Jonathan Aaron Arkin: a commemoration.
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Biomedical subjects
Publications and source records attributed to J G Raffensperger.
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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.
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.
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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.
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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.
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)
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.
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.
Children with foregut cysts of the mediastinum can present at any age with nonspecific respiratory symptoms or dysphagia. Chest radiograph and barium esophagram are recommended for initial evaluation, but they fail to identify some lesions. In other cases, an obvious mass may be confused with a solid neoplasm. We have operated upon 34 infants and children with mediastinal bronchogenic cysts and esophageal duplications from 1968 through 1985. This review of their clinical course and radiographic imaging studies emphasizes some of the diagnostic pitfalls that lead to operative delay. Twelve (35%) of these patients were asymptomatic. The correct diagnosis was delayed longer than 3 months from the onset of symptoms in 14 of the 22 symptomatic children. Fifteen of these presented with pneumonia or symptoms or airway obstruction. In 11, the cyst was in a perihilar or subcarinal location, areas in which a lesion can be "hidden" behind the cardiac silhouette. Five children with esophageal duplication had severe neonatal respiratory failure, chest pain, hematemesis or dysphagia. The immediate preop chest radiograph revealed a mass in 29 of 34 cases. However, the lesion was initially missed or never seen in eight of those who were symptomatic. Esophagram, performed in 23, was diagnostic in only six and was normal in four. Prior to 1979 when CT scanning became available at this institution, 11 of 19 children (57%) underwent extensive work-up, but the preop diagnosis was correct in only 50%. Since 1979, only 4 of 15 (26%) have required similar evaluation, and the preop diagnosis has been correct in all.(ABSTRACT TRUNCATED AT 250 WORDS)
Primary tissue closure of gastroschisis remains controversial. Some surgeons routinely place a silicone rubber sheet silo over the exposed bowel, planning a staged closure. In the past 14 1/2 years, we have cared for 106 newborns with gastroschisis, closing the defect primarily in 80%. The success of this technique depends on enlarging the abdominal cavity and decreasing the volume of bowel that must be replaced in the peritoneal cavity. Thorough preoperative rectal irrigation should evacuate all meconium. After undermining the skin around the abdominal wall defect for only 1 cm, a midline subcutaneous fasciotomy is created from the xiphoid to the pubis. The abdominal wall is then stretched in all quadrants beginning at the flanks. The eviscerated small bowel can often be returned without enlarging the initial skin defect. The skin is closed with subcuticular absorbable sutures reinforced by long skin tapes. The small ventral hernia that results is closed at about 1 year of age. Fascia could be closed primarily in 28% of these patients, and 17% required a prosthetic pouch. The duration of postoperative ileus and length of hospital stay were statistically significantly shorter in the infants who underwent primary closure. Even though more complicated patients were included in the primary closure group, the incidence of mortality and morbidity was not higher than in patients treated with silicone rubber pouches. Deaths were inevitable in five infants with gangrenous bowel, multiple anomalies, and extreme prematurity. Deaths were related to sepsis in three infants and were the result of operative or anesthetic technique in four. Only two preoperative factors were prognostic of morbidity and mortality: gestational age (but not birth weight) and the presence of intestinal ischemia or atresia.
House staff stipends and other costs related to maintaining a residency are listed on hospital budgets as "education." This has become a tempting target for hospital administrators and third-party payors, who ask, "Should the patient pay for medical education?" We have studied the workload of house officers on a typical pediatric surgical service in a children's hospital. General surgical residents spend 56.3 hours per week in patient care. This does not count "lag" time or "on call" time, but work that someone else--either a physician, technician, or nurse practitioner would do if there was no residency. This study, as well as others, clearly indicates that residents provide an enormous amount of patient care. It is perfectly justified to include the costs of house officers in the hospital budget.
General and pediatric surgeons commonly treat hypertrophic pyloric stenosis. A skillful pyloromyotomy without complications culminates in a gratifying experience. An instrument designed for this purpose should be safe, versatile and easy to use. A modified curved, 6 inch clamp facilitates doing a pyloromyotomy by implying the same action as other more commonly used instruments. The natural spreader for the pylorus is easy to make and it is also relatively inexpensive.
Mesenchymal hamartoma is a benign, cystic lesion that arises from connective tissues of the portal tracts. It is most common in children under 5 years of age. The diagnosis can be suspected when a relatively asymptomatic child has a large, smooth mass in the liver. Radionuclide scans demonstrate a solitary lesion, and on ultrasound there are cystic spaces and septa. These are perfectly benign lesions and may be simply enucleated.