Spencer W. Beasley

Intra‐abdominal manifestations of Henoch‐Schönlein Purpura

Gastrointestinal involvement occurs in approximately two thirds of children with Henoch‐Schönlein Purpura (HSP) and usually is manifested by abdominal pain. Abdominal symptoms precede the typical purpuric rash of HSP in 14–36%; the symptoms may mimic an acute surgical abdomen and result in unnecessary laparotomy. Major complications of abdominal involvement develop in 4.6% (range 1.3–13.6%), of which intussusception is by far the most common. The intussusceptum is confined to the small bowel in 58%; its frequent inaccessibility to demonstration by contrast enema means that ultrasonography is the investigation of choice. Ultrasonography complements serial clinical assessment, clarifies the nature of the gastrointestinal involvement and reduces the likelihood of unnecessary surgery. Bowel ischaemia and infarction, intestinal perforation, fistula formation, late ileal stricture, acute appendicitis, massive upper gastrointestinal haemorrhage, pancreatitis, hydrops of the gallbladder and pseudomembranous colitis are seen infrequently. Earlier diagnosis and prompt treatment of intra‐abdominal complications has reduced the mortality from 40% to almost zero.

Descent of the testis

Part 1 Evolution of descent of the testis and early history of research: evolution of testicular descent early work on testicular descent. Part 2 Transabdominal migration of the testis: the first hase of descent the gubernacular swelling reaction oestrogen-treated mouse persistent mullerian duct syndrome the role of mullerian inhibiting substance (MIS) conclusion. Part 3 Inguinoscrotal descent of the testis: migration of the gubernaculum intra-abdominal pressure the role of testosterone the genitofemoral nerve hypothesis sexual dimorphism of the genitofemoral nerve the effect of CGRP conclusion. Part 4 Classification and causes of undescended testes in humans: classification of undescended testes the incidence of undescended testis what causes cryptorchidism? cryptorchidism in rare syndromes. Part 5 The postnatal effects of cryptorchidism: a primary anomaly or secondary effects? endocrine effects morphological effects fertility malignancy. Part 6 Diagnosis of undescended testis: introduction aims of clinical examination technique of examination undescended testis versus retractile testis clinical features of ectopic testes impalpable testis conclusion. Part 7 Operative treatment: planning surgery the standard orchidopexy unusual findings during orchidopexy spermatic cord of inadequate length the unilateral impalpable testis surgical management in the post-pubertal child indications for orchidectomy complications. Part 8 Hormonal treatment: historical background rationale for treatment standard regimes does hormonal treatment work? possible benefits of hormonal treatment why hormonal treatment fails conclusions. Part 9 Results of treatment: criteria of assessment cosmetic result fertility malignancy conclusions. Part 10 Conclusions and future developments.

The clinical implications of non-idiopathic intussusception.

Abstract A pathological lesion can be identified at the leadpoint of intussusception in about 6% of episodes. Occasionally, general manifestations of an underlying disease indicate the specific cause of an intussusception (e.g., perioral pigmentation in Peutz-Jeghers syndrome), but usually the clinical features provide no clues as to the aetiology. Neonatal intussusception may be caused by a duplication cyst or Meckels diverticulum. Beyond 12 months, the proportion of intussusceptions due to a pathological lesion at the leadpoint increases with age. There is an identifiable lesion in the majority of children over 5 years of age. Postoperative intussusception accounts for between 0.5% and 16% of intussusceptions, although it has a variety of causes; it typically follows retroperitoneal dissection. It is unusual for an intussusception due to a pathological lesion at the leadpoint to be reduced by enema. If it is reduced, the lesion may be seen at the time of reduction or fluoroscopy, or subsequently on ultrasonography.

Diagnosis and treatment of foregut duplications

In its broadest sense, the term, foregut duplication encompasses the full spectrum of developmental aberrations of the embryonic foregut (bronchopulmonary and alimentary tract). Evidence is emerging that the notochord may have a pivotal role to play in foregut development through the Shh-GLi signalling pathway. The investigation and management of these lesions depends on the clinical presentation and the level of the foregut affected. The presentation of symptomatic foregut duplications also depends on any space-occupying effect they exert and where specific complications related to the malformation occur, such as when the mucosal lining contains acid-secreting cells. In a minority of cases, (eg, where they cause respiratory compromise or spinal cord compression) urgent intervention is required. In the remainder, precise diagnostic imaging according to the level and location of the foregut duplication provides the necessary information to plan surgical excision of the lesions. Magnetic resonance imaging best shows the relationships of complex bronchopulmonary foregut malformations and associated anomalies of the spine. eg, neurenteric canal. Most lesions can be excised with minimal morbidity. Minimal-access surgical techniques can be applied to the simpler cysts, particularly some bronchogenic cysts. Thoraco-abdominal duplications and neurenteric cysts require careful preoperative delineation and more complex surgery.

Comparison of results of laparoscopic and open antegrade continence enema procedures.

Abstract Several modifications of the Malone antegrade continence enema (ACE) procedure have now been reported. In this study we have compared the results of our experience with the laparoscopic appendicostomy (LACE procedure) with the published results of previously described open ACE procedures. Children who had the LACE procedure at our institutions were reviewed. Intra- and postoperative problems were identified by review of hospital case notes and from a questionnaire completed by the childrens caregivers. These results were compared with the reported results of the original Malone procedure, the Peña modification, and the open appendicostomy as described by Wilcox. Since 1994, 30 children have had LACE procedures performed at our institutions. Two have required conversion to an open procedure because of difficulty locating the appendix. The stoma is being used for regular antegrade colonic washouts in 29 of the 30, compared with 19 of 31 in Malones series and 16 of 20 in Peñas. Improvement in soiling has been achieved in 27 (90%) of our patients, 15 of whom are completely clean, a rate similar to that of the other types of procedure (61%–78%). Stenosis of the stoma has occurred in 8 children (27%), compared with rates of 10%–33% in other series. Stomal leakage has been troublesome for 2 (6.7%), compared with leak rates between 5.6% and 15% in other series. The LACE procedure is technically the simplest. The laparoscopic approach to the ACE procedure appears to be a simpler and safe alternative to previously described methods. It causes minimal morbidity, has a high long-term viability rate, and resulted in improved control of soiling in 90% of the children with faecal incontinence in whom it has been employed.

The relationship between cerebral palsy and cryptorchidism

This study examined the reported association between cerebral palsy and cryptorchidism. A comparison was made among 25 boys with cerebral palsy under the age of 2 years and 6 months, 25 boys with cerebral palsy aged between 5 and 10 years, and age-matched controls. The testes remained in the same position with age in boys with cerebral palsy, whereas in normal children the testes were slightly lower initially (P less than .005) and became lower still with growth (P less than .001). This result, taken in conjunction with previous studies, casts doubts on the theories of early damage to the hypothalamic-pituitary-gonadal axis as the cause of maldescent in cerebral palsy. It is postulated that any apparent increase in cryptorchidism in older patients with cerebral palsy may be caused by spasticity of the cremaster muscle leading to pathologic retraction of the testis out of the scrotum.

Evidence of a common pathogenesis for foregut duplications and esophageal atresia with tracheo‐esophageal fistula

The pathogenesis of the alimentary tract duplications, including foregut duplications (FgD) remains speculative. The accidental finding of FgD in fetal rats with esophageal atresia and tracheoesophageal fistula (EA‐TEF) induced by Adriamycin provided an animal model to investigate a possible relationship between these two entities. Timed‐pregnant rats were intraperitoneally injected with Adriamycin (1.75 mg/kg) on gestational Days 6 to 9. Their embryos were harvested by Caesarean section from gestational Days 14 to 21. Forty‐six of embryos were processed and serially sectioned in the transverse or sagittal planes. EA‐TEF occurred in 43/46 (93%) embryos of which 11 (24%) were found to have an associated FgD located at the level where the esophagus was absent. Six FgDs communicated with the foregut or the trachea. Five noncommunicating FgDs were located between the foregut and the vertebral column. In the control embryo, the notochord was located in the centre of the vertebral column from Day 11 of the gestation. In Day 14, 15 and 16, however, embryos exposed to Adriamycin, an abnormal notochord or branch frequently was located within the mesenchyme of the maldeveloped foregut or attached to the duplication cyst. In some, it appeared that the notochord was drawing the cyst‐like structure away from the foregut. The present study confirms that duplications adjacent to the esophagus arise from the foregut and that failure of the foregut to detach from the notochord at the normal time may contribute to the development of foregut duplications. Anat Rec 264:93–100, 2001.

The contribution of the adriamycin-induced rat model of the VATER association to our understanding of congenital abnormalities and their embryogenesis

Abstract The adriamycin-induced rat model of the VATER association has provided a means of studying the morphogenesis of a variety of major congenital structural abnormalities similar to those seen in humans with the VATER association. Most interest has been centered on the foregut, where the model has clarified some aspects of the development of esophageal atresia (EA), tracheal agenesis, and other communicating bronchopulmonary foregut malformations. It has demonstrated aberrations in the nerve supply to the esophagus in EA and allowed the study of tracheomalacia. A relationship between an abnormal notochord, foregut abnormalities, and vertebral defects has been shown, and the model has reignited interest in the role of the notochord as a regional organizer of axial development. The normal temporospatial characteristics of apoptosis during fore- and hindgut development is disturbed in this model, resulting in abnormal morphology. The indications are that this model will continue to clarify the processes that lead to many of the structural congenital abnormalities that are seen in infants born with the VATER association.

Intussusception and rotavirus associated hospitalisation in New Zealand.

Aims: To describe the epidemiology of intussusception and its relation to rotavirus associated hospitalisation in New Zealand. Methods: National hospital discharge data between January 1998 and June 2003 for all children younger than 3 years of age with intussusception were reviewed. Independently, children from the same age group, admitted to eight paediatric units with rotavirus gastroenteritis between May 1998 and May 2000, were identified prospectively. Epidemiological characteristics of cases with intussusception were compared with those of hospitalised rotavirus disease. Results: During the 5.5 year study period, there were 277 cases of intussusception and no deaths. Most (72%) occurred in the first year of life (age adjusted incident rate 65 per 100 000 child-years, 95% CI 56 to 74). Risk of intussusception was less in females (risk ratio 0.58; 95% CI 0.43 to 0.78) and for Maori (risk ratio 0.52; 95% CI 0.35 to 0.77) when compared with European infants. In contrast to hospitalised rotavirus cases, intussusception peaked at a younger age and lacked seasonality. Conclusions: This study provides national baseline data on intussusception for future rotavirus vaccine programmes in New Zealand. Wild-type rotaviruses do not appear to have a major role in triggering intussusception. Prospective surveillance systems, using standardised case definitions and nested case-control methodology, are needed to further our understanding of the aetiology and epidemiology of intussusception.

Cryptorchidism in spina bifida and spinal cord transection : a clue to the mechanism of transinguinal descent of the testis

In spina bifida there is a high incidence of cryptorchidism, particularly where the lesion is at or above L2. This article describes the location of the testis in 32 spina bifida patients and the effect of spinal cord transection in neonatal rats: both studies suggest that the spinal cord influences testicular descent. It is already established that androgens, an intact genitofemoral nerve, and the gubernaculum are essential for the transinguinal phase of testicular descent to occur. Yet the mechanism by which testosterone exerts its effect on the gubernaculum remains obscure. To account for all the observed phenomena we postulate that a spinal cord nucleus may exist that is sensitive to androgens and mediates impulses through the genitofemoral nerve to the gubernaculum. Neuronal modulation of the gubernaculum may induce testicular descent.