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Biomedical subjects

J G Raffensperger

Publications and source records attributed to J G Raffensperger.

At least 19 recordsLinked to original sources

Choledochal duct cyst: resection with physiologic reconstruction.

BACKGROUND: The accepted surgical treatment of choledochal duct cyst is complete excision and enteric drainage through an intestinal conduit. Peptic ulceration and fat malabsorption have been reported after Roux-en-Y reconstruction. Such long-term complications may be avoided by a technique that simulates normal physiology. METHODS: Twenty-one patients have undergone resection of a choledochal duct cyst in the past 12 1/2 years. The pathologic duct is resected to the level of normal mucosa. A short segment of jejunum with a intussusception valve (1.5 to 2 cm) is interposed between the common hepatic duct and the duodenum. The medical records and all radiographs of each patient were reviewed. Eighteen children were reexamined or the parents were contacted by phone. RESULTS: Twenty of 21 patients recovered without major perioperative complications. Twelve of them are well and have no symptoms at 3 to 12+ years (mean, 6 years) after operation. Four children are currently well 6 to 19 months after operation. Three children were well when lost to follow-up. Two patients have radiographic evidence of incompetence of the interposition valve. One of these, who initially underwent operation at 9 months of age, was reexplored at 10 months and at 10 years for a stricture at the hepaticojejunal anastomosis. The other, a 7-year-old girl who was admitted with jaundice and pancreatitis, has had episodic abdominal pain for 7 years after operation but is well. CONCLUSIONS: The valved jejunal interposition hepaticoduodenostomy offers superior biliary reconstruction after excision of a choledochal duct cyst. Normal physiology is simulated, with bile draining directly into the duodenum. A short conduit prevents stasis, and biliary reflux is minimized with the addition of an intussusception valve.

Child

Polyglyconate suture in pediatric surgery.

The authors have clinically evaluated a synthetic monofilament suture and have found it very satisfactory for all wound closures, esophageal and intestinal anastomoses, and bronchial closures.

Adolescent

A long-term follow-up of three patients with biliary atresia.

This report reviews the courses of three children who underwent surgery for biliary atresia. They include a patient who is now 25 years of age who underwent correction of an "operable" biliary atresia in the neonatal period. This patient is alive and well without jaundice. The second is a 21-year-old woman who had a Kasai operation, also performed in the neonatal period; she is well and attending school but does have an elevated bilirubin and signs of liver dysfunction. The third patient, who underwent surgery performed by Dr Willis Potts in 1963 for what appeared to be "inoperable" biliary atresia, finally underwent a portal duodenostomy after her third operation. She had satisfactory bile drainage but had numerous bouts of cholangitis early in her life. She survived until she had several major episodes of gastrointestinal hemorrhage from varices and finally died after a portal systemic shunt. These three patients illustrate that prolonged survival is possible after a variety of operations performed for biliary atresia. Furthermore, even though these patients were severely jaundiced early on in life and at least two of them had complicated postoperative courses, they are perfectly normal from an intellectual standpoint and have functioned in a normal fashion.

Biliary Atresia

Transfusion requirements in conservative nonoperative management of blunt splenic and hepatic injuries during childhood.

Nonoperative management of splenic and hepatic injuries in children is safe, and the majority of those with isolated injuries do not require blood transfusion. Thirty-seven children were treated for blunt splenic or hepatic trauma from November 1983 to September 1989. There was one death in a patient with a lethal head injury. No operations were performed on those with isolated splenic or hepatic injuries. Three of those with multiple injuries underwent delayed laparotomy. Two had perirenal and retroperitoneal hematomas without active bleeding, and one had a bowel obstruction secondary to an intramural jejunal hematoma. There were no late complications related to the splenic or hepatic injuries. Eight children (22%) required surgery for other injuries. Twelve children were not transfused, including the majority (8/11) of those with isolated splenic or hepatic injury. The hematocrit of four of these children fell to below 28% and this anemia was well-tolerated. Two children with bleeding disorders (factor VIII [antihemophilic factor] and factor XII [Hageman factor] deficiency) did not require packed red blood cells transfusion. Two clinically distinct groups of children received blood transfusions: (1) eight patients with multiple injuries were transfused during initial resuscitation when unstable or during early operation for other system trauma (mean, 62.0 mL blood/kg body weight); and (2) three hemodynamically stable patients with isolated injuries and 14 stable patients with multiple injuries were transfused empirically after initial resuscitation solely because of decreasing blood counts. They received an average of 16.5 and 21.1 mL blood/kg body weight, respectively.(ABSTRACT TRUNCATED AT 250 WORDS)

Accidental Falls

Splenic microabscesses in the immune-compromised patient.

Four immune-compromised children who were receiving antineoplastic chemotherapy (three for leukemia), presented with recurrent episodes of fever and left upper abdominal pain. Blood cultures grew enteric gram-negative organisms in three children. Multiple blood cultures were negative for fungus although three patients had mucocutaneous and urinary candidiasis. All remained febrile and symptomatic despite treatment with broad spectrum antibiotics and antifungal chemotherapy. Computed tomography (CT) scans in all patients showed 2- to 10-mm focal defects in the spleen. The larger defects could be seen by ultrasonography but not on the live-spleen nuclear scan. A splenectomy was performed 2 to 4 weeks after the onset of symptoms in each child, and the cut surface of the spleens showed multiple small abscesses. All operative cultures were negative. A histological examination confirmed Candida infection in two patients and Aspergillus in one. Necrotizing granulomas strongly suggestive of fungus were seen in the fourth child. The patients defervesced and appeared well within three days. Antifungal therapy was continued. One child remains in remission from acute lymphocytic leukemia; one continues on chemotherapy; and one has recurrent widespread tumor. The patient with Aspergillus died following a bone marrow transplantation 6 months after the splenectomy. He had disseminated aspergillosis. An immune-compromised patient with persistent unexplained fever should have a CT scan of the abdomen. The presence of multiple splenic lesions strongly suggests fungal disease. If antifungal therapy does not result in complete resolution of fever and the splenic lesions, a splenectomy is indicated.

Abscess

Vaginal neoplasms in infancy: the combined role of chemotherapy and conservative surgical resection.

This retrospective review of nine infants treated for vaginal tumors between 1964 and 1986 identifies the spectrum of lesions and examines the trend toward more conservative surgical therapy. Our patients' ages ranged from 1 to 30 months. Symptoms of vaginal bleeding or protruding tissue prompted examination under general anesthesia in all cases. Two children had benign protruding polypoid masses treated by simple excision. Seven had malignant tumors, two with endodermal sinus lesions and five with embryonal rhabdomyosarcoma. Early in this series, surgical therapy for embryonal rhabdomyosarcoma consisted of either anterior or complete pelvic exenteration, with adjuvant chemotherapy given in two of the three patients. One patient was operated on prior to the development of effective chemotherapy, and died of recurrent tumor 14 months after surgery. A second patient died from cardiac failure secondary to adriamycin toxicity 6 years after initial therapy. A third patient underwent total pelvic exenteration followed by successful reanastomosis of the colon to the anal verge. This patient, a female, is the oldest survivor and is free of disease 14 years after therapy. The most recent therapeutic approach used in two patients with embryonal rhabdomyosarcoma consisted of local tumor excision followed by postoperative chemotherapy. Both patients are alive and disease-free 9 and 11 years after therapy. Two patients with endodermal sinus tumor were treated with chemotherapy after simple excisional biopsy. They were then followed with serial vaginal biopsies at 3-month intervals. One required a partial vaginectomy for local recurrence 21 months after initial presentation. Both patients are alive and disease-free 18 months and 4 years after surgery.

Antineoplastic Combined Chemotherapy Protocols

Anorectal malformations: evaluation of associated spinal dysraphic syndromes.

The early recognition and treatment of correctable lesions of the terminal spinal cord in patients with anorectal malformations may preserve important neurologic function. Tethered cord and intraspinal masses are detectable with the use of high-resolution ultrasonography in the neonate. Fourteen infants and children with anorectal malformations and associated spinal dysraphism have been identified in our institution over the past 7 years. Six patients had cloacal exstrophy, and eight had imperforate anus (four high and four low lesions). The spinal lesions caused symptoms in only seven children; progressive neurologic deficit in five, and urinary incontinence or retention in two others. Five asymptomatic patients with cutaneous abnormalities on the back were studied and two were discovered during scoliosis evaluation. Imaging techniques included high-resolution ultrasonography, computed tomography with and without metrizamide myelography, and magnetic resonance imaging. Spinal sonography was highly accurate in the neonatal period. The application of ultrasonography can be of great advantage in early screening of patients with anorectal malformations and, in some cases, may eliminate the need for invasive imaging techniques.

Anus, Imperforate

Bronchoplastic techniques for pediatric lung salvage.

Bronchoplastic reconstruction can salvage normal bronchial and pulmonary tissue distal to diverse obstructive bronchial lesions. Five children underwent proximal bronchial and limited pulmonary resections allowing preservation of normal distal parenchyma. Three patients were infants between 2 1/2 and 12 months of age. All five survived without immediate or late complications. Follow-up included interval bronchoscopy and chest radiography, and ranged from 8 months to 12 years. Cardiopulmonary bypass was unnecessary even in the case of a large carinal lesion. Techniques used to insure adequate ventilation during bronchotomy included positioning the endotracheal tube above the carina with clamp occlusion of the open mainstem bronchus, positioning the tube within the opposite mainstem bronchus, and inserting a sterile tube into an open mainstem bronchus (with connections to sterile ventilator tubing passed across the operative field). Preoperative bronchoscopy and radiographic studies localized the extent and base of the lesion, allowing precise placement of the bronchotomy. Conservative excision was accomplished by frozen section examination of all margins of resection. Ingenious usage of available tissue was essential to a successful reconstruction.

Bronchi

Long-term complications in a premature infant with tracheoesophageal fistula.

This is a report of the short- and long-term complications in a premature infant with tracheoesophageal fistula, including those related to central venous alimentation, seizures, chylothorax, bronchopulmonary dysplasia, dental erosions, gastroesophageal reflux, pulmonary problems, and gall stones. It offers analyses of possible alternate methods and treatments, which may have provided a better course.

Abnormalities, Multiple

Emergency lung biopsy: friend or foe of the immunosuppressed child?

An acute pneumonic process in an immunosuppressed child poses a diagnostic and therapeutic challenge. These patients tolerate infection poorly. An open lung biopsy may provide prompt diagnosis. Nevertheless, a beneficial change in therapy that results in survival does not necessarily follow. Fifty-six immunosuppressed children with acute respiratory symptoms and interstitial pulmonary infiltrates underwent lung biopsy from 1974 to 1985. The most common underlying diagnosis was acute lymphocytic leukemia (60%). A specific etiology was determined in 46 (82%). Operative morbidity in 52% included prolonged intubation, recurrent pneumothorax, and hemorrhage. Overall, mortality was 34%. Those patients with solid tumor and those who required postoperative ventilation had a statistically significant higher mortality than all others. We defined biopsy "patient benefit" as follows: (1) the biopsy yielded an etiology for which a change of treatment was required; and (2) the child survived this acute illness. Despite the successful diagnostic results of this procedure, only 13 (23%) of the patients derived clinical benefit. Even though a specific infectious etiology was diagnosed in 39 (69%) patients only ten (18%) of these improved and survived after an appropriate change in therapy. Eight of these had Pneumocystis carinii. One survivor benefited from the treatment of documented radiation pneumonitis. Another was successfully treated for graft v host reaction but this diagnosis also was made by skin biopsy. One half of the biopsies were performed very early in the course of the illness, specifically to exclude Pneumocystis carinii of which we saw a peak incidence in 1978 to 1979.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease

Eikenella corrodens infections in children.

Techniques for obtaining, transporting, and culturing bacteriologic specimens have improved in recent years. The laboratory is now identifying rare fastidious organisms in surgical infections, which were previously unknown to clinicians. One of these organisms, Eikenella corrodens, was found in 28 children over 5 years. It was most commonly found in patients with perforated appendices or in wounds with oral contamination. Eikenella most often occurs in multibacterial infections but has also been grown in pure culture. When this bacteria is found in a patient with a serious surgical infection the wound must be widely opened and debrided of necrotic tissue. Prolonged antibiotic therapy based on culture and sensitivity is necessary to prevent prolonged or recurrent infection.

Abscess

The valved conduit prevents ascending cholangitis: a follow-up.

Reflux of bacteria-laden intestinal contents into the biliary tree can be prevented by an intussuscepted valve in an isolated segment of jejunum interposed between the porta hepatis and the duodenum. This method of bile duct reconstruction was adopted in 1979 and since then has been used in 17 children with biliary atresia and 9 with choledochal duct cysts. Those with choledochal duct cysts are well. Follow-up HIDA scans demonstrate normal bile flow, and ultrasound examinations have not revealed dilated bile ducts. Bile flow was established in 13 infants with biliary atresia and was persistent in 8. Two expired because of unrelated problems between 2 to 6 months postoperatively. One of these children developed cholangitis, but at autopsy the nipple valve was incompetent. Six children currently have normal serum bilirubin levels and are clinically well. The average postoperative stay for these patients was 7.5 days. Three were rehospitalized for brief periods for suspected cholangitis which was not proven. Bile flow was never established in 4 babies and was present only briefly in 5. Five of these children died of progressive liver failure and one from unrelated causes. Two have had liver transplants, and a third is awaiting transplantation.

Bile