Daniel G. Young

Kasabach-Merritt syndrome

Eight infants with hemangiomata associated with severe consumption coagulopathy are reviewed; three died. The presentations and method of management are discussed. The varied response to different modalities of therapy initiated this report and led to a search of the world literature in an attempt to identify the pattern of response, if any, to particular therapy. From our own experience with two cases and from the review of the literature, it appears that radiotherapy alone or in combination with steroids gives a superior overall treatment in terms of reducing the death rate.

Collagen-coated Vicryl mesh is not a suitable material for repair of diaphragmatic defects

Encouraged by the results in abdominal wall defects, the authors used the collagen-coated Vicryl mesh (CCVM) in repair of diaphragmatic defects in two patients. In a patient with recurrent diaphragmatic hernia, CCVM was used to reinforce the anterior abdominal muscle flap. The hernia recurred after 8 months. In another patient, it was used to repair a large diaphragmatic defect. This patient had a recurrence of the hernia in 10 weeks. The results suggestthat CCVM is not a good material for repairing the diaphragmatic defect. However, it can be used to reinforce other types of repairs.

Ectomesenchymoma: One or two tumors? case report and review of the literature

Ectomesenchymoma has not previously been reported in a patient with the cutaneous nevus syndrome. A case of perineal ectomesenchymoma is presented with unusual cytogenetic findings. The significance of these is discussed and the world literature reviewed. This is the first such case reported.

The changing incidence of neural tube defects in Scotland.

The impact of neural tube defects on neonatal surgery has been declining in Scotland over the past two decades. The Scottish statistics for neural tube defects were studied from 1971 to 1988. The incidence of neural tube defects in Scotland has declined from 5.50 to 1.10 per 1,000 births over this period (3.00 to 0.58 per 1,000 births for spina bifida and 2.50 to 0.52 per 1,000 births for anencephaly). Antenatal maternal alpha-fetoprotein (AFP) screening was introduced to Scotland on a wide scale in 1976. The number of terminations for anencephaly peaked in 1980 (85), and for spina bifida in 1981 (70), and both have since declined. The Scottish birth rate has been about 67,000 per year over this period. The declining incidence of neural tube defects is not explained by the effect of antenatal screening and terminations alone. A downward trend was apparent before 1976, and although antenatal screening has had a considerable impact on anencephaly births (peak terminations 89% in 1983), it has had only a modest impact on spina bifida births (peak terminations 53% in 1984). We conclude that the natural decline in incidence of neural tube defects is the major factor in the observed decline in neonatal surgical admissions for these defects.

Pseudomembranous enterocolitis—A potentially lethal disease

The cases of nine children with pseudomembranous enterocolitis, five of whom died, are reported here. Six had prior antibiotic therapy, and in eight there were other diseases present that were considered to contribute to the development of this disease. Awareness of this condition, which requires aggressive medical and surgical management, is discussed.

Fiberoptic localization of the upper pouch in esophageal atresia

This report describes a technique that the authors developed as a simple, quick means of identifying the proximal pouch in neonates with esophageal atresia by fiberoptic instrumentation.

Gastrografin enema in meconium ileus: the persistent approach

This report describes the value of repeated use of gastrografin enema in uncomplicated meconium ileus. Fourteen of 21 neonates had successful intestinal decompression (66.6%) — 5 after a single enema and 9 after repeated attempts (up to 7 times in 1 case).

Cardiac tamponade: An unusual complication of ventriculo-atrial drainage of hydrocephalus

Abstract This is a report of a case of cardiac tamponade secondary to perforation of the myocardium by the introduction of a catheter on a stylet during revision of a ventriculo-atrial shunt.

Immunoreactive trypsin levels in neonates with meconium ileus.

Serum immunoreactive trypsin (IRT) is used as a screening test for cystic fibrosis (CF) in neonates in many countries. Variations in IRT levels are observed in healthy and cystic neonates within the first few weeks of life. Fifteen percentage of CF neonates present with meconium ileus (MI). We hypothesised that there may be differences in serum IRT levels in cystic babies with simple and complicated MI. The aim of this study was to investigate the serum levels of IRT in neonates with CF presenting with MI. IRT levels were sequentially measured in neonates (n=29) with CF with intestinal obstruction due to simple or complicated MI. These were compared to levels obtained from non-cystic neonates/controls admitted with a variety of other intra-abdominal pathologies (n=49) IRT levels were significantly higher in the CF–MI group than the non-cystic controls (P<0.001). There was no statistical difference in IRT levels between the simple or complicated MI groups. In the MI group there was no statistical difference between those who required operation, no difference between the pre- and post-operative IRT levels and no significant relationship between IRT levels and birth weight or gestation. Serum IRT levels are significantly elevated in neonates with CF and MI compared with non-cystic, non-MI neonates. The results of this observational study highlight that a single raised level of IRT in a neonate should prompt the analysis for CF regardless of any underlying surgical pathology.

A personal audit of pediatric surgical training before implementation of the calman guidelines

PURPOSE The purpose of this study was to audit the surgical training experience of a Higher Surgical Trainee in the United Kingdom during the tenure of the posts of Career Registrar, Research Fellow, and Senior Registrar from August 1992 to July 1997. Although the Report of the Working Party on the UK Specialist Medical Training (the Calman report) was published during this study, the training remained the same because the author elected not to transfer to the new grade. METHODS A 39-field database was designed, and the surgical experience was entered prospectively. The data were analyzed chronologically according to age of patient, condition, level of supervision, and nature of admission (emergency, elective). RESULTS Two thousand two hundred ninety patients or operations are presented. Only a small number of patients underwent surgery during the research year (1994). The pattern of operating changed from one of large numbers of supervised to one of more major procedures. The numbers of neonates and younger infants who underwent surgery increased significantly, and the level of supervision changed allowing more autonomy toward the end of the training period. This trend was reversed partially during the period of overseas secondment. The results are compared with those in a recently published USA/Canadian study. CONCLUSION The volume of work undertaken by the trainee ensures adequate exposure to a wide range of procedures to achieve a satisfactory level of competence. Changes that may affect this are discussed.