Biomedical subjects
Publications and source records attributed to M Prestipino.
An 8-month-old boy presenting with a fast-growing abdominal mass was operated upon to remove the tumor, which was confirmed to be a well-formed fetus in fetu. The authors describe the clinical, radiologic, and surgical findings and review the literature.
This study includes 117 patients operated upon in the period from 1970 to 1999. Indications, surgical techniques, complications, and results are reviewed. Indications included: long-gap oesophageal atresia with or without fistula in 81 patients; peptic stenosis in 19; caustic stenosis in 12; oesophageal varices in 2; and 1 case each of oesophageal epidermolysis bullosa, total oesophageal leiomyomatosis, and a non-functioning antiperistaltic retrosternal colic graft operated upon in another hospital. A retrosternal bypass was performed 106 times: 98 first operations and 8 redos; the intrathoracic technique was used 19 times. The left transverse colon was used in 107 cases (85.6%), the right transverse colon in 8 (6.4%), and the ileocecum in 10 (8%). All the intestinal bypasses were placed in the isoperistaltic direction. There were 5 deaths in the first 11 years of our experience; no patient died from 1982 on. Ten complications were treated conservatively (8%): 2 wound infections healed with medical treatment, and 8 leaks of the cervical anastomosis closed spontaneously. The major surgical complications were 8 gangrenous bypasses (6.4%), removed and reoperated about 1 year later utilizing an ileocolic retrosternal graft. Three cases of peptic disease of the colic bypass (2.4%) were successfully treated with the author's technique. Nine patients had minor surgical complications (7.2%): 3 strictures of the oesophagocolic anastomosis in a retrosternal bypass (resected and reoperated) and 6 cases of adhesive occlusion. In our opinion, the best substitute of the oesophagus is the colon, particularly the left transverse segment, which may be placed behind the sternum or in the oesophageal bed, always in the isoperistaltic direction. The low mortality (4%), restricted to the early period of our experience, and few major surgical complications (6.4%) are acceptable considering the importance of the operation, and the long-term results may be considered very satisfactory.
OBJECTIVE: To report our experience of the use of desmopressin to improve nocturnal dryness in patients who have undergone a staged reconstruction of the exstrophy-epispadias complex (EEC), who although continent by day, have nocturnal incontinence because their nocturnal urinary output exceeds their bladder capacity. PATIENTS AND METHODS: Seven children (aged 8-12 years) who had undergone a staged reconstruction for EEC (six with classical bladder exstrophy, one with incontinent epispadias) were treated with intranasal desmopressin for persistent nocturnal incontinence despite daytime dryness. Previous additional procedures for continence had been self-augmentation in one and periurethral collagen injection in three others. The criteria for inclusion in the study were: normal renal function, no upper tract deterioration, no urinary tract infections, spontaneous voiding during the day with dry intervals between micturitions, a postvoid residual volume of <10% of bladder capacity and night-time incontinence for 7 nights/week. Desmopressin was administered at bedtime at increasing dosages from 10 to 30 microg until effective. Body weight, arterial blood pressure, and serum electrolytes were measured, and all patients assessed using renal ultrasonography, a voiding diary and a nocturnal pad-test. RESULTS: Desmopressin at doses of 10-30 microg was successful in keeping all the patients dry. The nocturnal urinary output was decreased so that it did not exceed bladder capacity. There was only one minor side-effect (nose bleeding). CONCLUSIONS: In selected patients with EEC, desmopressin is effective in improving nocturnal dryness, with no significant side-effects.
The aim of this paper is to describe and discuss, on the basis of the available literature, the case of an old female patient, admitted to our university hospital because of a severe dysphagia for solid foods, in whom laboratory data showed a marked hypomagnesemia. She reported a long history (20 years) of allergic bronchial asthma treated with theophylline. Esophagography evidenced a disorder of esophagus motility with diffuse multiple spasm, reminiscent of the 'corkscrew esophagus'. A link with the severe hypomagnesemia (Mg 1.1 mEq/l, normal range 1.6-2.1) was suspected, and a therapy with oral pidolate of Mg (1.5 g/twice a day) was started and continued for 4 months. This was associated with a slow progressive normalization of the Mg plasma level and reverted radiographic esophageal findings with disappearance of dysphagia. Mg is an important element for health and disease, and today Mg deficiency in man has become an accepted medical problem which might complicate many diseases. Neuromuscular disorders, as laryngeal spasm, are recognized complications of hypomagnesemia, but until now only 1 case of motor esophageal disorder associated with a low Mg plasma level was briefly reported in the literature, even if dysphagia is generally included in the symptomatological pattern of hypomagnesemia. Our observation of a severe form of esophageal spasm, associated with hypomagnesemia, in an aged female patient underlines the pathophysiological meaning of the plasma Mg level and suggests the need for routine Mg determination in the clinical setting.
FVII deficiency is a rather rare inherited hemocoagulation disorder that predisposes to hemorrhagic events, especially from mucous membranes, that are not predictable and severe as in hemophilia A. This defect produces prolonged prothrombin time (PT), reduced activity of FVII and normal activated partial thromboplastin time (aPTT). We report the case of a 43-year-old obese woman with severe deficiency of factor VII (FVII), probably genetic in nature, and meno-metrorrhagia associated with multiple fibromas of uterus. Our patient had no history of bleeding in infancy and young age, and in the past, before the disease was diagnosed, underwent major surgery operations (thyroidectomy and caesarian section) without hemorrhage. Patient's relatives with mild heterozygous deficiency of FVII (the father, a brother, a sister, a sister's daughter and the patient's son) did not show any bleeding tendency. This case report is discussed in the light of literature data ((source: Medline from 1964 to 1996). The different forms of congenital (isolated or combined with other clotting disorders) and acquired FVII deficiency, with the appropriate therapies, are reviewed. The clinician must consider FVII deficiency in cases of recurrent bleeding, and this disease, even if rather rare, should not be underestimated in clinical practice because it is potentially fatal.
The Authors report their experience in the surgical management of three adults affected by intussusception. The different symptoms and different diagnostic approach, compared to those of childhood, induced the Authors to some evaluations on pathophysiology of intussusception in adults. On the basis of the different pathogenesis some differential criteria between the two forms are stressed, finally suggesting a systematic surgical approach in adults.
Aim of this paper is to describe and discuss, on the basis of the available literature, the case of a young woman, previously colectomized for diffuse lipomatosis of the colon, showing hypomagnesemia and symptomatic (precordial discomfort) changes of repolarization phase, detected by ECG, probably due to coronary spasm. This hypomagnesemia (1.4 mEq/1) was probably due to altered intestinal absorption of magnesium, linked to a short bowel syndrome. The ECG changes and the precordial symptom were completely reversed by a relative short treatment with magnesium per os, which increased the magnesium level to low borderline value (1.6 mEq/1). The observation of ECG changes with precordial discomfort, probably linked to hypomagnesemia, suggests the need for routinary magnesium determinations to detect deficiency of this electrolyte, with the scope of improving the diagnosis and the treatment of several symptoms, otherwise difficult to interpret.
Aim of this paper is to describe and discuss, on the basis of an exhaustive review of literature, the case of a 14-year-old girl with familiar Marfan's syndrome. This disease is a generalized inherited disorder with involvement of connective tissue and symptoms affecting ocular, skeletal and cardio-vascular systems, usually diagnosed in young age and associated with a poor prognosis because of late severe aortic complications (dissection, dilation or aneurysms, regurgitation, etc.). The young patient sought to our medical attention because of severe leanness, delayed menarca and irregular menstruations; physical examination disclosed the typical "morphotype of Marfan" with long limbs, slenderness of hands and feet, severe kyphoscoliosis, narrow chest with "pectus excavatum", marked hyperextensible joints, and high arched palate with malocclusion. Echocardiography demonstrated a mild mitral valve prolapse. The 43-year-old patient's mother presented an undiagnosed Marfan's syndrome, despite typical morphotype, muscle-skeletal alterations and moderate dilation of ascending aorta. The maternal grand-mother deceased prematurely for cardiopathy of unknown origin. It is stressed that the disease should not be underestimated, because the early diagnosis is important for the patient's prognosis, allowing the early preventive surgical intervention for correcting aortic or valvular alterations. The echocardiography represents a sensitive and noninvasive mean, useful to manage the patients with proven or suspected aortic dilation. The subjects with Marfan's syndrome who exhibit rapid progression of aortic dilation, or an aortic diameter in excess of 50 mm, should be considered for an elective surgical intervention (at low mortality and suitable to increase remarkably the lifetime expectation). Women with syndrome of Marfan should be advised on the high cardio-vascular risk during pregnancy.
The Authors review biliary peritonitis. After some evaluations on etiopathogenesis, biliary peritonitis without perforation in childhood is discussed. The rarity of this form is considered as well as etiopathological hypothesis, difficulties of diagnosis and surgical management. Two cases of primary biliary peritonitis without perforation observed and operated in two children are reported.
Diffuse and symptomatic lipomatosis of the colon, particularly when associated with hyperplasia of the appendices epiploicae, is a rare disease. We report here the case of a lean, 26-year-old woman who had polypoid lipomatosis of the colon with submucosal and subserous expression (involving the mesentery and peritoneum) associated with extreme hyperplasia of the appendices epiploicae. Her disease led to a recurrent subocclusive syndrome which required total colectomy. We discuss the case and review the literature on this subject. The particular characteristics of this case and early onset of disease would suggest that our patient was affected by a congenital lipomatous syndrome linked to mesenchymal dysplasia. Long-term monitoring will enable prompt intervention in the case of reformation of lipomatous tissue.
The Authors report their experience with 738 children observed and operated for minor diseases in the period January 1989-June 1993. The children were hospitalized for day surgery or one day surgery. After some surgical, anaesthesiological, and postoperative pathophysiological considerations, and on the basis of their results (reduced local and general postoperative complications), the Authors confirm the benefit of this type of organization in terms of reduced nosocomial infections, reduced psychological traumas and increased socio-economic advantages.
We report the case of a 22-year-old obese woman with severe protein S deficiency, probably genetic in nature, associated with recurrent venous thrombosis. Protein S deficiency is a rather rare disease: it may be an inherited, either homozygous (purpura fulminans at neonatal age), heterozygous, or acquired disorder. The thrombophilic state may be manifested as deep vein thrombosis or thrombophlebitis of the superficial veins with a high risk of pulmonary embolism in the young, and it is often exacerbated by pregnancy. In our case, the presenting event, bilateral deep venous (iliac-femoral) thrombosis complicated by disseminated intravascular coagulation, had occurred when the patient was 13 years old. We started long-term therapy with oral coagulants, i.e. warfarin even if the latter may cause skin necrosis ("warfarin dermatitis") in some patients with protein S deficiency. The clinician must consider protein S deficiency in cases of recurrent thrombosis, particularly in young patients: the importance of early implementation of long-term preventive therapy should not be underestimated.
The operative management of patients with hyperparathyroidism is controversial. High rates of persistent hypercalcemia and postoperative hypoparathyroidism are seen in multiple hyperplasia and bilateral neck exploration. Patients undergoing unilateral neck exploration with removal of a single parathyroid adenoma have a rapid clearance of PTH which declines within hours after surgery. There is a sensitive immunometric assay (IRMA) for the intact molecule which demonstrates a decline in 15 minutes during surgery. Intraoperative measurement of PTH may be complementary to surgical skill and histopathologic information and may modulate extension of neck exploration.
Intussusception in childhood, especially the idiopathic type, is herein considered. The Authors evaluate some etiopathological aspects on the basis of their experience in 34 patients, and affirm the need of an early and correct diagnosis. They also consider therapeutical management and surgical approach, evaluating local and general conditions of the patient as well as time passed since the onset of symptoms. Finally the importance of an accurate diagnostical examination during the quiescent period and some details of the surgical technique are stressed.
The Authors report their experience in the management of surgical hyperthyroidism, evaluating the different clinical pictures and the possibility of non surgical treatment. Advantages and disadvantages of both hemithyroidectomy and total thyroidectomy, are also described. The Authors conclude affirming their preference for techniques such as total hemithyroidectomy or thyroidectomy in some types of hyperthyroidism.