Degenerative leiomyopathy with massive megacolon. Myopathic form of chronic idiopathic intestinal pseudo-obstruction occurring in indigenous Africans.
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Adolescent↗
Biomedical subjects
S Cywes
Publications and source records attributed to S Cywes.
Biliary atresia--surgical management. A 10-year review.
The surgical results in 39 children operated on for biliary atresia at Red Cross War Memorial Children's Hospital between January 1975 and January 1985 are reported. The mean age at operation was 12.8 weeks (range 6 weeks-6 months). In the first 4 years no patient had sustained bile drainage after operation. Since 1979 16 of 32 patients (50%) have had significant bile drainage. Of these, 8 are alive and have been followed up from 6 months to 6 years after operation. Five are jaundice-free and well. The other 3 are mildly jaundiced, have evidence of ongoing liver damage, and have a poor prognosis. The deaths were due to progressive liver damage with liver failure, portal hypertension, and intercurrent infection. Cholangitis after 'successful' porto-enterostomy was the main cause of poor prognosis and can be related to the age at referral and the presence of cirrhosis of the liver at the time of surgery; thus bile drainage does not necessarily equate with cure. Early referral of infants with conjugated hyperbilirubinaemia to a major centre is of paramount importance if we are to improve the prognosis.
Age Factors↗
Colonic oesophageal replacement in children--functional results.
Since 1961 colonic replacement of the oesophagus has been performed in 38 patients of whom 16 were followed up long-term. The main indication was caustic soda injury to the oesophagus. We prefer a single-stage retrosternal isoperistaltic left colon interposition, based on the ascending branches of the left colic artery, with concomitant pyloroplasty. Oesophagectomy was not performed. The main complications were vascular insufficiency, upper anastomotic leak and subsequent stricture formation. Long-term follow-up (mean 9.5 years) revealed functional acceptance with normal swallowing, absence of pulmonary and gastrointestinal complications and rapid emptying of a passive conduit. Upper cervico-colonic anastomotic stricture of various degrees occurred in 14 per cent of the patients (5 of 35 patients).
Child↗
Postoperative intussusception in the paediatric patient.
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Child, Preschool↗
Button-battery ingestion--a hazard of modern living.
Disc batteries are ingested by children with ever-increasing frequency as they become more widely available for a multitude of small electronic devices in the home. Because of their unique properties their ingestion cannot be managed in quite the same expectant way as most other foreign bodies swallowed. Experience with 13 cases is briefly recorded, the literature is reviewed, and based on our findings a plan of management is suggested. The potential hazards of button-battery ingestion should be publicized, and prevention encouraged by parental education, manufacturer's packaging and safe disposal of used batteries.
Child↗
The birth of a child with a congenital anomaly. Part III. Response of parents to the diagnosis.
The parents of 90 babies diagnosed as suffering from cystic fibrosis (17 cases), Hirschsprung's disease (30), oesophageal atresia (23) and anorectal malformations (20) were interviewed to investigate their reactions at the time of learning the diagnosis. The majority of parents were satisfied with the manner in which the diagnosis had been conveyed to them: the most pervasive factor in this respect was the attitude of the teller. Where this was sympathetic, parents responded positively even when too little information had been given or one parent had been told alone. The largest proportion of dissatisfied parents were from the cystic fibrosis group, in which, in contrast to the surgically correctable anomalies, the element of hope was not present.
Anal Canal↗
The birth of a child with a congenital anomaly. Part II. The prediagnostic phase after discharge from the maternity home.
Thirty babies discharged from the maternity home undiagnosed were subsequently found to be suffering from cystic fibrosis (12 cases), Hirschsprung's disease (13) or an anorectal malformation (5). The efforts of parents in this respect and the factors influencing the establishment of a diagnosis were investigated. Maximum periods of 3,9 years, 3,75 years and 5 years respectively were required to confirm a diagnosis in patients with the above three conditions. Although the severity of symptoms is an important factor in speed of diagnosis, other mediating variables had influenced the outcome in our sample; patients referred to a children's hospital were diagnosed sooner. For this reason patients from the lowest social class were diagnosed earlier than those from the higher socio-economic groups. The problems experienced by parents during this phase are reviewed.
Anal Canal↗
The birth of a child with a congenital anomaly. Part I. Some difficulties experienced by parents in the maternity home.
An investigation study of 90 cases in which a baby had been born with cystic fibrosis, oesophageal atresia, Hirschsprung's disease or an anorectal malformation was undertaken to establish the duration of the prediagnostic phase and the difficulties experienced by parents before discharge from the maternity home. The congenital anomalies requiring immediate surgical intervention were diagnosed very soon after birth. A wider diversity of problems were encountered in the case of those conditions with a longer prediagnostic phase. The problems encountered by parents, viz. lack of communication with staff, fantasies of gross anomalies with fear for the baby's life, and the lack of viewing and mothering of the baby are discussed, and some long-term effects are highlighted.
Anal Canal↗
Blunt liver trauma in children: nonoperative management.
Since 1978, we have treated 19 of 23 (83%) children with blunt liver trauma nonoperatively. Management consisted of observation in an intensive care unit, repeated physical examination, frequent reevaluation of laboratory values, special investigations, and bed rest. The 19 patients all remained stable, required no surgical intervention, and showed resolution of the hepatic injuries with no early or delayed complications. Ultrasonography, although not as reliable a method as computed tomography or liver isotope scans for identification of hepatic trauma at first presentation, provided a very useful method for documenting subsequent progress and eventual healing of the lesions. The presence of an isolated hepatic injury is insufficient indication for surgery. If there is significant extrahepatic injury requiring surgery, or if the patient with hepatic trauma is deteriorating, operative intervention is mandatory.
Bed Rest↗
Falciform ligament abscess in the infant.
Two cases of abdominal abscesses related to the falciform ligament are described. The importance of ultrasonic localization is stressed, as these extrahepatic abscesses require surgical drainage, whereas an intrahepatic abscess, particularly amoebic, may be treated with antimicrobials.
Abdomen↗
Oesophageal atresia--severe tracheomalacia and its correction by aortopexy.
Tracheomalacia is an important cause of recurrent respiratory complications following correction of cogenital oesophageal atresia. It constitutes a well-defined syndrome, which requires clinical recognition and surgical correction in the most severely affected infants. Five cases are described. Special investigations confirmed tracheal collapse. Permanent relief of symptoms was achieved by suspension of the aortic arch to the sternum.
Aorta, Thoracic↗
The psychosocial impact of cystic fibrosis. A review of research literature.
Research literature on the psychosocial aspects of cystic fibrosis (CF) is reviewed. The findings indicate that patients with CF and their families are subject to major stresses, yet many manage to function without observable dysfunction. However, indications are that the coping mechanisms of these families need to be enhanced and developed in order to prevent possible dysfunction.
Cystic Fibrosis↗
Gastrostomy tube stabilizer.
A new method of gastrostomy tube fixation is described using a metal-ring appliance. This maintains the position of the tube, preventing progression of the tube towards the pylorus, and avoids movement, thus diminishing leakage around the tube. The wound and skin are exposed, easily accessible, and avoid the sogginess encountered with the nipple stabilization. The incidence of fungal infections is also reduced.
Enteral Nutrition↗
The use of a lateral skin-flap perineoplasty in congenital anorectal malformations.
A lateral skin-flap perineoplasty has been used in 15 patients born with an absent anal canal. The aim behind the introduction of this modification was the prevention of mucosal prolapse. On early assessment, this has been achieved in 11 of the cases. This represents a 50% improvement on our previous results.
Anal Canal↗
Prenatal perforation of the extraperitoneal part of the rectum, associated with a developmental defect of the pelvic floor.
Perforation of the extraperitoneal part of the rectum during foetal life is an exceptionally rare event. Two cases are reported where this has been associated with a para-rectal defect in the pelvic floor. It is postulated that in these instances, the rectal lesion is due to this abnormality. As a consequence of the perforation meconium extravasates into the buttock. This has a classic presentation which should be immediately recognised. Urgent rectal decompression, as an emergency measure before surgery, is required if further serious complications are to be prevented.
Extravasation of Diagnostic and Therapeutic Materi↗
Complete median cleft of the mandible and aplasia of the epiglottis. A case report.
A rare case of complete median mandibular cleft without associated anomalies of the adjacent soft-tissue structures is presented. Aplasia of the epiglottis was also present, a combination not previously reported. The anomaly seems to be the result of arrested development of the lower half of the first branchial arch, and in particular of the mesenchymal elements. It may assume varying degrees of severity, ranging from an incomplete cleft of the lower lip or mandible (or both) to a complete midline cleft of the lower lip, mandible and tongue, sometimes with associated deformities of soft-tissue structures derived from the lower branchial arches in the neck. Although there is no consensus on the timing of corrective procedures, the strategy favoured by most seems to be early repair of the soft-tissue abnormalities and deferment of the mandibular repair until after puberty. Persistent aspiration due to the absence of the epiglottis necessitated performance of a tracheostomy and feeding gastrostomy in our patient. The patient sustained an anoxic episode as a result of tracheostomy obstruction and died a few weeks later.
Epiglottis↗
Fecal incontinence: a simple pneumatic device for home biofeedback training.
A simple pneumatic biofeedback device has been designed for patients who have failed to develop social fecal continence following sacroabdominoperineal pull-through operations for anorectal anomalies. The components that make up the device are easily and cheaply available. This device was designed to be used by the patient at home and needs only the assistance of an adult.
Biofeedback, Psychology↗
Inadequate pouch emptying following Martin's pull-through procedure for intestinal aganglionosis.
Diarrhea is an expected problem following Martin's operation. Its cause is obvious. With medical management, the phase of intolerance to enteral feeding can be bridged. Adaptation is usually reached within 3-6 mo of surgery. This is confirmed by our experience with five patients treated in this manner. In two further patients, episodic bouts of diarrhea continued to mar their recovery. It was then appreciated that this was related to pouch content stasis. The nonoperative management of this complication proved unsuccessful. A death at 20 mo post-pull-through due to "enterocolitis" and an operation 13 mo post-pull-through to repair an acute pouch perforation followed. Attention is drawn to this complication. The following points aimed at preventing stasis, which in our series has been the most difficult problem to treat, are made: a low anal anastomosis--0.5 cm above dentate line; limit pouch size; and use normally innervated bowel. With the use of these changes, early postoperative diarrhea may be difficult to control. For this reason it is proposed that parenteral feeding be used routinely in the immediate postoperative period, and, as rapid fluid loss is better tolerated, surgery be deferred until the patient is 8 mo or more of age.
Colon↗