Neurogenic chronic idiopathic intestinal pseudo-obstruction, patent ductus arteriosus, and thrombocytopenia segregating as an X linked recessive disorder.

We present a family with three affected males in two generations with congenital neurogenic chronic idiopathic intestinal pseudo-obstruction (CIIP), patent ductus arteriosus, and large platelet thrombocytopenia apparently segregating as an X linked recessive disorder. The pattern of segregation of DNA markers within the family is consistent with linkage to the previously described neurogenic CIIP (CIIPX) locus at Xq28. This combination may represent a new contiguous gene disorder and appears to have a good prognosis with supportive therapy.

Skull fractures in children: a population study.

Objective: To provide information about children with a skull fracture in the Edinburgh area, since head injury is the single most important traumatic cause of death in children over 1 year of age.

Methods: During the period January 1983 to December 1989, all children in the Edinburgh area under 13 years of age who sustained a skull fracture were identified. Details of the children who died primarily from a head injury, and the total number of children who were admitted to hospital with a head injury not associated with a skull fracture, were also documented.

A simple alternative to circumcision.

Circumcision is overperformed in the United Kingdom. An alternative procedure, dorsal incision of the prepuce, was performed in 40 boys whose foreskins were nonretractable and showed a tight preputial band on retraction at operation with no scarring. The procedure was quick, simple, and effective at producing a retractable foreskin.

Distal penile lymphoedema following hypospadias repair: a case report.

A patient who developed isolated distal penile lymphoedema 10 years following hypospadias repair is reported. The classification and causes of genital lymphoedema and possible reasons for its occurrence in this case are discussed.

Skull fractures in children: altered conscious level is the main indication for urgent CT scanning.

It has been suggested that all children with skull fractures require urgent CT scanning to exclude intracranial injury. Adhering to such a policy could both tax limited scanning facilities and result in unnecessary exposure to radiation. The aim of this study is to assess the level of consciousness in determining the need for urgent CT scanning, and the possible role of the mechanism of injury as a secondary risk factor.

Extrarenal Wilms' tumour presenting with intraperitoneal haemorrhage.

Extrarenal Wilms' tumours are very rare and have usually been reported as isolated cases. Presentation occurs with a painless abdominal mass. This case report is the first presentation of such a tumour with intraperitoneal haemorrhage.

Out of hospital needlestick injuries.

Retrospective analysis showed that 67 children had presented in Edinburgh with needlestick injuries on 70 occasions over five years. Worryingly, 10 children sustained injuries pretending to be intravenous drug abusers. Despite risks of hepatitis B and HIV infection, protection and follow up were inadequate.

Anorectal myotomy combined with anterior resection in the treatment of Hirschsprung's disease.

A total of 42 patients with biopsy-proven Hirschsprung's disease were treated by anorectal myotomy or myectomy, alone or combined with anterior resection. Myotomy is a simple and complication-free procedure. The results compare favourably with those of other operative procedures for this condition.

The management of penile zip entrapment in children.

Thirty boys, aged 2-12 years, presented with penile zip entrapment to one Paediatric Accident and Emergency Department over a 7 year period. The rate of presentation was one zip entrapment per 4068 new patient attendances. In contrast to previous reports, injuries occurred most commonly whilst the zip was being done up and whilst underpants were being worn.

Pilomatrixoma as a diagnostic problem: the Edinburgh experience.

The purpose of this retrospective study was to review the paediatric experience in Edinburgh of a relatively benign skin tumour. The study group consisted of children less than 13 years of age with a pathological diagnosis of pilomatrixoma over the period between January 1980 and December 1989. The results reaffirmed a female preponderance.

Management of foreskin problems.

One hundred consecutive cases of boys with foreskin problems referred to local paediatric surgeons in the Edinburgh area were studied. Fifty five underwent circumcision and the remainder were managed more conservatively with or without the use of local or general anaesthetic. Although sometimes avoiding general anaesthetic, the more conservative methods involved more frequent visits to the clinic, a larger number of complications, and a longer follow up period.

Microbiological studies of the enterocolitis of Hirschsprung's disease.

The results of a prospective study of 20 cases of newly diagnosed Hirschsprung's disease (nine of whom developed enterocolitis) and 10 normal controls showed no variations in the bacterial flora (including Clostridium difficile) in the stools of the groups studied. Viral studies showed that rotavirus was present in the stools of seven of the nine cases of enterocolitis during the episode. We suggest that Hirschsprung's enterocolitis may have a complex infective aetiology and that rotavirus plays a part.

Repair of coronal and subcoronal hypospadias: an 8-year retrospective study.

Twenty-three patients undergoing treatment for coronal and subcoronal hypospadias over an 8-year period were reviewed. A ventral skin tube inlay graft was used in all cases, and a comparison made between Nesbitt's button hole technique and three other procedures used for ventral skin cover. The button hole procedure was associated with fewer fistulae and provided acceptable cosmesis.

Disc battery swallowers--two contrasting cases.

Increased use and availability of disc batteries has been reflected in the number of children presenting following ingestion of such batteries. An expectant, non operative policy is advocated. Battery manufacturers, however, recommend immediate removal of the battery.

The role of the paediatric surgeon in alleged child sexual abuse.

Two hundred ninety cases of alleged child sexual abuse (CSA) were reported in Edinburgh and the Lothian region over the 7-year period from 1982 through 1988. We have found an increase in the incidence of alleged CSA, which has resulted in greater involvement of the paediatric surgeon. CSA was found to be occurring with increasing frequency in younger children.

Management of some common surgical conditions in children: a comparison of practice between surgical units in Edinburgh.

Many common surgical conditions in children are managed as day cases by the Paediatric Surgical Service in Lothian but this practice varies within the separate units in Lothian. Five common conditions suitable for day case treatment were looked at during 1987. There was a considerable difference in the time that some conditions were kept in hospital, an orchidopexy, for example, staying for up to 7 days in one unit.

Impaired gastrointestinal mucosal defense in Hirschsprung's disease: a clue to the pathogenesis of enterocolitis?

Twelve patients with Hirschsprung's disease were studied to investigate why some children develop enterocolitis. Previous studies have failed to explain this adequately; they have also failed to explain why enterocolitis can occur many years after definitive surgery. Six of the 12 patients had enterocolitis.