Challenges and dilemmas for a pediatric surgeon.
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Child↗
Biomedical subjects
S Cywes
Publications and source records attributed to S Cywes.
Long-term clinical, manometric, and histological evaluation of obstructive symptoms in the postoperative Hirschsprung's patient.
One hundred seventy-eight of 326 patients had surgery for Hirschsprung's disease at Red Cross Children's Hospital (1957 to 1990) agreed to participate in a recall follow-up study. Assessment of the postoperative outcome of the Swenson, Duhamel, and Soave procedures included manometric evaluation in 43 patients by perfused-tube and balloon-series techniques. Rectal suction biopsies were performed for patients who had persistent problems with stool evaluation. One hundred fifteen of the 178 patients were more than 4 years of age, and long-term functional outcome could be assessed. Although good results were obtained in 94% overall, 16 had clinical evidence of a degree of persisting obstruction. Results of manometric assessment of anorectal function in these patients were not significantly different from those of 28 patients who had normal stool evacuation. A biopsy was performed in 14 of the 16 patients who had symptoms of obstruction postoperatively, and abnormal histological features were noted. There was aganglionosis in four, features of neuronal intestinal dysplasia in nine, ganglioneuromatosis of the colon in one. The results of two biopsies were entirely normal. Implications of postoperative dysfunction after surgery for Hirschsprung's disease are discussed, and a protocol for investigation and management is proposed.
Child, Preschool↗
Potential danger of 'trial of life' approach to congenital diaphragmatic hernia.
This is the report of a newborn girl who was treated by "trial of life" before surgical repair of her left-sided diaphragmatic hernia. Surgery was performed 60 hours after birth, at which time she was found to have an organo-axial volvulus of the stomach. Her postoperative course was complicated by a jejunal perforation on the fourth postoperative day, necessitating another laparotomy.
Combined Modality Therapy↗
Fenestrated duodenal membranes: an analysis of symptoms, signs, diagnosis, and treatment.
Duodenal obstruction typically occurs in the ampullary region and presents in the early neonatal period. If the obstruction is incomplete, as with a fenestrated duodenal membrane, the presentation may be delayed and the diagnosis overlooked. Sixteen patients with fenestrated duodenal membranes presented over a 23-year period. Six presented in the neonatal period, with vomiting; an abdominal x-ray was diagnostic in four. Ten presented later (5 weeks to 14 years) with nonspecific symptoms, ie, failure to thrive, postprandial epigastric distension, and recurrent chest infections. Vomiting occurred in all, but was of short duration (< 1 week). Two patients in the delayed group had Down's syndrome. Diagnosis was confirmed on barium meal, and optimal surgical treatment was a bypass duodeno-duodenostomy. The diagnosis of fenestrated duodenal membrane must be considered in all cases with symptoms and signs suggestive of upper gastrointestinal tract obstruction, and excluded by contrast radiology.
Adolescent↗
Trans-axillary transpleural sympathectomy for Palmar hyperhidrosis in children--a 3 to 7 year follow-up of 9 cases.
Primary palmar hyperhidrosis can be a most unpleasant and distressing affliction. When normal daily activities, viz. writing, schoolwork, are interfered with and other treatments have failed, surgery is indicated. Between 1983-1987 9 children (6 F, 3 M), mean age 10.2 years (range 5-14 years) underwent bilateral transaxillary sympathectomy, 4 simultaneous and 5 1-4 weeks apart. On 14 sides a standard excision of dorsal ganglia (DG) 2, 3, and 4 was performed. The technique of lateral displacement of the sympathetic chain after transection distal to DG 4 and division of preganglionic fibres of DG 4, 3 and 2 was used on 5 sides. In addition to clinical evaluation a pilocarpine stimulation test was performed on the palms of the hands before and at follow-up 3-7 years after surgery. 17/18 hands appeared sympathectomised at early follow-up. One inadequate result required reoperation. Other complications included 2 Horner's syndromes--1 transient and 1 mild but permanent; 3 temporary intercostobrachial paraesthesias, 2 mild late recurrences of sweating and 2 compensatory increases in sweating. In 14 palms where sweat volume was measured before and 3-7 years after surgery there was a mean decrease in sweat of 84% (mg), those sweating the most prior to surgery having the best response. In 2 further palms insufficient sweat was obtained for testing. All but 1 considered the procedure worthwhile. Transaxillary transpleural sympathectomy is a safe, effective, and cosmetically acceptable operation to control symptoms of excessive palmar sweating.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
A histological grading system for the evaluation of co-existing NID with Hirschsprung's disease.
The significance of dysplastic features in the surgical pullthrough segment of bowel in patients with Hirschsprung's disease (HD) has not yet been clarified. The aim of this study was to evaluate prospectively the ganglionated proximal bowel in 26 patients with HD (January 1988 through January 1991). The significance of dysplastic features and their influence on post operative outcome were evaluated by means of a newly devised histological scoring system based on the morphological features. Functional outcome was assessed clinically at follow-up interview. Comparison was with control specimens from 22 patients undergoing unrelated bowel surgery and a further 5 patients with neuronal intestinal dysplasia (NID). Results indicated a wide spectrum of histologically identified dysplastic features in patients with NID, the ganglionated bowel of HD and controls. Although individual abnormal features were noted in the control group, significant degrees of dysplasia were absent. The overall degree of dysplasia was less striking than that observed in NID and in the 5 patients in whom NID co-existed with HD. Dysplasia of the ENS in residual bowel could be correlated with postoperative dysfunction in 4 out of 5 patients (80%) with HD and features of co-existing NID. In addition, milder symptoms were noted in 50% of patients having a borderline score (5-6/12). This study emphasizes the relationship between clinical obstructive symptoms and a high degree of dysplasia within the ENS. A histological grading system is of value in evaluating the spectrum of abnormal findings and prospectively identifying those with functional significance in patients with NID co-existing with HD.
Acetylcholinesterase↗
Heterogeneity and low detection rate of RET mutations in Hirschsprung disease.
Mutations in some exons of the RET proto-oncogene were recently observed in Hirschsprung patients. Using DNA polymorphisms and single-strand conformation polymorphism analysis for the whole coding sequence of the RET proto-oncogene, 82 unrelated Hirschsprung patients were screened systematically. A total of 4 complete deletions of RET and 12 point mutations were identified, each present in no more than one patient and distributed along the whole gene. De novo mutations could be documented in 4 patients. Southern blot and fluorescence in situ hybridization analysis carried out in a restricted number of patients did not reveal any deletion of RET. The low efficiency in detecting mutations of RET in Hirschsprung patients (20%) may originate mainly from genetic heterogeneity.
Blotting, Southern↗
Ambulatory paediatric surgery. The development of a day-care surgical centre.
Ambulatory surgery has become an important component in the provision of surgical care. In 1987 a day-case surgical unit was established at Red Cross War Memorial Children's Hospital, Cape Town. Experience over a 6-year period (1987-1992) is reviewed in relation to the nature and total number of surgical procedures, pre-operative preparation, daily utilisation of the facility, postoperative care and complications. During this period 16,538 patients (mean age 3 years) were operated on in the unit. Nine surgical disciplines participated. Pre-operative assessment and preparation reduced the rate of cancellations on the day of surgery to less than 6%, with a 90% effective use of schedules operating time. Scarce nursing resources were also maximally utilised. Most procedures took less than 30 minutes and only 0.8% exceeded 1 hour. The average ward stay was 6 hours. No major complications were encountered and acceptance by patients and parents was excellent. Only 16 children who were not scheduled to do so needed to stay in hospital overnight. Recommendations to establish and improve day-stay surgical services are presented. It is concluded that a practical, efficient service can be established and that the authorities should be encouraged to expand appropriate facilities.
Ambulatory Surgical Procedures↗
Left-sided liver abscess in childhood.
Abscesses occur less frequently on the left side of the liver than on the right, and are implicated in a higher incidence of complications. Of a total of 124 children with liver abscesses seen at Red Cross War Memorial Children's Hospital from 1974 to 1990, 26 had left-sided and 98 right-sided lesions. A similar spectrum of organisms was cultured, and 20 (77%) of the left-sided abscesses were found to be caused by pyogenic organisms as opposed to 78 (79%) of those on the right. The remainder were of amoebic origin. In 5 patients with multiple abscesses involving predominantly the left side of the liver, the right side was also involved. These were excluded from further comparison of left and right liver abscesses. Of the remainder, 16 (62%) left-sided and 77 (83%) right-sided abscesses were solitary. Clinical features were similar in both groups but epigastric mass was more frequent in left-sided lesions. After an initially conservative management policy, surgical drainage was necessary in 87.5% of solitary left-sided liver abscesses as opposed to 64% of solitary right-sided abscesses. No intrapericardial ruptures were noted, but 2 pericardial effusions required drainage. There was no mortality but 3 patients with solitary left-sided abscesses ruptured. The important role of ultrasound in the diagnosis and follow-up period is stressed. Patients with solitary left-sided abscesses are identified as being at risk. Abscess drainage is recommended in this group.
Child↗
Secondary effects of prolonged intestinal obstruction on the enteric nervous system in the rat.
Motility disturbances following prolonged intestinal obstruction have been attributed to secondary effects. This study aimed to demonstrate the effects of incomplete obstruction on the enteric nervous system (ENS) of a rat model. Surgical placement of a nonstrangulating ligature encircling the distal bowel was performed in 41 freshly weaned rats. Anesthetic protocol included Ketamine, ether, or Xylazine (an alpha 2-adrenergic agonist). Histological evaluation was by ganglion cell morphology, histochemical staining for acetylcholinesterase (AChE) and tyrosine hydroxylase (TOH) immunocytochemistry. Forty-one freshly weaned LE rats were divided into controls (8), sham procedures (8), intestinal obstruction (16), and a group of rats with colonic biopsy performed prior to and following experimental obstruction (9). The rats were sacrificed at periods varying between 14 and 45 days post experimental obstruction (median survival, 27 days). Histological changes included elongation of ganglion cells and a decrease in the number per 5-mm slide in obstructed animals. No other obstruction specific differences were detected. A significant (P < .01) increase in AChE in the submucous plexus was recorded in Xylazine-anesthetized animals. No obstruction-specific effects could be demonstrated in the ENS, suggesting that prolonged obstruction without ischemia does not result in any significant alterations in the ENS. Pharmacological stimulation of the alpha 2-adrenergic receptor appeared to result in an increase in AChE. This mechanism may help to explain a possible role for the adrenergic system in the increased AChE levels in affected bowel in patients with Hirschsprung's disease.
Acetylcholinesterase↗
Bowel stomas in infants and children. A 5-year audit of 203 patients.
A 5-year audit of the formation, management and closure of small-bowel stomas and colostomies at Red Cross War Memorial Children's Hospital, Cape Town, is described. Of the 203 patients, 128 (63%) required the stroma in the neonatal period. Anorectal malformations (80), Hirschsprung's disease (65), necrotising enterocolitis (33), trauma (11) and neoplasm (5) comprised most of the indications. One hundred and thirty large-bowel stomas were sited in the proximal sigmoid and 37 in the transverse colon, all but 11 being divided with each end brought out through a short muscle cutting incision or through the laparotomy wound. Thirty-six ileostomies were performed and in 30 of these the stoma was sited in the wound. Complications, which included necrosis, bleeding, prolapse and wound sepsis, occurred in 31%. The colostomies sited in the transverse colon had the highest incidence of prolapse (38%). Neonatal stomas brought out in the wound had an acceptably low incidence of complications. Most stomas were temporary in nature. One hundred and eighty-eight were closed, all with excision and end-to-end intraperitoneal anastomosis. There was a 4% incidence of complications (5 would sepsis, 2 leaks, 1 stenosis). The formation, management and closure of bowel stomas represents a considerable section of the work of the paediatric surgeon. Correct meticulous technique is essential in keeping complications to a minimum.
Colostomy↗
Degenerative leiomyopathy in African children: a review of current perspectives.
The syndrome of degenerative leiomyopathy causing intestinal obstruction clinically manifests in young indigenous African children as massive megacolon without aganglionosis. Eighteen children have been seen over a 16 year period from a localized geographic area. There were 10 males and 8 females having a mean age of 9.5 years at presentation and a mean duration of symptoms of 4.3 years. The youngest was 6 months old. All had progressive abdominal distension and infrequent stooling but 11 had intermittent diarrhoea and 9 had colicky abdominal pain. Gross gaseous distension of the large bowel with extension into small intestine occurred in 9 and this extended into stomach and oesophagus in 4. Biopsy of the dilated, thin walled bowel showed smooth muscle degeneration and necrosis with replacement by fibrous tissue. Neuronal cells of Auerbach's plexus tend to be displaced into the circular layer of smooth muscle with mild inflammatory changes. Some small arteries show medial fibrosis with subintimal fibroblastic proliferation. Acetylcholinesterase and immunohistochemical staining with neural and muscle markers are within normal limits. One child died while 14 have been maintained on prokinetic agents, low residue diets, laxatives and enemas. Nine children have required surgical intervention of whom 4 had volvulus and 3 adhesive bowel obstruction. Degenerative leiomyopathy is a distinctive entity with classical clinical and histological features. The aetiology is still obscure.
Biopsy↗
Liver transplantation for children in South Africa.
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Adolescent↗
Paediatric surgery in the RSA--practice and training.
A survey of the current practice of general paediatric surgery in South Africa and Namibia was conducted by means of a postal questionnaire. One hundred and eighty-one replies were obtained (52%). Analysis showed that although surgeons are located predominantly in the major cities, 18% practised in the larger towns or rural areas. Although paediatric surgery represents a small volume of their work, most surgeons do attend to the surgical needs of children. The majority of surgeons have received some paediatric surgical training but this was considered inadequate by nearly half of all surgeons and by 60% of those who qualified in the last 10 years. Current postgraduate training at most of our universities involves a 3-6-month rotation, but from our survey this is considered insufficient in practice and there appears to have been some deterioration in paediatric surgical training in recent years. Steps needed to maintain paediatric surgical standards are discussed.
Child↗
Gastrointestinal tract perforation in children due to blunt abdominal trauma.
Over a 14-year period 587 children under 13 years of age were admitted with blunt injury to the abdomen. Twenty-nine (4.9 per cent) of these were found to have bowel rupture. Evidence of peritonitis was present at initial evaluation in 11 children (38 per cent). Radiological evidence of perforation (pneumoperitoneum) was present in only five of 27 (19 per cent) with a further six of 27 (22 per cent) showing dilated loops of bowel or fluid levels. Thus 59 per cent of radiographs were not diagnostic. The mean time from admission to laparotomy was 17 h. Proximal bowel perforation was common and perforation at multiple sites occurred in five patients; 59 per cent had a concomitant injury which resulted in two deaths (from head injury). Initial clinical and radiological evidence of bowel perforation can be misleading and reliance on such indicators may result in significant diagnostic delay. Frequently repeated clinical examination is advocated; progression of abdominal signs should alert the clinician to proceed to laparotomy.
Abdominal Injuries↗
Reflux strictures of the esophagus in children.
Although the therapeutic approach to gastroesophageal reflux in children is well established, there are differences of opinion regarding the management of esophageal strictures, viz bougienage with medical therapy, fundoplication without dilatation, preoperative dilatation followed by fundoplication with intraoperative and postoperative dilatation, or resection and interposition. Sixteen consecutive children (mean age, 30.2 months) with reflux strictures were evaluated, constituting 12% of children operated on for gastroesophageal reflux. The strictures became clinically apparent 22.4 months (mean) from the onset of symptoms and were diagnosed by contrast studies and endoscopy. At first endoscopy all the patients had well-established fibrotic strictures. The strictures were mostly situated in the middle or lower esophagus and 7 were longer than 3 cm in length. All 16 were treated with antacids, H2-receptor blockers (Cimetidine), prokinetic agents, and intense nutritional resuscitation, together with preoperative stricture dilatations (average, 3.6 times). This was followed by fundoplication when nutritional parameters had been restored, esophagitis improved, and the strictures dilated to adequate size. Seven children required concomitant gastrostomies for prograde esophageal dilatations. Twelve children needed postoperative esophageal dilatations. The results were satisfactory in 14 (88%). Two required endoesophageal resection for localized unyielding strictures. One child responded only after failed reflux surgery was corrected at a second procedure. During an average follow-up of 8.2 years (range, 3 to 11) there has been no stricture recurrence and growth velocity was restored in all. We conclude that our preferred method is preoperative in-hospital management of gastroesophageal reflux with maximum nutritional support and careful evaluation of the degree and extent of esophagitis and fibrous scarring.(ABSTRACT TRUNCATED AT 250 WORDS)
Child, Preschool↗
Colorectal carcinoma in children.
An increasing incidence of colorectal carcinoma has been noted at this institution. We report seven children with colorectal carcinoma. The average delay between onset of symptoms and diagnosis was 41/2 months, and in five patients distant metastases were present at the first operation. Initial symptoms were ignored in all cases and in only one was the serious nature of the condition realized at first presentation. In five lesions the histology was mucin secreting adenocarcinoma, a poor prognostic variant. All seven died on average 11 months after diagnosis. These three factors--delay in diagnosis, advanced stage of disease, and poorly differentiated histology--contribute overall to a poor prognosis in the young.
Adenocarcinoma↗
Is tube cecostomy safe in the surgery of Hirschsprung's disease?
The use of concomitant tube cecostomy remains an option for decompression after distal large-bowel surgery but has been criticised because of a reported high complication rate. Two hundred and three patients who underwent a Soave endorectal pull-through procedure for Hirschsprung's disease (1974-1990) were evaluated. Operative technique included a modified sutured Soave endorectal pull-through procedure and a Stamm tube cecostomy utilising a large-lumen catheter. One hundred and twenty patients who had a modified two-stage procedure plus concomitant tube cecostomy were compared with 83 patients who underwent a three-stage procedure with colostomy cover. Early postoperative complications occurred in 8 patients (6.6%) in whom cecostomies were performed. A distal cuff abscess developed in 1 patient (0.8%), an anastomotic leak in 1 (0.8%) and 3 patients (2.5%) had colo-anal stenosis. The cecostomy tube dislodged in 2 patients and 1 required operative closure of a faecal fistula at the cecostomy site. Colonic venting was adequate and little nursing care was required. In 2 instances colonic distension developed after cecostomy clamping. Decompression was achieved by opening the cecostomy tube; this resulted in relief of symptoms and a good subsequent recovery. In contrast, there were 11 postoperative complications in the 83 patients undergoing a three-stage procedure (13.2%). In 2 patients (2.4%) an anastomotic leak occurred and 5 distal cuff abscesses (6%), 3 (3.6%) early strictures and 1 (1.2%) neorectal retraction developed. The use of a concomitant tube cecostomy with a two-stage Soave procedure is an effective and safe means of providing proximal colonic venting and did not add to mortality or morbidity.(ABSTRACT TRUNCATED AT 250 WORDS)
Cecostomy↗