Congenital hemangiopericytoma: report of a case.
A congenital hemangiopericytoma of the lower lip, first detected by prenatal ultrasound, was only partially resected and involuted over the next 20 months.
Biomedical subjects
Publications and source records attributed to N M Bax.
A congenital hemangiopericytoma of the lower lip, first detected by prenatal ultrasound, was only partially resected and involuted over the next 20 months.
Laryngotracheo-oesophageal cleft has been considered a rare congenital anomaly, diagnosed when there is respiratory distress on feeding, associated with multiple congenital disorders. A tracheo-oesophageal fistula should be excluded. Diagnosis, even in skillful endoscopic hands, is difficult. Different approaches to surgical repair have been advocated in literature. Most authors prefer the lateral approach. Only 7 patients have reportedly been treated via the anterior approach. The anterior approach offers the advantage of excellent and also easy exposure of the anomaly. The theoretical disadvantage of postoperative laryngeal instability is not substantiated by experimental work (Calcaterra, Ann Otol 83:810-813, 1974), nor by our own clinical experience in six juvenile patients who underwent laryngofissure for other reasons. A further advantage of the anterior approach is that the laryngeal nerves are not jeopardized as in the lateral approach. We have treated 4 patients. Two patients have been treated successfully via a lateral pharyngotomy approach; each of them has a left recurrent nerve palsy. The other two patients were treated via a laryngofissure, one of them successfully; in the other patient the cleft has been closed with success, but the larynx is still stenosed by excessive mucous membrane folds. Cicatricial subglottic stenosis, which may occur postoperatively, and the frequently coinciding tracheomalacia, have been successfully treated with prolonged nasotracheal intubation with siliconized silicone rubber tubes. Four patients were treated successfully. Three other patients have come to our attention. As is often the case, this disorder seems to occur more frequently than was previously thought.
The antenatal ultrasonographic findings in a fetus with a left-sided congenital posterolateral diaphragmatic defect (CPLDD) are presented and discussed. Displacement of the heart towards the right and absence of a normally positioned fluid-filled stomach at repeated examinations are diagnostic. In the event of an intrathoracically displaced fetal stomach, which occurs in about 60% of the cases, a sonolucent area in the left chest may be found. In view of the dynamic nature of fetal stomach filling, more than one examination may be required to detect this feature. Antenatal recognition of congenital diaphragmatic hernia (CDH) has important consequences for perinatal management and can improve neonatal outcome.
A phenotypic boy presenting with gynaecomastia showed a mixed karyotype of 46,XX/46,XY/47,XXY. The left gonad was normally descended into the scrotum, but proved to be an ovary without any testicular structures. After left gonadectomy, plasma androgen and estrogen levels showed that the right gonad only contained testicular tissue. Seven patients with this form of triple mosaicism have been described but the clinical features are strikingly different among the described cases.
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In a double-blind placebo-controlled study in 49 boys with cryptorchidism the effect of intranasal synthetic LHRH was studied. After 8 weeks improvement in testicular location was found in 13 testes (37%), but this improvement was considered sufficient of only six testes. Placebo resulted in an improved location in 18% of the testes. The mean change in testicular position (expressed in cm) after LHRH therapy was slightly greater than after placebo but only in the squatting position did this difference reach significance. Aggressive behaviour was reported in 23% of the children treated with LHRH. A second LHRH course did not result in significant improvement in any of the patients. At follow-up reascent was frequently seen. The final results in unilateral cryptorchidism are poorer than those in bilateral cryptorchidism. LHRH therapy leads to higher plasma LH levels and a lower FSH in response to an intravenous LHRH test. In 15 boys plasma testosterone levels rose above 0.4 nmol/l. We conclude that intranasal LHRH application has a limited value for the treatment of cryptorchidism but may be suitable as a diagnostic test.
We describe 4 patients with a neonatal small left colon. From these cases and from the literature we know that in 40 to 50% the mother has diabetes mellitus. We describe the differential diagnosis with morbus Hirschsprung, meconium plug and the occurrence of a microcolon in atresias and meconium ileus. Three of the four patients were prematures. From the literature we know this abnormality is found mostly in term infants.
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Differences in the coagulation patterns between humans and dogs and the lack of reagents in the veterinary field to control haemostasis require a modification of existing screening techniques. The adaptations which were applied and which are described in this paper consist of greater dilutions of the samples. Two modified screening techniques in blood and plasma are described. The normal values of the different clotting factors and thrombokinetics, (thrombokinetogram, T.K.G.), using a normal beagle pool as a reference, are presented.
Repair of a congenital posterolateral diaphragmatic defect (CPLDD) by direct suture results in a flat drum-head diaphragm, which has very little function. Other harmful effects of such a repair include enlargement of the thoracic cavity, resulting in more overexpansion of the hypoplastic lungs which may contribute to pulmonary vascular obstruction, and decrease in volume of the abdominal cavity, making closure of the laparotomy difficult. Reconstruction of a diaphragmatic dome would result in a more functional diaphragm and would prevent the above complications. This was substantiated in a series of 10 babies, all severely symptomatic from birth and treated according to the proposed technique. Eight babies survived, the two deaths occurring 36 hours and 3 months after surgery, in babies with severe cardiac anomalies. It is concluded that reconstruction of the dome of the diaphragm in patients with a CPLDD is well tolerated and may result in an increased survival.
Forty-six babies with an unruptured omphalocele were admitted over a 10-year period. The conservative treatment consisted of the application of mercurochrome or an antibiotic powder, while the primary surgical treatment consisted of either full-layer closure or silastic sac insertion. Liver containing omphaloceles were considered large. Of the 25 babies without associated life-threatening congenital anomalies, all 9 with a small omphalocele survived, irrespective of the method of treatment. Sixteen babies had a large omphalocele of which all 8 conservatively treated babies survived against only 4 of the 7 who underwent surgery. The remaining baby, weighting 960 g, died prior to treatment, due to respiratory distress. Eighteen of the 21 babies with associated life-threatening congenital anomalies died, irrespective the extent of the defect. Although the conservative treatment of the large defects did not result in an improved survival rate, therapy-related complications did not occur. From this study it appears that large unruptured omphaloceles should be treated conservatively. Babies not doing well with a small omphalocele or a large one treated conservatively, will have one or more major associated anomalies, necessitating urgent diagnosis and treatment.
An experimental study was carried out investigating the feasibility of using only a portion of a donor liver in orthotopic, nonauxiliary transplantation, establishing a canine model. After being totally hepatectomized, 20 immunosuppressed adult beagles received a 60% donor liver graft, derived from tissue-typed identical littermates. Preservation time was 5 hr. In control experiments, 10 beagles, only half of them immunosuppressed, underwent an operation involving complete liver mobilization, vascular exclusion, cooling of the liver in situ, and a 40% partial hepatectomy with biliary anastomosis. The surgical procedure, including the method of cooling and preservation, is described in detail. Survival rate amounted to 90% for the control group and 50% for the transplanted group, while all surviving dogs were sacrificed 24 wk postoperatively. At sacrifice, the liver weight amounted to the weight of the total native liver at operation. Body weight profiles reflect good general condition of the surviving dogs. No major differences were noted between the immunosuppressed and the nonimmunosuppressed control dogs. No complications were related to the fact that only a partial liver was grafted or left in situ.
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Over a ten-year period 21 children with gastroschisis were treated either with a direct full-layer closure or with a silastic sac closure of the abdominal wall defect. No mortality or longterm morbidity resulted from either form of therapy in the group of patients that had no anatomical interruption of the gastro-intestinal tract. Silastic sac closure did, however, result in a higher incidence of septicemia. In the group of patients that presented with an anatomical interruption of the G.I. tract, considerable mortality and morbidity resulted. The outcome in these patients was determined by the associated bowel lesion rather than by the gastroschisis as such.
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Perforation of the appendix in the neonatal period may be a complication of neonatal necrotizing enterocolitis and should be differentiated from perforating appendicitis in later life. A patient is presented together with a review of the literature to illustrate this concept. Perforation of the appendix occurred in a 12-day-old preterm baby. Th cause of this perforation is assumed to be localized full thickness necrosis of the appendiceal wall, a form of neonatal necrotizing enterocolitis. The similarity between the clinical histories of neonates with so called "appendicitis" and those with necrotizing enterocolitis is pointed out. It is argued that "idiopathic primary peritonitis" probably does not exist, but that the peritonitis may be secondary to similar small perforations of the bowel. The importance of a thorough search for such a perforation is stressed.
The patency of the vascular anastomoses in a canine liver transplantation model was imaged by serial pervenous angiography using ioxaglate, which is a new ionic low osmolar contrast medium. No premedication or general anesthesia was administered. No major adverse reaction was noted, not even after repeated angiography. Except for the occurrence of one hematoma formation and one paravenous injection, which resolved without leaving sequellae, no local complications occurred. Sufficient information regarding the patency of the hepatic artery anastomosis was obtained in 94% of the angiographic studies, while in 70% the patency of the caval anastomoses could be determined. In addition, portal vein patency was imaged in 21% of the angiograms. Even better visualization results can be expected when combining this method with video subtraction techniques, although patient cooperation or general anesthesia would then be mandatory.