[Pseudo-obstruction and colonic ischemia]. / Pseudo-obstruction et ischémie colique.

We report the case of a 78 year old male schizophrenic with pseudo-obstruction of the colon complicated by patent-vessel ischaemia that was diagnosed after sub-total colectomy. Surgery was motivated by the chance discovery of a pneumo-peritoneum without peritonitis and without any evidence of perforation in the gastro-intestinal tract. We believe that early surgery is required in patients with pseudo-obstruction of the colon, to prevent complications such as ischaemia of the distended bowel wall which, though rare, is also well recognised.

[Diffuse digestive ganglioneuromatosis]. / Ganglioneuromatose digestive diffuse.

Discovery of intestinal ganglioneuromatosis implies the presence of the MEN IIb syndrome or, more rarely, von Recklinghausen's neurofibromatosis. The two conditions are due to dysfunction of the neural crest. This very rare intestinal pathology is illustrated by two observations. The intestinal motility disorders, due to the diffuse proliferation of the peripheral autonomous nervous system which ganglioneuromatosis represents, may have serious implications and indeed prove fatal. The etiologic role of excessive nerve growth factor production in these two entities is mentioned. The physiopathologic implications of an increase in vasoactive intestinal polypeptide, and of possible ectopic secretion of calcitonin by ganglioneuromatosis, are discussed.

[Medullary cancer of the thyroid gland]. / Les carcinomes médullaires de la thyroïde.

Thirty-one cases of medullary carcinoma of the thyroid have been studied over the past fifteen years at the University Hospital of the Canton of Vaud, Switzerland (CHUV). Twenty cases were of sporadic nature and eleven presented as part of the familial MEN II syndrome (multiple endocrine neoplasia), one of which showed the classical features of the rare MEN IIb type. It is important to distinguish between the familial and sporadic cases, because membership of the former group implies the investigation of associated endocrinopathies (pheochromocytoma, hyperparathyreoidism) and study of the family tree as the syndrome is autosomal dominant. Medullary carcinoma of the thyroid is a constant feature of the MEN II syndrome and is the cause of premature death in these patients. The familial type should be suspected if the carcinoma appears early in life, is located in the superior pole of the thyroid or is bilateral or multicentric, if the histology shows hyperplasia of the C cells and, of course, if there is a history of surgery for pheochromocytoma or hyperparathyroidism. Although total thyroidecomy is the rule for these familial cases, its role is debatable in sporadic medullary carcinoma of the thyroid. Postoperative follow-up of these patients is based on serum calcitonin determination, as this is an extremely sensitive marker. The ten year survival rate is 50%, with the worst prognosis in MEN IIb type.

[A rare variety of polyendocrine neoplasms: the MEN IIb and MEN III syndrome]. / Une variété rare de néoplasies polyendocriniennes: le syndrome MEN IIb ou MEN III.

The MEN IIb syndrome is a rare form of multiple endocrine neoplasia, clearly defined more than 15 years ago, since which time more than 100 cases have been identified. It has a characteristic phenotype appearance which is unique and should permit more frequent diagnosis. The patient who is the subject of this study had passed through several different hospital departments without his very characteristic appearance being recognized or a diagnosis established. The marfanoid aspect of such patient is however very obvious, with kyphosis, skeletal asymmetry and funnel chest. The facial appearance of blunt features and thick lips is even more specific. The tongue and digestive mucosa are covered with small polypoid lesions which are submucosal ganglioneuromas. The visible presence of fine hyperplastic nerve fibres on the cornea is pathognomonic. This syndrome occurs sporadically but is generally transmitted genetically in an autosomal dominant manner. A medullary thyroid carcinoma, followed or preceded by pheochromocytoma (often bilateral), is associated with these dysmorphic signs. The tumours are themselves preceded by C-cell hyperplasia or adrenal medullary hyperplasia. The ganglioneuromas which predominate in the digestive tract exist from birth. The accompanying symptoms may, initially, be mistaken for inflammatory bowel disease. The prognosis is poor, since it relates to the evolution of the medullary thyroid carcinoma which is particularly malignant and presents at an early age. We have been following the course of this unusual disease of the APUD system in a young man, now aged 26 years, for over 2 years.

Treatment of acute abscesses in the casualty department.

In the treatment of acute pyogenic soft-tissue abscess incision, curettage, and primary suture was compared with incision and drainage alone in a randomised prospective trial. Operations were performed under antibiotic cover by casualty officers, and patients were reviewed by an independent observer in a septic dressing clinic. Altogether 114 patients were studied, of whom 54 were treated by curettage and primary suture and 60 by simple drainage. The mean healing time was 8.9 days in those treated by primary suture and 7.8 days in those treated by simple drainage (p less than 0.05). Primary healing failed to occur in 19 (35%) of the sutured wounds, but there were no other complications in either group. It is concluded that incision and drainage alone is adequate treatment for acute soft-tissue abscess.

[Gastric lymphomas and pseudolymphomas]. / Lymphomes et pseudolymphomes gastriques.

We report 30 cases of non-Hodgkin's gastric lymphomas (according to the Rappaport classification): 1 nodular lymphocytic lymphoma well differentiated, 7 diffuse lymphocytic lymphomas poorly differentiated, 2 diffuse mixed cellularity lymphomas, 20 diffuse histiocytic lymphomas and 4 pseudolymphomas, over a period of 21 years at the CHUV, in Lausanne (1958-1979). There are 56% of advanced stages (IIIE and IVE) according to Ann Arbor. Survival to 5 years is of 27%. The mean survival of patients who died from their lymphomas is of 5 months only. Lymph node invasion worsens considerably the prognosis (75% of survival to 5 years for stage IE against 25% for stage IIE). This phenomenon is particular to non-ganglionary lymphomas. We do not observe good remission for the diffuse histiocytic forms at an advanced stage, remission being characteristic of the ganglionary lymphomas only. Treatment is poorly codified. Surgery along seems possible for the localised forms (IE): triple therapy (surgery, radiotherapy and chemotherapy) is necessary for advanced stages and histological unfavorable forms.