitoneal, but after subcutaneous transposition most of the collaterals were parasplenic and the portal venous pressure fell.     

Oral ammonia tolerance test in patients with portal hypertension

Eighty patients with portal hypertension due to various hepatic diseases were subjected to the oral ammonia tolerance test (OATT). Blood samples were collected before and at 30-min intervals after the administration of ammonium chloride (50 mg/kg). The ammonia levels, and the OATT curve patterns and sigma NH3-N values were determined and it was investigated whether there are correlations between these values and various other parameters of hepatic function. The results of OATT correlated with the plasma disappearance rate of indocyanine green and the molar ratio of branched chain amino acids to aromatic amino acids. Our findings suggest that the OATT may reflect the degree of hepatic parenchymal disorders rather than the hepatic circulatory abnormalities and that they may represent a valuable aid in the differential diagnosis of hepatic diseases and in determining the operative indication.     

Anatomo-clinical conference: lymphoma and mediastinal mass

We analysed retrospectively 460 patients treated between March 1991 and February 1992, in respect to a correct perioperative antibiotic prophylaxis. In our opinion a correct prophylaxis is a single-shot application, with a second dose given only during long operations (over 3 hours) or when blood loss is great. 240 of the 460 patients analysed (52.2%) were given antibiotics prophylactically. In 76.6% of the cases (n = 184) the prophylaxis was performed correctly. In 23.4% (n = 56) antibiotic application was extended indiscriminately into the post-operative phase. This calculated to costs three times higher than those actually necessary, causing us to spend 22,000-Swiss francs unnecessarily, one twelfth of the total cost of antibiotic treatment in our hospital each year. For economic reasons as well as to keep growth of resistant strains in check, an antibiotic prophylaxis should be restricted to the perioperative period in most cases as a single shot only.     

Baller-Gerold syndrome associated with congenital portal venous malformation

This article describes a case of corneal marginal ulcer caused by relapsing polychondritis (RPC) that was treated with keratoepithelioplasty. The patient underwent keratoepithelioplasty with lamellar keratoplasty in the left eye. No relapse occurred in the nasal side in which corneal lenticules were grafted, whereas vascular invasion developed in the temporal side in which no lenticules were grafted. Keratoepithelioplasty was an effective surgical procedure to prevent a recurrence of corneal marginal ulcer in a patient with RPC.     

Recanalized umbilical vein in portal hypertension

Experience with splenoportography suggests that patency of the umbilical vein occurs in about 9% of the patients with portal hypertension. A widely patent umbilical vein might serve as a decompressive portosystemic shunt. Percutaneous transhepatic portography was performed in 107 patients with cirrhosis of the liver and portal hypertension. A patent umbilical vein was found in 28 patients (26%). This finding significantly paralleled the number and size of other collateral veins, apart from gastroesophageal varices. No significant relation was found between umbilical vein patency and portal pressure, extrahepatic shunting, variceal bleeding, or ascites. It is concluded that a large patent umbilical vein does not effectively relieve portal hypertension, prevent gastroesophageal varices, or protect against variceal bleeding or ascites.     

Pulmonary vascular disorders in portal hypertension

The wide spectrum of pulmonary vascular disorders in liver disease and portal hypertension ranges from the hepatopulmonary syndrome characterized by intrapulmonary vascular dilatations, to pulmonary hypertension (portopulmonary hypertension), in which pulmonary vascular resistance is elevated. Since hepatopulmonary syndrome and portopulmonary hypertension have been reported in patients with nonhepatic portal hypertension, the common factor that determines their development must be portal hypertension. The clinical presentations are very different, with gas exchange impairment in the hepatopulmonary syndrome and haemodynamic failure in portopulmonary hypertension. The severity of hepatopulmonary syndrome seems to parallel the severity of liver failure, whereas no simple relationship has been identified between hepatic impairment and the severity of portopulmonary hypertension. Resolution of hepatopulmonary syndrome is common after liver transplantation, which has an uncertain effect in portopulmonary hypertension. The pathophysiology of both syndromes may involve vasoactive mediators and angiogenic factors.