Hyperplasia of the mandibular coronoid process: an analysis of 31 cases and a review of the literature.

PURPOSE: This study was undertaken to investigate the demographic and clinical features of mandibular coronoid hyperplasia and to assess the response to current treatments. PATIENTS AND METHOD: Case notes were studied and records made of age, sex, duration of symptoms, operative procedures, and response to treatment in all patients presenting at the Queen Victoria Hospital, East Grinstead, over a 20-year period from 1970 to 1990 with a confirmed diagnosis of mandibular coronoid hyperplasia. RESULTS: Thirty-one cases were recorded, 23 bilateral and 8 unilateral. The average age on presentation was 27.8 years for bilateral and 23.6 years for unilateral cases, with symptoms predating presentation by an average of 9 years and 6.75 years, respectively. Surgery was disappointing in terms of improving mouth opening. CONCLUSION: The results of this large series are supported by meta-analysis of the previous literature. They suggest that surgical management may be improved by a greater understanding of the pathogenesis of this condition.

Pre-operative percutaneous transhepatic biliary drainage: the results of a controlled trial.

The operative mortality for biliary tract obstruction due to malignancy is high. In 1981 a controlled clinical trial of pre-operative percutaneous drainage was started at the Royal Postgraduate Medical School. At the time of percutaneous transhepatic cholangiography patients were randomized either to laparotomy or to pre-operative percutaneous transhepatic biliary drainage ( PTBD ) followed by laparotomy. Only patients with malignant biliary tract obstruction and serum bilirubin greater than 100 mumol/l were included. Seventy patients entered the trial, and five were withdrawn. Of the 65 remaining, 31 underwent laparotomy and 34 had pre-operative PTBD followed by laparotomy. The median duration of drainage was 18 days and during this time the median bilirubin fell from 305 to 115 mumol/l. Five patients required early surgery for complications of PTBD and two died within 30 days of surgery. The mortality for laparotomy was 19 per cent (6/31) compared with 32 per cent (11/34) for drainage plus laparotomy. This trial highlights the hazards of PTBD in high risk patients and has failed to demonstrate a reduction in mortality with the use of pre-operative PTBD .

Localized amyloidosis of the ureter.

We report a case of localized ureteral amyloidosis. This condition usually presents as hematuria and a ureteral stricture, and is clinically indistinguishable from a neoplasm. Treatment should consist of local resection if possible.

Idiopathic localized bile duct strictures: relationship to primary sclerosing cholangitis.

Two patients have been described who presented with localized hilar bile duct strictures initially diagnosed as cholangiocarcinoma. Resection of the strictures showed benign disease. The subsequent development of further independent benign strictures was consistent with sclerosing cholangitis. Problems in diagnosis and management of this form of primary sclerosing cholangitis have been discussed.

CT abnormalities of the pituitary in hyperprolactinaemic women with normal or equivocal sellae radiologically.

Sixteen young women with hyperprolactinaemia and normal or equivocal sella in radiographs underwent computed tomography using a Siemens Somatom II. In all but one case an abnormality was found. The sella was full in seven and partially empty in nine. A tumour was visible in six of the full and in four of the partially empty sellae. All but one of the 10 tumours was unilateral, and in seven the pituitary stalk was deviated away from the tumour. After administration of intravenous contrast (Urografin) four tumours showed diffuse enhancement, four ring enhancement, and two enhanced less than adjacent normal pituitary tissue. Two of the tumours have been subsequently shown histologically to be prolactinomas. Prolactin response to thyrotrophin-releasing hormone predicted a tumour in seven out of eight with visible tumours but also in three out of four without visible tumours; using metoclopramide, a tumour was predicted in six out of seven with tumours, but again in three out of four without visible tumours. Such results question the value of dynamic tests for the discrimination of tumours. We conclude that practically all women with sustained hyperprolactinaemia and a normal or equivocal sella radiologically have pituitary disease.

Adult-onset Still's disease. Twenty-year followup and further studies of patients with active disease.

Eleven female patients with adult-onset Still's disease were followed for 7-36 years (mean 20.2 years) after the onset of their illness. Ten of these patients had a chronic course characterized by remissions and exacerbations of arthritis associated with fever and rash. Five patients had terminal interphalangeal involvement, and carpal ankylosis was demonstrated on x-ray film in 10. Two patients developed a widespread polyarthritis, and renal amyloidosis was diagnosed 10 years after disease onset in the most severely affected patient. In 4 patients studied during an exacerbation of the disease, circulating immune complexes were detected by the staphylococcal A binding assay, but not by the C1q binding assay. Synovial fluid analysis in 1 patient revealed a low C3 level and total hemolytic complement (CH50) together with immune complexes and IgG rheumatoid factor. Immune complexes were not identified in the characteristic Still's rash by immunofluorescence or electron microscopy, although mast cell degranulation, neutrophil lysis, and perivascular fibrin deposition were reminiscent of immune complex--mediated vascular injury. The clinical and laboratory features as well as the long-term course of adult- and juvenile-onset systemic Still's disease are similar, but further studies of genetic markers and immunopathology are required to establish a common pathophysiology.

Percutaneous transhepatic drainage in obstructive jaundice: advantages and problems.

This study is a critical prospective assessment of 37 patients with obstructive jaundice, treated by percutaneous transhepatic biliary drainage. The median duration of drainage was 18 days (range 44-55), and during this period clearance of bilirubin and improvement in creatinine clearance were obtained. Only 10 patients gained weight. Three patients required early laparotomy. Thirty-three patients underwent definitive surgery. Of these, 8 died without leaving hospital. The incidence of infection rose during drainage, and infected bile was clinically significant. Two deaths were associated with infection, arising in the drainage system, producing intrahepatic abscesses around the drain track. While the evidence for a staged approach in the severely ill patient with obstructive jaundice is substantial, the procedure of percutaneous transhepatic tubal drainage carries significant hazards, underemphasized in previous reports. Further controlled assessment is required before this technique is accepted as the initial best option for decompression of the obstructed biliary tract.

Congenital hypertension due to unilateral renal vein thrombosis.

A boy was found on the day of birth to have hypertension and radiological evidence of calcification of the left renal vein. Persistent hypokalaemia and hyper-reninaemia in the presence of a small left kidney and normal right kidney led to the decision to perform a left nephrectomy. The biopsied specimen showed old calcified renal vein thrombosis with accompanying medullary necrosis. Postoperatively hypertension resolved.

The chest X-ray in pulmonary capillary haemorrhage: correlation with carbon monoxide uptake.

Serial changes in carbon monoxide uptake (KCO or DLCO/VA) were used to monitor episodes of pulmonary capillary haemorrhage in Goodpasture's syndrome (13 cases), immune complex nephritis (three cases) and idiopathic haemosiderosis (one case). Air-space shadowing on the chest X-ray (on a scoring system 0 to 12) was assessed in the light of the changes of KCO. In 14 out of 27 episodes of bleeding, the rise and fall of KCO was matched in time by the appearance and disappearance of air-space shadowing on the chest X-ray. In six episodes the chest X-ray remained normal despite a rise of KCO. In two cases air-space shadowing appeared up to 48 h after the rise of KCO. On five occasions chest X-ray abnormalities preceded the rise of KCO but chest infection or fluid overload accounted for three of these. In cases with suspected pulmonary capillary haemorrhage, measurements of carbon monoxide uptake will provide additional information and will assist in the interpretation of the chest X-ray.

The chest X-ray in antiglomerular basement membrane antibody disease (Goodpasture's syndrome).

The chest radiographs of 25 patients with proven antiglomerular basement membrane antibody disease (Goodpasture's syndrome) were analysed. All except two of the patients had pulmonary haemorrhage at some stage of their disease. Altogether there were 39 episodes of pulmonary haemorrhage, 25 being relapses. During seven episodes the chest radiograph was normal. Relapses of pulmonary haemorrhage never occurred in isolation but were usually associated with infection (not necessarily a chest infection) or occasionally fluid overload. Conversely fluid overload or infection were always associated with pulmonary haemorrhage provided there were high or rising titres of circulating antibodies at the time. Therefore in a patient with antiglomerular basement membrane antibody disease, the presence of shadowing in the lung fields on the chest radiograph almost invariably means the patient has pulmonary haemorrhage whether or not pulmonary oedema or a chest infection are present. Limitation of shadowing by a fissure, loss of major portions of the diaphragmatic or cardiac silhouette, involvement of the lung apex or costophrenic angles suggest an underlying chest infection. Septal lines suggest fluid overload. Pleural effusions are seen with chest infections and fluid overload. The carbon monoxide uptake (KCO) was invariably high in the presence of pulmonary haemorrhage even if the chest radiograph was normal. A combined use of KCO and chest radiographs is the best method of monitoring lung disease in these patients.