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Biomedical subjects

R J Touloukian

Publications and source records attributed to R J Touloukian.

At least 19 recordsLinked to original sources

Pediatric surgery between 1860 and 1900.

The twentieth century begins the modern era of pediatric surgery, a time of innovation and vastly improved operative survival. Until then, many correctable congenital anomalies had been described in case reports only as medical curiosities, seemingly having no chance for surgical cure, but the challenge was available to those wishing to accept. The first texts devoted to surgical diseases of infancy and childhood were descriptive, emphasizing visible and palpable lesions, tuberculosis, musculoskeletal deformity, and major trauma, but also recognized that "surgical diseases of children by their number, their variety, and their character must, we think, be studied in a special manner and demand a special chapter in surgical books." The construction of Children's Hospitals during the nineteenth century identified the need to provide special facilities for children. Surgical antisepsis was practiced and general anesthesia used. Physicians such as Hirschsprung recognized a wide spectrum of treatable surgical conditions that would soon become the domain of pediatric surgeons. Indeed, at that moment in time at the very beginning of the twentieth century, Roentgen discovered the x-ray, Ladd received his MD degree at Harvard, the first pediatric appendectomies for appendicitis were successfully performed, and Fredet and Ramstedt recognized that pyloroplasty was not the preferred treatment for hypertrophic pyloric stenosis. Modern pediatric surgery had passed from embryo to fetus to newborn.

Denmark

Midgut volvulus. An ever-present threat.

OBJECTIVE: To identify risk factors for midgut volvulus (MGV) and to seek clues to early diagnosis of MGV in children with malrotation. DESIGN: Retrospective patient series. SETTING: Academic medical center. PATIENTS: Sixty-eight consecutive children who had a Ladd operation performed between January 1970 and December 1991. Excluded were three patients whose records were unavailable and patients who had a Ladd operation during the course of repair of an abdominal wall defect or congenital diaphragmatic hernia. RESULTS: Forty of 68 patients had MGV at operation. There was an inverse correlation between age at onset of symptoms and the probability of MGV: 85% (29/34) of patients less than 1 month of age had MGV compared with 43% (10/23) of older children. Patients who had symptoms for less than 4 days were more likely to have MGV (88%; 30/34) than patients who had more chronic symptoms (43%; 10/23). Bilious (green) vomiting was more highly associated with MGV (80%; 35/44) than nonbilious vomiting (38%; 3/8) or pain (50%; 6/12). Roentgenograms of the upper gastrointestinal tract were very accurate for the diagnosis of malrotation but frequently failed to identify MGV (sensitivity, 54%; 13/24). Despite the high proportion of MGV, only three patients had gangrenous bowel. Of these three patients, one died and two have short-gut syndrome. CONCLUSION: Neonates with a short history of bilious vomiting are most likely to have MGV-complicating malrotation, but older children who have chronic intermittent symptoms are also at risk. Since there is no way to predict which patients will develop catastrophic bowel necrosis, early diagnosis and operation are necessary to prevent mortality and short-gut syndrome.

Acute Disease

Retrosternal ileocolic esophageal replacement in children revisited. Antireflux role of the ileocecal valve.

The risk of postoperative reflux and pulmonary aspiration with straight colon or gastric tube esophageal replacement in children prompted us to reevaluate the presumed antireflux role of the ileocecal valve with retrosternal ileocolic interposition. This operation was done in eight patients with esophageal atresia (six) and lye stricture (two) from 19 to 50 months of age between 1983 and 1992. There were no operative deaths. The duration of follow-up ranged from 4 to 115 months. Barium swallow obtained in all patients showed unobstructed esophagoileocolic transit without reflux. Two patients with esophageal atresia had localized proximal anastomotic leaks, which healed spontaneously without stricture. In the two patients with lye ingestion ileoesophageal strictures developed that necessitated revision. None of the patients had postoperative respiratory complications or symptomatic gastroesophageal reflux. All eight children have had their gastrostomy tubes removed, are eating a regular diet, and are growing well. In conclusion, the retrosternal ileocolic conduit provides an excellent substitute esophagus in selected pediatric patients, with potential advantages over delayed primary anastomosis or the straight colon or gastric tube interposition because of the antireflux role of the ileocecal valve.

Burns, Chemical

Predictors of postoperative respiratory complications in premature infants after inguinal herniorrhaphy.

There is a significant incidence of inguinal hernia in premature infants and the optimal timing of repair is controversial. A high rate of postoperative respiratory complications has been reported in this group. In this study, the records of 47 premature infants (mean gestational age, 30.3 weeks) who underwent herniorrhaphy while still in the neonatal intensive care unit were reviewed in an effort to define those conditions that are independent risk factors for complications. Forty-three percent of infants had complications, including postoperative assisted ventilation (34%), episodes of apnea and/or bradycardia (23%), emesis and cyanosis with first feeding (6%), and requirement for postoperative reintubation (4%). Although low gestational age and postconceptual age at operation, low birth weight for gestational age, and preoperative ventilatory assistance were significantly associated with postoperative complications, only a history of respiratory distress syndrome/bronchopulmonary dysplasia (odds ratio 2.3), a history of patent ductus arteriosus (odds ratio 2.5), and low absolute weight at operation (odds ratio 3.5 for 1,000-g decrease) were independent risk factors for postoperative complication. Despite previous reports citing postconceptual age as the factor having the greatest impact on postoperative complications, these results indicate that a history of respiratory dysfunction and size at operation may be more important predictors of postoperative respiratory dysfunction in preterm infants.

Age Factors

Intestinal rotation and fixation abnormalities in heterotaxia: early detection and management.

Intestinal rotation and fixation abnormalities (IRFA) are known to coexist with heterotaxia (defined as an abnormal arrangement of body organs that is different from complete situs solitus or complete situs inversus), but little is known about the incidence of this association or its clinical management. We have reviewed the records of 34 patients diagnosed with heterotaxia during a 12-year period at Yale-New Haven Hospital in order to develop a plan for the early diagnosis of IRFA and to assess the value of preventive treatment in this complicated group of patients. Of the 34 patients with heterotaxia, all except one presented with complex congenital heart disease. The 34 patients were divided into two groups on the basis of their gastrointestinal workup for suspected IRFA. The 28 patients in group A had no upper gastrointestinal (GI) contrast study performed prior to symptoms suggestive of IRFA. Four of these 28 patients (14%) eventually developed complications of IRFA requiring emergency surgery. Group B consisted of six patients seen during the past 5 years with heterotaxia who had upper GI contrast studies while asymptomatic. All six (100%) were shown to have IRFA and subsequently underwent an uncomplicated elective Ladd procedure once their cardiac condition stabilized. This study confirms the high incidence of IRFA in patients with heterotaxia. We propose that evaluating patients with heterotaxia syndrome for IRFA while asymptomatic may prevent the need for emergency abdominal surgery in patients that have major cardiovascular anomalies.

Abnormalities, Multiple

Outcome of nonoperative management of splenic injury with nuclear scanning. Clinical significance of persistent abnormalities.

Uncertainties remain about the frequency and need for diagnostic imaging following recovery from splenic injury with nonoperative management. To gain further understanding, the final appearance of the splenic roentgenographic image was evaluated in 20 consecutive children (mean age, 10.1 years) undergoing serial studies up to 70 weeks following injury. A total of 65 technetium 99m sulfur colloid scans, including 45 follow-up studies, were obtained and evaluated. By 20 weeks following injury, six patients (30%) were normal, four (20%) demonstrated minimal residual effects, and 10 (50%) had significantly improved, leaving some persistent abnormality. None of the patients in the last group showed any clinical problem. No distinctions could be made by comparing the severity of the initial injury with a persisting imaging defect. We conclude that clinical considerations alone should determine whether any follow-up imaging be performed in children recovering from splenic injury.

Adolescent

Reassessment of the end-to-side operation for esophageal atresia with distal tracheoesophageal fistula: 22-year experience with 68 cases.

End-to-side anastomosis (ES) and ligation of the tracheoesophageal fistula (TEF) has been the procedure of choice for esophageal atresia at our institution since 1967. This report summarizes our operative and long-term results with the ES operation in 68 babies, including 33 in Waterston group A (50%), 23 in group B (35%), and 12 in group C (15%). An additional 10 patients had a primary end-to-end (EE) anastomosis, while 14 others required either staged EE repair or an esophageal replacement procedure. Overall survival rate with ES was 93% including two deaths attributed to major anastomotic leaks and sepsis, and three others in group C from cardiac anomalies. Six (9%) of those having ES anastomosis developed a recurrent TEF between 40 days and 21 months of age, necessitating reoperation. Predisposing factors to recurrent TEF were surgical inexperience (three cases; first operation for each surgeon), forceful vomiting secondary to gastroesophageal reflux (GER) in two, and drug overdose in one. Anastomotic leak occurred in seven (10%) following end-to-side repair and was implicated in two deaths. Three patients developed minor anastomotic stricture requiring less than three dilatations, while one with a tight stricture needed as many as five dilatations over the first 14 months of life. Mild dysphagia and respiratory symptoms were uniformly observed during the first year, but only five patients (7%), including the two with recurrent TEF, required fundoplication for persistent GER. All patients were eating table foods after 1 year of age, while 10 (15%) have required periodic endoscopic removal of solid food lodged at the radiographically unobstructed anastomosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical

Familial occurrence of esophageal atresia with and without tracheoesophagel fistula: report of two unusual kindreds.

We describe 2 unique kindreds with familial occurrence of esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and reviewed the literature on familial EA +/- TEF. EA +/- TEF appears to be causally heterogeneous with evidence pointing to the existence of non-genetic developmental and multifactorial forms. The literature suggests that the parents of a single affected child should be given an empiric recurrent risk between 1/2 and 2%, rising to 20% if more than one sib is affected. The empiric risk of an affected child born to an affected parent is 3-4%. Empiric risk figures are useful in counseling families at the present time; however, the 2 kindreds presented here raise the possibility of autosomal dominant transmission in certain families. A third generation of affected offspring, or additional family reports should help to clarify this issue in the future.

Adult

Evaluation of Nissen fundoplication in neurologically impaired children.

The value of performing Nissen fundoplication in neurologically impaired children is a controversial issue. To evaluate the benefit of fundoplication in these children, hospital records were reviewed for 77 children who underwent fundoplication for gastroesophageal reflux (GER). Fifty-two children were neurologically impaired; 25 children had no neurological impairment. Impaired children had significantly fewer hospital admissions (1.8 v 0.7; P less than .005) and total days of hospitalization (36 v 14; P less than .005) during the first postoperative 6 months, compared with the immediate preoperative 6-month period. Normal children had fewer hospital admissions and days postoperatively, but the difference was not significant. Impaired children with preoperative failure to thrive (FTT had significantly increased average monthly weight gain over the first 6-month period postoperatively, compared with preoperative growth rate (3.0% v 0.9% of total body weight; P less than .05). Average monthly weight gain at 1 and 2 years postoperatively was not significantly different from preoperative values for impaired children. Growth rate of normal children with FTT did not change significantly postoperatively. Symptomatic relief was comparable in the normal and impaired children. Perioperative mortality was 0% in the normal children and 6% in the impaired children. This study demonstrates that Nissen fundoplication in neurologically impaired children with GER can be performed safely, reduces the frequency of hospitalization, and improves short-term weight gain.

Child

Malignant potential of juvenile polyposis coli. Report of a case and review of the literature.

Juvenile polyps of the colon and rectum traditionally have been viewed as being benign inflammatory or harmartomatous lesions without potential for malignant change. The authors report a case of adenocarcinoma developing in a patient with sporadic juvenile polyposis. Juvenile polyposis was diagnosed in the patient at age 4 years. He underwent subtotal colectomy at age 6 years. At age 12, he underwent a proctectomy and a Swenson pull-through because of adenomatous changes in the rectal stump. At age 19 surveillance endoscopy revealed invasive cancer in a juvenile polyp.

Adenocarcinoma

Partial splenectomy for symptomatic splenic hamartoma.

Lower pole splenectomy with preservation of residual splenic function was successfully performed in a 3-year-old boy with a symptomatic splenic hamartoma. Following resection, several of the patient's constitutional symptoms resolved. This is the first reported case of a splenic hamartoma treated by partial splenectomy.

Child, Preschool

Cesarean section does not improve outcome in gastroschisis.

Elective cesarean section (CS) following prenatal diagnosis of gastroschisis has been advocated to decrease morbidity and mortality. To examine this hypothesis, we reviewed the records of 28 consecutive patients with gastroschisis treated between 1975 and 1987. Fourteen infants were delivered vaginally (V) and fourteen by CS, of which seven were elective, five were for fetal distress, and two were for breech presentation. Prenatal diagnosis was made in nine infants in the CS group and none in the vaginal group. The two groups were comparable in gestational age (V = 37.6 weeks, CS = 35.8 weeks, P = .05), birth weight (V = 2,508 g, CS = 2,444 g, P = NS), and five-minute Apgar score (V = 7.8, CS = 6.8, P = NS). Outcome was similar as measured by hospital mortality (V = 0/14, CS = 1/14, P = NS), complications (V = 4/14, CS = 5/14, P = NS), days to enteral feeding (V = 14, CS = 19, P = NS), and days in the hospital (V = 27, CS = 34, P = NS). The only complication related to mode of delivery was preventable; an infant delivered vaginally had avulsion of a short segment of mesentery requiring bowel resection. Infants born by CS were slightly more likely to have primary closure (5/14) than babies delivered vaginally (3/14, P = NS), but this may reflect independent trends in the last 5 years. Elective CS following prenatal diagnosis in seven patients did not improve outcome; primary closure was achieved in only one infant, and three had a complication. Since these data show no significant difference in morbidity and mortality between vaginal and CS delivery, we suggest that CS should not be recommended simply because a prenatal diagnosis of gastroschisis is made.

Abdominal Muscles

Congenital lobar emphysema. The roles of CT and V/Q scan.

An infant with congenital lobar emphysema of the left upper lobe presented with unusually severe mediastinal shift and underwent computed tomography (CT) and radionuclide V/Q scans. The V/Q scan confirmed the non-functioning nature of the hyperinflated lobe while the CT scan depicted the abnormal anatomy as well as the normal morphologic characteristics of the remaining lung thus allowing for safe and appropriate surgical management.

Emphysema

Meconium pseudocyst presenting as a buttock mass.

A newborn 2.4-kg baby girl with a prenatal diagnosis of sacrococcygeal teratoma by ultrasound had a soft cystic mass extending from the infracoccygeal region into the right buttock. The cyst contained meconium leading to a large retroperitoneal cavity. The cyst was drained through an extended buttock incision, and healed without a site of bowel perforation ever identified. Meconium pseudocyst should be included in the differential diagnosis of sacrococcygeal and buttock tumors in the newborn.

Buttocks

High proximal pouch esophageal atresia with vertebral, rib, and sternal anomalies: an additional component to the VATER association.

The base of the proximal esophageal pouch in babies born with esophageal atresia varies in location from the low cervical region to the upper third of the mediastinum, but the relevance of this finding to the VATER association is not known. The initial radiographs and hospital records of 42 newborns with esophageal atresia and a distal tracheoesophageal fistula treated in the Newborn Special Care Unit of the Yale-New Haven Hospital between 1977 and 1986 were examined to (1) determine the location of the base of the upper esophageal pouch on its superimposed vertebral body; (2) identify coexisting VATER associated anomalies; and (3) detect the presence of vertebral, rib, or sternal anomalies. Ten of 11 babies with the upper esophageal pouch at the first thoracic vertebra or above had additional VATER defects including vertebral, rib, or sternal anomalies. These patients also required staged repair or circular myotomy for wide gap esophageal atresia and were at greater risk of anastomotic leak, stricture, or gastroesophageal reflux requiring fundoplication. High proximal pouch esophageal atresia accompanied by vertebral, rib, and sternal anomalies constitutes a heretofore unrecognized high risk group with the VATER association.

Abnormalities, Multiple