Biomedical subjects
Publications and source records attributed to F Helmer.
This is a report of a prenatal diagnosis and successful intrauterine drainage of a pulmonary cyst by placement of a thoracoamniotic shunt at 36 weeks gestation. At the time of delivery the newborn had no signs of pulmonary hypoplasia or respiratory insufficiency.
A case of scimitar syndrome with pulmonary sequestration is reported. Anomalous pulmonary venous return from the right lung to the infradiaphragmatic vena cava inferior was diagnosed by pulmonary angiogram and sequestration of the right lower lobe was confirmed by aortogram. Venous return from the sequestrated lung was partly into the vena cava inferior and partly into the left atrium. Successful repair was achieved by resection of the sequestrated lobe and direct reimplantation of the scimitar vein into the left atrium. Accurate preoperative diagnosis and intraoperative evaluation of the anatomy is mandatory to correct this rare anomaly.
31 children with intraperitoneal masses were operated upon during a 10 year period between 1975 to 1985, 15 tumors were malignant (3 hepatoblastomas, 6 NHL, 3 RMS, 1 mesothelioma, 1 teratocarcinoma of the ovary, 1 haemangioendothelioma of the liver). 16 benign lesions included 3 solitary liver cysts, 2 haemangiomas of the liver, 2 parasitic cysts, 3 ovarian cysts, 2 choledochal cysts, 2 intraperitoneal encapsulations, 1 mesenteric cyst, 1 duplication of the intestine. Half of the benign masses were found in the first month of life, two thirds (69%) under 1 year of age, later the malignant tumors are more frequent.
The case report is presented of a newborn that developed pseudomembranous colitis and intestinal obstruction after antibiotic therapy with cefoxitin and gentamicin in the first week of life. At laparotomy on the fifth day a complete obliteration of the descending colon by pseudomembranes was encountered. After resection of the diseased segment the recovery was uneventful. We believe this case is the first to be published of antibiotic-associated pseudomembranous colitis causing intestinal obstruction.
Two infants were admitted for severe, intractable hypoglycemia. "Fasting" tests lasting 45 and 30 minutes respectively revealed hypoglycemia with inappropriately elevated levels of plasma insulin and plasma C-peptide. Subsequently, somatostatin was infused to test the individual sensitivity of the B-cells. Basal and glucose-induced insulin and C-peptide secretion were significantly reduced. Preoperative treatment with somatostatin was introduced, which controlled the hypoglycemia. In both patients, subtotal pancreatectomy was effective in restoring normal glucose homeostasis.
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After a brief comment on the origin and a summing up of types of postoperative cardiac arrhythmias in open heart surgery, mainly the therapy of these arrhythmias is discussed Besides drug therapy (antiarrhythmica) and cardioversion resp. pacing especially the possibility of a metabolic therapy is presented. In the postoperative phase after the trauma by the operation with the heart lung machine - which is similar to the shock phase after a myocardial infarction - a disordered energy metabolism with a decreased effectiveness of insulin occurs. It exists a diminished intracellular potassium-shifting, which causes an insufficient myocardial cell membrane potential. By means of exogenous high insulin and potassium supply with glucose infusion the potassium-shifting is improved, the FFA-level is lowered and the incidence of arrhythmias is reduced as sinusrhythm is induced.
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Standard treatment of infants with "wide gap" esophageal atresia usually requires a staged procedure consisting of gastrostomy at birth (with or without cervical esophagostomy) and then a bowel interposition between the oesophagus and stomach at 1 year of age. Because this approach procedures significant morbidity and swallowing dysfunction, an alternative method for treating the wide gap atresia is recommended. The upper and lower esophageal pouch can be elongated by preoperative bougienage. This technique allows successful primary esophageal reconstruction in infants with wide gap esophageal atresia previously considered uncorrectable except by use of colonic or gastric tube interpositions.
Uniform treatment based on the therapeutic approach of the 1st and 2nd US National Wilms' Tumor Study was decided on in March 1976 by paediatricians, surgeons, urologists and radiotherapists in Austria. Wilms' tumour was diagnosed in 34 children between 1 january 1976 an 29 february 1980 (stage I: n = 11, stage II: n = 8, stage III: n = 8, stage IV: n = 7). Parents of two children refused treatments; both children have since died of metastases. Of the remaining 32 children 29 (90.6%) are alive, 10 for more than 4, 15 for more than 3 and 19 for more than 2 years after diagnosis. 21 children are without need of treatment. Three children have died, one due to postoperative complications, one due to haemorrhagic chickenpox, but free of tumour, and one after insufficient treatment. Two of the five children with a recurrence between 2 1/4 to 15 months after diagnosis had been treated inadequately in the initial phase. The tumour free survival rate in 74.2%. Two children with early occurring or recurrent lung metastases have survived for 53 1/2 and 54 months up to now.
A report is given on the results of different forms of mitral valve operations in 866 patients. Closed mitral valvulotomy was performed in 519 patients and mitral valve replacement in 291 cases. Open mitral valve anuloplasty was performed as reconstructive surgical procedure in 15 patients. The overall mortality was 10.4%. Follow-up of the surviving patients showed that the long-term results of mitral valve replacement are significantly worse than those of mitral valve reconstruction.
A report is presented of the occurrence of peripheral pulmonary stenosis due to tumour compression in a 12 year-old boy. The tumour was identified as a liposarcoma of the mediastinum, which caused complete occlusion of the left pulmonary artery and stenosis of the right pulmonary artery. This was followed by an extreme increase in prestenotic pressure with right heart failure.
31 infants with anterior abdominal wall defects were treated over the past 10 years. Despite the falling birth rate gastroschisis occurred almost twice as often as omphalocele (19 and 12 cases, respectively). The mortality rate of 71% in omphalocele were related to large defects, rupture of the sac and associated cardiac malformations. The 73% incidence of prematurity, additional malformations and sepsis contributed to the mortality rate of 58% in gastroschisis.
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A female patient with a heart murmur typical for mitral insufficiency that was first heard, when she was 17 months old, is reported. In the ECG flattened T-waves in the left praecordial leads are seen. Because of a typical x-ray seven years later the diagnosis of leftventricular aneurysm was suspected, guaranteed by angiocardiography and because of repeated supraventricular tachycardias the congenital aneurysm was operated. A differentiation between congenital diverticulum and congenital aneurysm is established, the clinical symptoms of this malformation and the therapeutic consequences are discussed.
Two rarely-observed tumours of the liver are discussed: firstly, primary liver carcinoma in a 5-month-old infant, and secondly, myxoma in a newborn infant. Both tumours were removed surgically. The child with primary liver cell carcinoma was treated for 2 1/2 years with cyclophosphamide. She has been followed-up for 8 years and can be assumed to be cured. Both children developed normally. The pathogenesis of liver tumours at this early age is discussed.
Cholelithiasis is a rare, but important condition to be considered in the differential diagnosis of abdominal symptoms in childhood and adolescence. A survey over a 20-year period revealed 10 cases between the ages of 11 and 20 years, with a clinical history of cholelithiasis of between 5 days and 6 years. The most consistent finding was epigastric or right upper quadrant pain. Serum chemistry did not contribute conclusively towards the diagnosis. Hereditary spherocytosis was demonstrated in 3 cases, 2 had familial gall bladder disease, whilst in the rest no cause could be found. Two patients are described in some detail: one girl with spherocytosis and a bilirubin of 78 mg/100 ml, presenting with choledochal obstruction and pancreatitis, and another girl with a history of recurrent abdominal pain, negative radiologial and chemical pathological findings and a tentative diagnosis of neurosis. Oral cholecystograms lead to the diagnosis in most cases; however i. v. cholangiograms should be considered whenever the diagnosis appears in doubt. All cases were treated by cholecystectomy and recovery was uneventful in 9 patients. Cholecystectomy should be performed in all patients with gall stones, even in the absence of symptoms.
Surgical intervention in organic hyperinsulinism is based on prior accurate biochemical assessment. The emergent problems are discussed in a case with hypoglycaemic attacks, where "blind" pancreatic resections had been performed in two occasions without success. Finally, after demonstration of hyperinsulinaemia and localization of the tumour by selective angiography an adenoma was removed surgically. The postoperative complications, including the development of insulin-dependent diabetes, demonstrate the inherent risks of blind distal pancreatectomy for suspected insulinoma.