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Biomedical subjects

J G Raffensperger

Publications and source records attributed to J G Raffensperger.

At least 55 records · Page 3Linked to original sources
PubMed1982

The spectrum of meconium disease in infancy.

Meconium diseases in infancy cannot be neatly separated into discrete categories of meconium plug syndrome, meconium ileus, and meconium peritonitis; nor can the therapy of each condition. A patient with any of the three may or may not have cystic fibrosis. All babies with any form of meconium obstruction or atresia must have a sweat chloride test to confirm or rule out this diagnosis. Repeated gastrografin enemas can decrease the need for operative intervention in all babies with meconium plug syndrome and in selected babies with meconium ileus. Babies with meconium diseases can now be managed with a low perinatal mortality.

Barium Sulfate↗
PubMed1982

Congenital colonic atresia.

Nineteen cases of congenital colon atresia are presented with two deaths resulting in a mortality rate of 10.5%. The authors conclude that operative management of atresias proximal to the splenic flexure should include resection of the dilated proximal colon with primary ileotransverse colostomy or ileosigmoidostomy. Atresias distal to the splenic flexure should be managed with a colostomy with later establishment of gastrointestinal continuity.

Colon↗
PubMed1982

Megarectum: a rare complication of imperforate anus repair and its surgical correction by endorectal pullthrough.

Nine patients ranging in age from 3 mo to 15 yr presented with severe constipation and/or increasing incontinence. All had a huge, dilated atonic rectum and rectosigmoid demonstrated by barium enema. Six patients underwent resection of the abnormal bowel by an endorectal pull-through procedure with good to excellent results while 1 patient was corrected by a Swenson procedure.

Adolescent↗
PubMed1982

Endodermal sinus tumor: a clinical and pathological correlation.

Endodermal sinus tumors occur in the gonads and in a variety of extragonadal locations, particularly as the malignant component of sacral teratomas. Light and electron microscopic studies as well as the presence of alpha fetoprotein support the concept that these lesions arise from germ cells that migrate from the extraembryonic yolk sac. These tumors in all locations are highly malignant and metastasize to lymph nodes, lung, liver and bone. Peritoneal implants are seen from tumors in the ovary, testis, and following ventriculoperitoneal shunts for yolk sac tumors of the pineal gland. There is no satisfactory therapy for the endodermal sinus tumor, regardless of location, when there are distant metastases at the time of diagnosis; however, we have documented complete regression of metastases in one child following therapy with vinblastine, bleomycin, and cis-platinum. Nine of 13 boys with endodermal sinus tumors of the testis survived in this series of patients. All survivors were treated with orchiectomy and lymph node excision. Surgical excision combined with chemotherapy and radiation led to survivors in children with large extragonadal pelvic and retroperitoneal tumors. We recommend vigorous multimodal therapy with surgery and chemotherapy. Radiation is given to the primary lesion when it is too extensive for initial resection or to eradicate residual disease following chemotherapy. Alpha fetoprotein is a valuable tumor marker.

Adolescent↗
PubMed1982

Mini-thoracotomy and chest tube insertion for children with empyema.

In contrast to the two prior decades, empyema in children has become a rare disease during the past 10 years. Thirty-two children with empyema from all causes were treated at The Children's Memorial Hospital in Chicago in the 11 years between 1970 and 1982. Twenty cases followed postinfectious pneumonia, most commonly due to Staphylococcus aureus. The remaining patients had either an esophageal leak or a pleural infection following an intrathoracic operation. Fourteen of our patients were managed with a "mini-thoracotomy," which allowed accurate drainage of purulent material, débridement of fibrinous exudate, and rapid expansion of the lung with prompt relief from fever and toxicity. In our hands, this procedure provided results which were superior to those obtained with simple closed chest tube drainage. In addition, during this period of time, ultrasound examination of the chest has provided a rapid, accurate technique for the early detection and localization of fluid accumulations within the pleural cavity. Pre-drainage thoracentesis can be accurately guided by ultrasonic imaging.

Child↗
PubMed1981

The evolution of a valved hepatoduodenal intestinal conduit.

Ascending cholangitis remains among the most serious complications following operations for biliary disorders. The bacterial count of refluxing intestinal contents can be reduced by using an enteric conduit from the biliary tract to the relatively sterile duodenum. A valvular conduit prohibits reflux of intestinal contents and permits unobstructed antegrade flow of bile. This can be created by intussuscepting approximately 1 cm of intestine in the midportion of the conduit. During the last 3 yr, valvular conduits were created in 11 patients. Seven of these children were treated for biliary atresia, and 4 had operations for choledochal duct cysts. There have been no deaths or morbidity resulting from the use of the valvular conduits. The postoperative courses in these patients indicate that the use of an intussusception valve may be beneficial in the prevention of ascending cholangitis.

Animals↗
PubMed1981

Circular esophagomyotomy for primary repair of long-gap esophageal atresia.

Twelve of forty patients (30%) treated for esophageal atresia at the Children's Memorial Hospital in Chicago between June 1976 and May 1980 required a circular esophagomyotomy with or without upper pouch bougienage to achieve an anatomical repair. Ten patients had a distal tracheoesophageal fistula; 1 had a proximal fistula as well; and 2 had no fistula. Over 40% of the patients were small for gestational age. One half had severe associated anomalies including congenital heart disease in 1/3 and the VATER association in 1/4. Three patients died, all from congenital heart disease. Immediate complications in 6 patients included anastomotic leaks (3), tracheal injuries (2), and mucosal entry at the myotomy site (1). Late complications included symptomatic gastroesophageal reflux (60%), anastomotic strictures (40%), and severe tracheomalacia requiring long-term tracheotomy (20%). Altered esophageal motility (77%) contributed to poor weight gain (less than 3rd percentile) in most patients. In spite of these complications, satisfactory results were achieved in 8 of the 9 long-term survivors. The ninth patient had a turbulent neonatal period and has persistent swallowing difficulties at 36 mo follow-up. Although an anatomical repair can now be achieved in all patients with esophageal atresia utilizing bougienage and myotomy, some ill neonates may be better served by traditional staging techniques.

Esophageal Atresia↗
PubMed1980

Brain heterotopia in the lungs. A rare cause of respiratory distress in the newborn.

This article documents the presence of multifocal, bilateral glial heterotopia in the lungs manifesting as symptomatic lung masses during the newborn period. Prior to the present report, this rare form of heterotopia was known to occur exclusively in anencephalic infants. The observed bilateral pulmonary disease had an important cystic component, clinically and radiologically similar to cystic adenomatoid malformation. Theoretically, several explanations may be elaborated to account for the presence of central nervous tissue in the lungs: embolic events; protrusion of embryonic brain structures that are subsequently dislodged from their normal site; in-situ transformation of local developing tissues; and teratoma with predominance of neural tissue. The evidence is insufficient to conclude in favor of any of these explanations.

Brain↗
PubMed1980

Pancreatic pseudocysts in children.

The clinical presentation and operative treatment of 15 children with pseudocysts of the pancreas were reviewed. All had a self-limiting process, usually resulting from trauma, and did well, regardless of the type of drainage. Those children undergoing internal drainage had fewer complications and left the hospital earlier than did those undergoing external drainage. However, there were no recurrences after either internal or external drainage, and the two fistulas which developed following external drainage closed spontaneously.

Adult↗
PubMed1979

The effect of overdistention of the lung on pulmonary function in beagle puppies.

The removal of one lung from a beagle puppy results in minimal interference with lung function or the arterial gases. The removal of air from the empty pleural cavity results in a shift of the mediastinum and overdistention of the contralateral lung. An immediate decrease in the PO2 and increase in the PCO2 is seen. Significant increase in the alveolar-arterial CO2 gradient reflected marked increase in dead space ventilation. Biopsies of the overdistended lung demonstrated emphysema and disruption of alveoli. These changes may explain some of the deterioration of lung function and the complication of contralateral pneumothorax following repair of a Bochdalek diaphragmatic hernia. Our study suggests that the mediastinum should be stabilized in the midline after repair of a diaphragmatic hernia or after a pneumonectomy in an infant or small child.

Animals↗
PubMed1978

Perforation of the gastrointestinal tract in infancy and childhood.

Eighty-eight patients with perforations of the gastrointestinal tract in infancy and childhood, excluding those caused by appendicitis, are presented. Fifty-five patients were four weeks of age or younger. Fourteen of the perforations occurred during the first year of life and 19 occurred between one and 15 years of age. The locations of perforations by order of frequency were the ileum, rectosigmoid, stomach and duodenum. The causes in order of frequency were necrotizing enterocolitis, ulcers, unknown causes, Hirschsprung's disease, atresia of the small intestine, volvulus, trauma, gastroschisis and ventriculoperitoneal shunts for hydrocephalus. A high index of suspicion, aided by roentgenograms, is essential for an early diagnosis. The over-all mortality was 49 of 88 patients. No reduction in mortality was observed in the last ten years, despite improved surgical techniques and better antimicrobial agents. Early recognition and rapid transport of the child to a pediatric intensive care unit with better supportive measures plus antimicrobial agents effective against both anaerobic and aerobis bacteria should reduce this high mortality.

Adolescent↗
PubMed1978

Surgical management of tracheosophageal fistula complicating caustic ingestion.

This report outlines principles of management for extensive corrosive esophageal burns in children complicated by formation of a tracheoesophageal fistula (TEF). Direct operative attack on the fistula usually is unsuccessful, since the tracheal tissues are so damaged that they will not hold sutures. On the basis of experiences with six children, we suggest the following plan of management: (1) early investigation of suspected TEF with thin barium or Dionosil; (2) early tracheostomy using a short, plastic tube; (3) end cervical esophagostomy with closure of the distal stump of the cervical esophagus; (4) gastrotomy; (5) complete disconnection of the intra-abdominal esophagus from the stomach. This can be completed in a single operation and leaves the thoracic esophagus containing the fistula completely isolated so that the trachea is protected from contamination by saliva and gastric juice. The esophageal mucosa in all cases has been destroyed so extensively by the corrosive material that the esophagus heals as a band of muscle and scar. If protected from continuous contamination by saliva, the trachea heals itself with little long-term defect. The esophagus is replaced with colon or a gastric tube 6 to 12 months later when the child is in good health. All four children treated by this regimen have survived and are able to eat normally.

Burns, Chemical↗
PubMed1977

Surgical complications of Ehlers-Danlos syndrome in children.

The Ehlers-Danlos syndrome is a special challenge to the surgeon because of its relative infrequency and the late onset of overt symptoms, such as hyperelastic skin. We should consider this diagnosis in every child with recurrent inguinal herniae and search for its stigmata. A child with a known Ehlers-Danlos syndrome who requires an elective operation should be carefully studied for coagulation disorders. If there is a history of past injuries or operations in which there was poor healing, the benefits of the proposed operation must be carefully weighed against possible complications. Operations must be carried out with great care to avoid tearing the skin and fascia. Meticulous attention is given to hemostasis to prevent postoperative bleeding or hematomas. Inguinal hernias in these children resemble those seen in elderly patients. The transversalis fascia is thin and the internal ring is hugely dilated. An adult-type repair, with the use of mesh or felt may result in a lower incidence of recurrence. In addition, the Ehlers-Danlos syndrome may be yet another etiology for obscure gastrointestinal bleeding in children. Specific enzyme defects have been identified in 2 of the 7 subtypes of this disease. Further, biochemical study of connective tissue defects may contribute to our knowledge of normal collagen synthesis and wound healing.

Child↗
PubMed1977

Pediatric esophageal perforation.

Although esophageal perforation in children is associated with a spectrum of disease different from the one in adults, management is essentially the same for both groups. Over the past 11 years, 12 patients ranging in age from 2 days to 10 years were treated for 13 perforations. Perforation in the adult is associated with a high mortality rate. All children in this series survived. Injury was secondary to instrumentation in 10 cases and three resulted from the chronic erosion of a trapped foreign body. Of the 10 acute perforations, five occurred 12 days to 5 months after lye ingestion. Anastomotic narrowing was a factor in three other cases. Seven of the acute perforations involved the thoracic esophagus and were associated with a high morbidity rate. Management consisted of operative as well as nonoperative approaches. Consideration must be given to several clinical variables including location, cause, predisposing factors, underlying illnesses, associated injury, and promptness of medical attention.

Bronchial Fistula↗
PubMed1977

Acute abdominal pain.

Acute abdominal pain is a daily problem in a busy pediatric practice and in hospital emergency rooms. An initial, carefully performed history and physical examination should differentiate the child who has a surgical disease from one who requires medical therapy or only reassurance. Whenever there is doubt about the diagnosis, the child must be admitted to the hospital for observation and appropriate laboratory examinations. This will allow for the early diagnosis of appendicitis and other diseases requiring an operation and will prevent needless operations on children with medical illnesses.

Abdomen↗