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Biomedical subjects

S Z Rubin

Publications and source records attributed to S Z Rubin.

34 records · Page 2Linked to original sources

Necrotizing enterocolitis: a chronic colonic disease.

While acute necrotizing enterocolitis is a common neonatal condition, the chronicity of the pathologic process is often overlooked. Between 1979 and 1983 at the Foothills Hospital in Calgary, a diagnosis of acute necrotizing enterocolitis was made in 211 infants. Seventeen infants (8%) required urgent surgical treatment in addition to standard medical management. Overall, 206 (98%) infants survived the acute episode. Long-term survival was 92% (194 children). The 12 late deaths were ascribed to chronic cardiovascular, pulmonary and cerebral diseases and "short gut syndrome". Colonic strictures developed in 9% of those treated medically; strictures distal to the mucous fistula developed in 50% of those requiring urgent resection and exteriorization. None of the strictures, which formed between 14 days and 18 months after the diagnosis was made, resolved spontaneously. They tended to be multiple and to involve longer segments in infants who required surgery. Diarrhea prolonged the morbidity in these children. The time course and varied presentation of strictures in this series suggests a rationale for management of infants who survive acute necrotizing enterocolitis.

Chronic Disease↗

Localized rigidity and narrowing of the antrum: a cause of gastric outlet obstruction in infancy.

In infants with persistent vomiting without bile staining, in whom congenital hypertrophic pyloric stenosis has been excluded, an upper gastrointestinal roentgenogram may show antral obstruction. Of four infants with partial gastric outlet obstruction described by the authors, an antral membrane was demonstrated radiologically in two. Other causes of antral obstruction, such as granulomatous disease, hour-glass deformity of the antrum, cholecystogastrocolic band and antral dysmotility, were considered and excluded as the cause of the vomiting. After medical management failed, gastroscopy revealed a rigid stenotic circumferential area in the antrum in all four infants. An antral membrane was not found. Laparotomy confirmed this finding and a pyloroplasty successfully resolved the symptoms. Localized rigidity and narrowing of the pyloric antrum, masquerading as an antral membrane radiologically, should be considered among the causes of gastric outlet obstruction in infancy. Treatment is determined by the severity of the symptoms. Pyloroplasty is successful when medical management fails.

Body Weight↗

Mitochondrial myopathy of cerebro-hepato-renal (Zellweger) syndrome.

The muscles of four infants with cerebro-hepato-renal (Zellweger) syndrome were studied during life and/or at necropsy. A mitochondrial myopathy was demonstrated, similar to mitochondrial alterations demonstrated in liver and brain in this disease. Muscle fibers with red-staining subsarcolemmal aggregates were identified with Gomori trichrome stain in two cases. Subsarcolemmal and intermyofibrillar zones of increased concentrations of NADH-TR, SDH, and cytochrome-c-oxidase activity were demonstrated histochemically in all four cases. Degenerative and cytoarchitectural changes in muscle fibers were not found. Ultrastructural studies showed large aggregates of mitochondria and increased lipid in the subsarcolemmal and intermyofibrillar spaces. Degenerative changes in mitochondria and lipid also were demonstrated, but paracrystalline inclusions were not seen. The distribution of these changes was not uniform between patients or between different muscles in the same patient. The diaphragm was affected more severely than proximal or distal muscles of the extremities. Direct involvement of muscle mitochondria in this disease may interfere with energy metabolism and contribute to the clinical findings of hypotonia, weakness, and respiratory insufficiency. The muscle biopsy with histochemistry and electron microscopy may be used as a diagnostic adjunct in suspected cases, but the variation encountered dictates dictates caution in the interpretation of negative findings.

Brain Diseases↗

Persistent fetal circulation in neonates postoperatively: the value of manual ventilation.

Three critically ill infants who had persistent fetal circulation postoperatively, refractile to tolazoline and 100% oxygen with mechanical ventilation including continuous positive airway pressure, responded favourably to prolonged manual hyperventilation. Two infants had undergone repair of a diaphragmatic hernia and the third had correction of a tracheoesophageal fistula. Normal respiratory function was documented in two survivors. The third died 14 days postoperatively. The use of this simple technique indicated that perseverance can be rewarded in the treatment of persistent fetal circulation unresponsive to the usual medical management.

Carbon Dioxide↗

Testicular venography as an accurate indicator of true cryptorchism.

It may be impossible to detect a testis on physical examination because of testicular agenesis, testicular atrophy or an intra-abdominal location. When the condition is bilateral, hormonal studies are useful. Inability to locate the testicle at operation is not synonymous with testicular absence. The presence of a testicular vein excludes gonadal agenesis. Visualization of the pampiniform plexus indicates a formed testicle and accurately locates its anatomic site. Four patients with unilateral impalpable testes underwent testicular venography. General anesthesia was used for the first patient only. There were no complications related to the investigation. All four patients underwent exploratory operations that confirmed the testicular venographic findings. Three patients had unilateral testicular dysgenesis or atrophy; one patient had an intra-abdominal undescended testis. Testicular venography is a safe, practical diagnostic investigation in boys with true cryptorchism.

Child↗

Carcinoid in a rectal duplication: a unique pediatric surgical problem.

Since 1935 at the Hospital for Sick Children in Toronto, 13 cases of carcinoid have been seen as incidental findings in the appendix, but carcinoids of the rectum have never previously been reported in childhood. The authors report a child with a rectal duplication in which this tumour was found. The rationale for local resection of the duplication with its contained carcinoid is understood in the light of the principles for treatment of intestinal carcinoids in general and carcinoids of the rectum in particular. Two years after operation the child is well without signs of local or systemic recurrence.

Adolescent↗

Gastroschisis: primary closure or Silon pouch.

There has been a substantial increase in the incidence of gastroschisis in the last few years, and total parenteral nutrition has enabled many of these neonates to survive the long post-operative course. From 1969 to 1976 inclusive, 44 neonates with gastroschisis were treated with either primary closure or the application of a Silon pouch. Fifteen neonates were managed by primary closure. Three of these neonates developed clinical sepsis, but no other intraabdominal complications ensued. There were two deaths (13%) in the early postoperative period, due to CNS hemorrhage and aspiration. This gorup of 15 babies had 17 abdominal operations and the survivors averaged 32 days of parenteral intravenous nutrition before complete oral alimentation could be successfully started. Two ventral hernias required late repair. Twenty-nine neonates were managed primarily with a Silon pouch. Eighteen of these newborns developed sepsis, three Silon sacs had to be removed because of necrotic bowel within, and two other infants developed small bowel fistulae. There were 12 deaths (35%). These 12 infants had prolonged intestinal malfunction that lasted an average of 67 days. This group of 29 babies had 64 abdominal operations and the survivors averaged 46 days of parenteral intravenous nutrition prior to full oral feeds. Four ventral hernias required later repair. The comparison of results in these two groups of newborns reveals a striking difference in their post-operative course and survival. Although the Silon sac has until now become the accepted surgical procedure for gastroschisis, these results conclusively indicate the advantage of primary reduction and closure of the abdominal wall defect.

Abdominal Muscles↗

The unusual presentation of pancreatitis in infancy.

Two infants with acute pancreatitis are described. It appears that acute pancreatitis in infancy presents in one of two ways: part of a severe generalized illness, or as ascites. The outlook for the former depends on the prognosis of the generalized illness. If pancreatitis can be recognized as the cause of the ascites, then adequate treatment will result in survival. Although the cause in some infants is said to be idiopathic, exploration should be performed, the lesser sac investigated and if the child is in reasonable condition, appropriate study of the biliary tree and pancreatic ductile system should be considered. If a pseudocyst is present, adequate drainage is mandatory. Early diagnosis and appropriate treatment will be possible only if this rare disorder is suspected. The eventual prognosis is excellent and the chance of recurrent attacks is minimal.

Acute Disease↗

Ureterocele--a familial congenital anomaly.

Nonidentical twins with ectopic ureteroceles, and duplication of the urinary collecting system in two more members of the same family are described. This incidence may indicate that inborn anomalies of the urinary tract may have a genetic background.

Diseases in Twins↗

A critical look at delayed intestinal motility in gastroschisis.

The motility of the gastrointestinal tract after the accepted management of gastroschisis by using a Silon pouch and gradually reducing the herniated intestine is compared with the intestinal motility after primary reduction and closure of the defect. Since 1970, 46 children with gastroschisis have been treated at the Hospital for Sick Children, Toronto. Twenty-seven were treated with a Silon pouch. Eleven died after an average postoperative survival of 17 weeks; not one had bowel motility that allowed oral feeding. Among the 16 survivors an average of 10 weeks elapsed between operation and the institution of full oral feeding. Seventeen children were treated by forceful stretching of the abdominal cavity, primary replacement of the eviscerated intestines and closure of the defect. Two died in the early postoperative period of conditions unrelated to the gastroschisis or to the procedure. The average period between operation and full oral intake of food was only 4 weeks. In addition, two neonates were admitted after primary closure was done at the referring hospital. Roentgenograms obtained postoperatively in 24 of the patients showed the "typical" dilated loops with air fluid levels in patients treated with a Silon pouch. The authors' findings indicate that primary reduction and closure of the defect has a distinct advantage over use of a Silon pouch in the treatment of gastroschisis.

Abdominal Muscles↗

Experience with 55 Silon pouches.

The application of a Silon pouch usually indicates a desperate if not impossible situation in which the abdominal contents cannot be returned to the abdominal cavity and therefore require some type of covering. Since 1968, we have had experience with 55 Silon pouches in infants and children. Almost all of these pouches were applied to newborns. There were 22 deaths in this series (40%), although 12 of these deaths were in the diaphragmatic hernia group and were almost all due to respiratory failure unrelated to the pouch itself. There were nine deaths in the gastroschisis and omphalocele group (28%) and in all these instances infection (especially Candida) was the underlying cause. While much debate exists as to how the Silon pouch should be handled after its application our series demonstrated that the longer it stayed in place the greater the chance of infection. Furthermore if broad spectrum antibiotics were used for a prolonged period of time, Candida overgrowth of the pouch followed by Candida sepsis and death were almost inevitable.

Abdomen↗

Use of clindamycin and gentamicin in pediatric colonic surgery.

The combination of clindamycin and gentamicin was used to treat 25 pediatric surgical patients. In 9 of the 11 patients with perforated appendicitis, B. fragilis was isolated from the peritoneal cavity. There was a rapid clinical response to treatment in all cases. No side effects were observed. Clindamycin and gentamicin seem to be the antibiotics of choice for such infections.

Appendicitis↗

Complications of the Grice-Green operation.

A case presentation involving complications resulting from errors in surgical technique with the Grice-Green procedure is discussed in this report. The patient was treated 18 years after bilateral tendo achillis lengthenings and Grice-Green extra-articular subtalar arthrodeses with autogenous tibial bone grafts. The complications encountered were severe adductovarus deformity of the feet with multiple painful plantar callosities, in-toed gait with weightbearing on the lateral aspect of the feet, inversion ankle instability, and tripping over the feet. Deformity and symptoms were worse on the right foot. The patient required further surgery, consisting of bilateral calcaneal osteotomy, metatarsal neck osteotomies, excision of callosities and ostectomies for painful plantar metatarsal base lesions, tenotomy of the abductor hallucis and digital fusions. The original surgery was performed for idiopathic flatfeet. Despite the many different causes of flatfoot for which the Grice-Green procedure has been employed, most authors believe it should not be used for idiopathic flatfeet, and restrict its use to the original indication, that of paralytic pes valgus secondary to poliomyelitis. The reader should also realize that there are very long-term consequences to surgery on the pediatric patient.

Adult↗