Sigmund H. Ein

Intussusception: 354 cases in 10 years

Abstract There are about 35 cases of intussusception treated annually at The Hospital for Sick Children, Toronto. Only cases of intussusception observed by barium enema examination or at operation were included in this 10-year study involving 336 patients. Three fourths of the intussusceptions occurred in children under 2 years. Males predominated in a ratio of 2:1. A peak monthly incidence was noted in May and June; this did not correlate with the hospital incidence of upper respiratory tract infection and/or gastroenteritis. The average duration of signs and symptoms prior to making the correct diagnosis was 24 hours. Pain was the commonest presenting symptom, along with vomiting, a palpable abdominal mass, and blood per rectum. Forty-five per cent of these children who were given barium enemas had their intussusceptions reduced. Attempted hydrostatic reduction was not carried out in 25 per cent of the cases, mainly because the children were too sick. Seventy per cent of the children were operated on. Most of the intussusceptions were of the ileocolic variety. Pathological lead points were not frequently recognized. Postoperative complications were more frequent in those cases where the duration of the presenting signs and symptoms were longer than the average. Five cases of postoperative intussusception were recognized. The total recurrence rate was 4 per cent. Although the incidence of recurrent intussusception after barium enema reduction was 11 per cent, only 3 per cent recurred after operative manual reduction. The mortality in our series was zero.

Timing of surgery for congenital diaphragmatic hernia: Is emergency operation necessary?

Congenital diaphragmatic hernia (CDH) is considered by most researchers to be a surgical emergency. However, early repair does not necessarily improve respiratory function or reverse fetal circulation, and many patients deteriorate postoperatively. As a result, in 1985, we began to employ a protocol in which surgery was delayed until the PCO2 was maintained below 40 and the child was hemodynamically stable; children in whom these criteria could not be achieved died without surgical repair. Sixty-one consecutive infants with CDH were managed over 4 years; 31 from 1983 to 1984 (group 1) and 30 from 1985 to 1986 (group 2). The groups were similar with respect to sex, side of the defect, birth weight, gestational age, incidence of pneumothorax, and blood gases. High frequency oscillation was used with increasing frequency during the study period, for patients with refractory hypercarbia (13% in group 1, 30% in group 2). All patients were initially paralyzed and ventilated. Mean time from admission to surgery was 4.1 hours in group 1 and 24.4 hours in group 2 (P less than .05). In group 1, 87% of patients had surgical repair (77% within eight hours of admission, 10% after eight hours), and in group 2 only 70% of patients had surgery (10% within eight hours, 60% after eight hours). All patients who were not operated on died. Overall mortality was 58% in group 1 and 50% in group 2; this difference was not statistically significant. These data indicate that our current approach has not increased overall mortality.(ABSTRACT TRUNCATED AT 250 WORDS)

Nonoperative management of traumatized spleen in children: How and why

In the absence of the apleen, overwhelming infection is a real threat. Therefore, there is a trend to try to preserve all spleens. Recognizing this, and realizing that not every damaged spleen must be removed, a nonoperative approach has been advocated and practiced for 35 yr at the Hospital for Sick Children in Toronto. This experience is recorded herein, providing indications and methods of management. The results are considered to be very satisfactory.

Leading points in childhood intussusception

Children whose intussusception is caused by a specific pathologic lesion are harder to diagnose and have a higher morbidity than those with the idiopathic variety. We have collected and analyzed 31 such cases found in a series of over 500 intussusceptions. The average age of these children was greater than is usually found in most cases, and the duration of the signs and symptoms was also longer than is usually seen. Almost 50% presented with a picture of advanced small bowel obstruction. Fewer barium enemas were done (50%) and none was successful in reducing the intussusception. There was a higher number of ileo-ideal intussusceptions in this group. The commonest leading points were Meckels diverticula, polyps, and duplications. All patients with leading points required operation; three-fourths had a bowel resection performed. This study of 569 cases suggests that older children with intussusception and children with recurrent intussusception do not necessarily have leading points causing their intussusceptions.

Lipoblastoma in infants and children.

Lipoblastoma and lipoblastomatosis are rare benign tumors of embryonal fat with a tendency to local invasion but not to metastasize. To date, there have been 60 cases described in the international literature. There has never been a report in a child older than 8 yr. The main clinical characteristics are the presentation, usually below 3 yr of age, the rapid growth of the mass and the peripheral location, mainly in the extremities. Although CT scan may show a fatty tumor, there is no single test to make the differential diagnosis (which includes benign lipoma, liposarcoma and myxoliposarcoma) and the treatment should be based on clinical findings. We have treated four patients with this condition. Two were intrathoracic, one was intraperitoneal, and one was a tumor of the upper arm. All presented as rapidly-growing tumors and were clinically assumed to be malignant. We recommend complete but conservative excision of the tumor; there are reported recurrences after inadequate excision. This is a benign tumor and radical cancer surgery should be avoided.

Ruptured spleen—When to operate?

Sixty-three patients with splenic injuries were treated during a 5-yr period from 1974-1979. The decision to operate was based on the patients clinical course, not on the presence of splenic injury alone. Those who were stable on admission or after initial resuscitation were treated nonoperatively. This consisted of strict bed rest, nasogastric suction, and i.v. fluids--including blood--as required. Those who bled massively were operated on promptly. At operation, the spleen was repaired if possible or excised if damaged beyond repair. Forty patients were treated nonoperatively. Sixteen of these required blood transfusions (mean 31.2 +/- 5.3 ml/kg). One patient in this group developed a large defect on spleen scan at 3 wk post injury. There was no other morbidity and no mortality following nonoperative treatment. Nineteen required operation all within 16 hr of admission. Fifteen underwent splenectomy, 2 partial splenectomy, and 1 splenorrhaphy. In 1 the bleeding had stopped. All required blood before operation (mean 80.4 +/- 10.1 ml/kg). Seven in this group died (6 from head injuries and 1 from bleeding). Thus surgery was avoided in 2 out of 3 and the spleen saved in 3 out of 4 patients with documented splenic injuries. We believe that where adequate facilities exist nonoperative treatment of splenic injuries is both safe and effective. When bleeding is massive from the beginning or replacement requirements exceed 40 ml/kg, operation is indicated.

The late-presenting pediatric bochdalek hernia: A 20-year review

A 20-year retrospective study was made of children with congenital posterolateral (Bochdalek) hernias presenting more than 8 weeks after birth. The records of 26 patients (16 boys and 10 girls) were evaluated. Sixteen infants and children (62%) were originally misdiagnosed clinically and radiologically as having either infective lung changes, congenital lung cysts, or pneumothoraces; inappropriate thoracentesis occurred in four patients misdiagnosed as having a pneumothorax. Five patients had previously normal chest radiographs. The most useful investigation was a plain radiograph following passage of a nasogastric tube. Coexisting abnormalities (in particular, gut malfixation and malrotation) were common. All patients except one were operated on within days of presentation, and as emergencies if symptoms were acute. More than one third of our patients were left with a smaller than normal ipsilateral lung after their diaphragmatic hernia repair, and these lungs must be considered hypoplastic to some degree. Chest tubes made no difference in the lungs eventual expansion. Two deaths occurred as a result of acute cardiorespiratory arrest in previously well children. Therefore, the symptoms, signs, and radiologic findings of patients with diaphragmatic hernias presenting after the neonatal period may be difficult to interpret, and may result in diagnostic delay, misguided therapy, and a potentially fatal outcome.

A 13-year experience with peritoneal drainage under local anesthesia for necrotizing enterocolitis perforation

Between 1974 and 1986, inclusive, over 400 newborns with clinical, radiological, and/or pathological evidence of necrotizing enterocolitis (NEC) were treated at the Hospital for Sick Children, Toronto, Ontario. Within this group were 37 babies who had a bowel perforation that was treated with peritoneal drainage under local anesthesia. Eighty-eight percent of the 41 weighed less than 1,500 g and 65% weighed less than 1,000 g; during the same time 40 other neonates (9% of the total) with perforated NEC had laparotomies. Twelve neonates (32%) required only drainage with complete recovery of their intestinal tracts. The remaining 25 (68%) fell into one of three groups: (1) nine (24%) had rapid downhill course, sepsis, and death without laparotomy; (2) nine (24%) had rapid downhill course, sepsis, and laparotomy (five deaths); (3) seven (20%) had slow development of bowel obstruction requiring operation (two deaths). The overall survival rate was 56%. These results continue to indicate that this method is effective in temporizing 88% of the small and/or very ill babies with a NEC perforation. However, an added bonus is that 32% of these newborns treated in this fashion had complete resolution of their disease.

Congenital tracheal stenosis: A review of 22 patients from 1965 to 1987

Twenty-two infants with congenital tracheal stenosis (CTS) were treated in our hospital between 1965 and 1987. Diagnosis was confirmed by endoscopic and radiographic methods. Patients had a spectrum of tracheobronchial lesions from localized stenosis to more complex deformities involving the carina and bronchi. Other anomalies were found in all patients with the occurrence of vascular slings or rings in 11 patients (50%). Six infants treated nonoperatively died from severe CTS and other lethal anomalies. Five of 16 patients (31%) treated surgically survived. Localized CTS in four cases was treated by dilatation, tracheostomy, or tracheal resection with primary anastomosis (two survivors, 2 non-survivors). Funnel-shaped deformities and extensive tracheobroncial stenosis were treated by tracheal reconstruction using a variety of autogenous tissue and prosthetic grafts (three survivors, nine non-survivors). The overall mortality was 77%. A new intratracheal stent was used in two patients. The stent was a flexible steel spring covered with a silicone rubber sheath. In one patient, it was placed within the trachea at the time of repair and removed later with the bronchoscope. A stent was used in a second patient with intermittent airway obstruction following an esophageal tracheoplasty. In this case, the device failed to alleviate the obstruction, and the infant expired from progressive respiratory failure. Issues of importance in the management of infants with CTS are: (1) adequate evaluation of the tracheobronchial tree, (2) awareness that tracheobronchography may precipitate further respiratory decompensation, (3) assessment of vascular and other anomalies requiring surgical correction, and (4) selection of an appropriate therapeutic approach.

Esophageal atresia with distal tracheoesophageal fistula: Associated anomalies and prognosis in the 1980s*

Ninety-seven newborns with esophageal atresia and distal tracheoesophageal fistula (EA-TEF) were treated between 1979 and 1985 inclusive; there were 54 boys and 43 girls. Their weights ranged from 800 to 4,000 g (average, 2.5 kg). They included: 28 neonates with cardiac defects (most common: patent ductus arteriosus [PDA], ventricular septal defect and atrial septal defect [VSD-ASD]), of whom 18 survived (64%); 17 babies with other gastrointestinal anomalies (imperforate anus, duodenal atresia), of whom 12 survived (70%); 12 patients with skeletal malformations (digital, vertebral), of whom 11 survived (91%); 8 newborns with genitourinary abnormalities (hypospadias, undescended testis), of whom 6 survived (75%); and 16 infants with other congenital lesions (trisomy 18, lung agenesis-hypoplasia), of whom 3 survived (18%). Forty-six infants (average, 2.7 kg) had no other anomalies and all survived. As the number of systems with defects increased, both the weight of the baby and survival rate decreased. From this entire series of 97 newborns with EA-TEF, 81 (83%) survived (average, 2.3 kg). Sixteen babies died (average, 1.9 kg); 11 had defects incompatible with life. Eleven of the 16 were never operated on (seven patients with trisomy died within 5 days, and four patients with complex cardiac defects died within 3 weeks). Four of the 16 who were operated on died between 3 months and 2 years from chest problems and one newborn died in the operating room. We concluded that (1) newborns who have the common type of EA-TEF will almost certainly survive if there are no other anomalies; (2) the most frequently associated congenital defects are cardiovascular (28%), gastrointestinal (17%), skeletal (12%), and genitourinary (8%); (3) as the number of systems with defects increases, the weight of the baby and its survival rate fall; and (4) 11% will have trisomy and/or complex cardiac defects with no survival.