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Biomedical subjects

S Cywes

Publications and source records attributed to S Cywes.

At least 19 recordsLinked to original sources

Oesophageal replacement in the management of corrosive strictures: when is surgery indicated?

The accidental ingestion of corrosive agents is a major cause of oesophageal strictures in children. The mainstay of treatment is repeated dilatations. Despite this, a significant number of patients eventually require oesophageal bypass. We reviewed the records of all cases managed with this condition at the University of Cape Town teaching hospitals between 1976 and 1994. Dilatation therapy alone was successful in 14 out of 39 patients (41%). Morbidity of failed dilatation therapy included repeated hospital admissions over an average 11.5 months and 17 dilatations each. Oesophageal perforations occurred in 7 cases (18%). Early factors predictive of failure of conservative treatment were: delay in presentation of more than 1 month; severe pharyngo-oesophageal burns requiring a tracheostomy; oesophageal perforation; and a stricture longer than 5 cm on radiological assessment. The size of dilators accepted during early bougienage also correlated with eventual outcome. These criteria may be useful in predicting which patients will not respond to repeated dilatations. Early surgical intervention in such cases will prevent fruitless dilatations and related complications.

Acids

Conjoined twins--the Cape Town experience.

This paper records our experience in the management of 25 sets of conjoined twins seen over a 32 year period (1964 - 1996). The twins were classified into 14 complete and symmetrical sets and 11 incomplete or heteropagus. The 14 symmetrical sets included 9 thoracopagus, 2 ischiopagus, 1 craniopagus and 1 omphalopagus twins. In the incomplete heteropagus group there was 1 ischiopagus, one twin being anencepahlic, 2 dipygus, 5 parasitic, 2 fetus-in-fetu and I cranial and caudal. The management is detailed case by case. Overall 10 of 14 symmetrical sets underwent attempts at separation with 16 surviving the procedure, but there were 3 late deaths. In the incomplete group 10 of 11 were operated on with 9 survivors. The importance of a multi-disciplinary approach, the extensive investigations required pre-operatively to define areas of organ and bony conjunction, congenital anomalies of each twin and surgical teamwork is emphasized. Specific problems encountered were identified. In thoracopagus twins the hearts were of paramount importance as conjuction was usually fatal, being associated with major congenital defects. The greater the extent of thoracic cage fusion the greater the chance of associated severe anomaly. Skin expansion to assist coverage of the defects after separation was of great assistance, as was the use of collagen coated vicryl. Evaluation of the liver and pancreatico-biliary systems with isotope excretion scanning was crucial to pre-operative planning. Where there was fusion of the duodenum a single pancreatico-biliary system could be expected and prior strategies for separation and Roux-en-Y enteric drainage of both pancreatic and biliary secretion should be planned. Gastro-oesophageal reflux led to considerable morbidity in both twins of a thoraco-omphalopagus set. In ischiopagus and dipygus conjoined twins bilateral posterior iliac osteotomies were an essential component to anatomic reconstruction of the pelvic ring and wound closure. Also in this group, due to the frequency and extent of shared genital, urinary and ano-rectal structures, long-term morbidity was expected and a component of this might be due to spinal cord tethering, or as in one of our cases, a progressive hydrosyringomyelia. Timing of separation was ideally set at between 5 and 9 months with 6 to 8 weeks of prior tissue expansion but earlier operation was frequently required because of cardio-respiratory problems or organ failure in one twin. In most cases the goal of obtaining separate, independent and intact individuals was achieved.

Abnormalities, Multiple

The value of laparoscopy for impalpable testes.

Impalpable testes constitute up to 20% of cases of undescended testes and present both a diagnostic and a therapeutic problem. Knowledge of testicular site prior to orchidopexy is useful in planning surgical strategy. Misdirected blind inguinal exploration can be avoided. Many imaging techniques are time-consuming and expensive and can neither confidently locate an impalpable testis nor exclude its absence. Recently laparoscopy has been advocated as a safe and efficient method of identifying presence and site of an impalpable testis. The first stage of a divided-vessel orchidopexy may also be performed simultaneously. Fifty children with 60 impalpable testes--40 unilateral and 10 bilateral--underwent laparoscopic examination prior to groin exploration. In 5 (8%) an inguinal testis was found which had been missed on pre-operative palpation. The testis was absent in 23 (38%), of whom 13 had a limited negative groin exploration because cord structures were seen entering the internal ring. In 30 (50%) an intra-abdominal testis was identified; 14 were relocated into the scrotum with a standard orchidopexy, 16 required a divided-vessel orchidopexy. In 2 (4%) intra-abdominal testicular remnants were removed. There was no operative morbidity. We conclude that laparoscopy is a useful technique in the management of children with impalpable testes. Open perumbilical insertion of the trochar is a safe method of introducing the telescope into the peritoneal cavity. In addition to accurately identifying a testis in 60%, 40% were spared the pain and morbidity of an extensive groin exploration.

Child

Delayed presentation of congenital diaphragmatic hernia.

Patients with congenital diaphragmatic hernias (CDH) usually present soon after birth with respiratory distress. Occasionally presentation is delayed. Twelve patients with delayed presentation (outside the neonatal period) of a total of 91 with CHD were seen over 17 years (1977-1994). Case records were reviewed to identify reasons for late presentation, evidence of morbidity, treatment and outcome. Age at presentation ranged from 6 weeks to 30 months (mean 8 months). Ten patients were aged 6 months or younger and there was an equal number of boys and girls. Seven cases were left-sided, 4 right-sided and 1 was anteriorly placed and bilateral. Ten patients presented acutely, 5 with small-bowel obstruction and 5 with respiratory distress, but all of the latter had a history of previous recurrent chest infection. Diagnosis was confirmed by radiographic examination of the chest in 10, barium meal in 1 and a 5-month-old patient had an inappropriate laparotomy and gastrojejunostomy for duodenal obstruction at an outlying hospital. Two had documented normal chest radiographs 3 and 6 months prior to diagnosis. Patients who presented with gastro-intestinal symptoms were older (mean age 1 year) compared with those with predominantly respiratory symptoms (mean age 19 weeks). None had any evidence of bowel strangulation. All were repaired via an abdominal approach. A hernial sac was found in half the cases (4/7 left and 2/5 right). Five had non-rotation of bowel. Primary repair without patch was carried out in all. One child with lung hypoplasia and multiple cardiac abnormalities died; the others recovered well. Delay in presentation of CDH is not uncommon (14% in this series). Most patients will have respiratory symptoms. A normal previous chest radiograph does not exclude the diagnosis. All patients with recurrent chest infection should have a contrast meal investigation early on. Long-term prognosis is favourable and postoperative morbidity is minimal, despite late presentation.

Child, Preschool

A 25-year review of the acute scrotum in children.

Controversy persists with regard to the diagnosis and treatment of the acute scrotum in children. The differential diagnosis includes torsion of the testis, torsion of one of the appendices testis and epididymo-orchitis. Clinical differentiation is notoriously difficult and our policy has been to explore the scrotum of all who present with signs of an acute scrotum. The medical records of 199 boys aged less than 13 years who presented with an acute scrotum at Red Cross War Memorial Children's Hospital in Cape Town during the period 1970-1996 were retrospectively reviewed. Diagnosis was made clinically in all cases. Sixty-two boys (31%) were found on exploration to have torsion of the testis (mean age 6.3 years), the left side being affecting 2.5 times more frequently than the right (1 neonate had bilateral torsion), 62 (31%) had torsion of testicular appendages and 56 (28%) had epididymo-orchitis. Nineteen were grouped separately and 13 (6.5%) of these were treated conservatively. Boys needing an orchidectomy (N = 38) for testicular torsion presented three times later than those who did not (48 v. 16.5 hours). Successful conservation of the testis was directly related to the time interval of symptom development and surgical derotation. Boys with torsion of a testicular appendage presented later (mean 51 hours) and were older (mean 10 years) than boys in the other two groups. Those with epididymo-orchitis were younger (mean 3.3 years) and 40% were younger than 1 year. Only in one-third was there any clinical supportive evidence of the diagnosis. Surgical complications were infrequent. We advocate expedient surgical exploration and derotation with contralateral orchidopexy, since clinical differentiation between torsion of the testis, testicular appendages and epididymo-orchitis is inexact, even with highly specialised investigation such as Doppler ultrasound and radionuclide scanning, which may not be widely available, may delay definitive treatment and suggest a lack of urgency where time is of the essence.

Acute Disease

Abdominal aortic aneurysms in children.

Abdominal aortic aneurysms are rare in children. Causes include mycotic aneurysms, vasculitides (eg, Takayasu's arteritis), connective tissue diseases (eg, Marfan's syndrome, Ehlers-Danlos syndrome, and tuberous sclerosis) and traumatic false aneurysms. Four cases are described. Case 1 was a 12-year-old boy who presented with an acute unheralded rupture of the subdiaphragmatic aorta accompanied by lower limb paralysis and ischemia. Attempted repair failed because of extensive friability of the large arteries. Histological evaluation confirmed cystic medial necrosis despite Marfanoid phenotype. Cases 2 and 3 were boys aged 12 and 11 with Takayasu's arteritis who presented with hypertensive encephalopathy and heart failure. Although both had involvement of the origins of the renal arteries, one aneurysm was predominantly suprarenal and the other infrarenal. Currently both children are being managed successfully with antihypertensive therapy. Case 4 was a 5-year-old girl who presented with hypertension and a pulsatile abdominal mass after treatment of infective endocarditis 18 months previously. Arteriography and three-dimensional computed tomography confirmed an aneurysm (6 x 5 x 4 cm) arising from the aorta and involving the right renal artery. Aneurysmectomy, removal of a small ischemic right kidney, and Gore-Tex grafting resulted in cure of the hypertension and uneventful recovery. The present series confirms that rupture is a fatal complication, renovascular complications are common, and medical control of hypertension is an essential part of management. Management strategies need to be highly individualized, and may be successful without surgical intervention. Close clinical and ultrasound follow-up of those managed nonoperatively is essential.

Age Factors

Clinical outcome and long-term quality of life after surgical correction of Hirschsprung's disease.

One hundred seventy-eight of 330 patients were recalled after undergoing surgery for histologically proven Hirschsprung's disease (HD). One hundred fifteen were older than 4 years at interview (Mean age, 10 years). This sample appeared to be representative of the whole in terms of demographic features such as ethnic group, sex, length of aganglionic segment, timing of presentation and surgery performed. Anthropomorphic indices for weight and height were comparable to norms, but many younger patients were below expected weight for age. In general, weight and height for age was regained with time. Nine patients had delayed developmental milestones, which were owing to specific causes in four. Nine patients had a poor functional outcome, of which two had neurological impairment. Satisfactory school performance was achieved in all but 19 (26%) of the remaining patients. Long-term functional results were comparable for the Soave and Duhamel procedures with less favorable results noted following the Swenson procedure. Assessment of complications demonstrated a significantly (P < .01) lower incidence of constipation, sexual dysfunction, and micturition disturbance following the Soave procedure when compared with the Duhamel and Swenson procedures. Neurological impairment and length of aganglionic segment beyond the rectosigmoid area appeared to influence functional outcome, as did persisting enterocolitis. Enterocolitis was observed in 16.6% of patients on presentation, but continued in only 6%. Constipation was particularly associated with the Duhamel procedure, and a higher incidence of micturition disturbance, abdominal distension, and cuff stricture was noted following the Swenson procedure. Functional assessment by three different scoring methods showed that 86 (74.7%) of the 115 patients over the age of 4 had excellent anorectal function and appeared to be well adjusted. Twenty-two patients (19.2%) had relatively minor long-term problems but seven (6.1%) had persistent fecal soiling with resulting psychosocial maladjustment.

Anal Canal

Iatrogenic esophageal perforation in children: patterns of injury, presentation, management, and outcome.

Iatrogenic esophageal perforations in children are rare. To evaluate patterns of injury, clinical presentation, and treatment options for such patients, the authors reviewed the case records of 11 children who had sustained transmural injury to the esophagus during a dilatation procedure at their institution between 1967 and 1994. Strictures requiring dilatation were attributable to caustic ingestion in eight, esophageal atresia repair in two, and congenital stenosis in one. Eight were penetrating injuries, and three were disruptions. Two involved the cervical esophagus; and nine involved the thoracic esophagus. Pain, pyrexia, and tachycardia were early signs. Proximal thoracic perforations led to signs in the left chest region (effusion/pneumothorax); with distal perforation the signs were on the right side. Treatment along conventional lines (local drainage, gastrostomy, primary repair after early recognition, and antibiotic therapy) was successful, with two major complications, both empyemas. The most recent case, a disruption of a tracheoesophageal fistula stricture, was successfully treated with oral water irrigation and antibiotics only. Six had colonic interposition (all caustic ingestion), one required endoesophageal resection of a distal stricture, and four had resolution of the stricture, three without additional dilatation. The majority of children with iatrogenic injuries of the esophagus can be managed successfully by conservative measures and pleural drainage, with surgical procedures reserved for large disruptions of the esophagus, intraabdominal perforations, and cases that do not respond to appropriate conservative measures. Patients with caustic injury to the esophagus have a greater risk for the development of penetrating injury, and this may be one indicator of the severity of scarring. There are distinct clinical patterns of presentation, which depend on the level at which the esophagus is perforated. Dilatation disruption of a localized stricture has a good long-term prognosis for the esophagus and may even cure the stricture. The role of oral irrigation still must be fully evaluated where disruption has occurred as distinct from a penetrating injury.

Adolescent

Transposition of the antropylorus for anal incontinence--an experimental model in the pig.

An experimental model in the pig rendered incontinent of feces was developed to assess the sphincteric activity of the transposed antropylorus. In the control group, normal defecation was studied clinically, radiologically, and manometrically. Nineteen 7- to 10-week-old pigs were rendered incontinent by resection of 20 cm of colon and rectum to below the dentate line. The antropylorus was prepared on its own blood supply and transposed to the anus, initially with a colostomy, which was closed 15 to 21 days later. Clinically these pigs passed semisolid stool in a piecemeal fashion. Contrast defecography showed hold-up at the pylorus, reflux of contrast into the colon, with pyloric contraction independent of antral stimulation. Manometry showed pyloric contraction with rise in antral pressure and independence. The authors conclude that transposition of an antropyloric segment to the anus provides a sphincter-like mechanism and could have application in fecal incontinence.

Animals

Hydatid cyst of the pancreas--a case report in a child.

Primary pancreatic hydatid disease is rare. The diagnosis may be difficult when the presentation is that of an unexplained epigastric mass, despite suggestive radiological and ultrasonic features. We describe a 12-year-old girl in whom the definitive diagnosis was only made at repeat operation, when appropriate surgical therapy was effected.

Child

Conservative initial treatment for liver abscesses in children.

A total of 124 children aged less than 14 years with a liver abscess were seen in a 16-year period (1974-1990) and treated by non-operative initial management. Of the abscesses 98 occurred in the right liver and 26 in the left. The abscesses were solitary in 93 patients. Overall, 77 of the solitary and 21 of the multiple abscesses were confined to the right liver. In 78 of the right-sided and 20 of the left-sided abscesses the infection was primarily pyogenic in nature with Staphylococcus aureus being the usual organism cultured. The remainder were of amoebic origin. Clinical features were similar in patients with amoebic and pyogenic abscesses. Clinical and ultrasonographic follow-up demonstrated successful non-operative management and healing in 37 per cent of all patients submitted to an initial protocol of medical supportive care and antibiotic therapy. Of the multiple abscesses 60 per cent responded to non-operative management. Fourteen of the 16 solitary left-sided liver abscesses required drainage and three left-sided abscesses ruptured before drainage. Patients with a solitary left-sided abscess warrant early operative intervention.

Anti-Bacterial Agents

Management of hemobilia and persistent biliary fistula after blunt liver trauma.

A 10-year-old girl had hemobilia and a biliocutaneous fistula after blunt abdominal trauma. Embolization of the right hepatic artery occluded the hepatic artery aneurysm, but the cutaneous biliary fistula persisted despite prolonged (8 months) conservative management including sphincter decompression with an endoscopically placed biliary stent. Roux-en-Y fistuloenterostomy eventually cured the bile leak.

Abdominal Injuries

Yersinia pseudotuberculosis affecting the appendix.

Yersinia pseudotuberculosis is an uncommon cause of abdominal pain. It has a much lower incidence than Yersinia enterocolitica, and most reports have emanated from Europe or North America. This report is about a patient with Yersiniosis affecting the appendix alone, in contrast to the usual picture of mesenteric adenitis or septicemia associated with this organism.

Appendix

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