An audit of the management of the acute scrotum in children with Henoch-Schonlein purpura

Many children with Henoch-Schonlein anaphylactoid purpura syndrome (HSAPS) who develop an acute scrotum have scrotal explorations to exclude torsion of the spermatic cord. However, the cause of the acute scrotum in the context of HSAPS is known to be vasculitis and not torsion. The aim of this study, therefore, was to identify factors that underlie this practice. In a 10-year retrospective study of male patients admitted to a Children’s Hospital with a diagnosis of HSAPS, 22 out of the 93 children identified (22/93=24%) had scrotal involvement. Three children (3/22=14%) were investigated radiologically, eight children (8/22=36%) had surgical exploration and none had testicular torsion. We believe that greater awareness of the syndrome and its clinical presentation amongst paediatric surgical staff could allow the adoption of a conservative approach in children with an unequivocal diagnosis of HSAPS provided such an approach is supported by high resolution colour Doppler sonography and a fully informed parental consent. Surgical exploration is indicated if the diagnosis of the syndrome is not beyond doubt and torsion cannot be excluded on clinical grounds.

The Henoch-Schonlein anaphylactoid purpura syndrome (HSAPS) is a common systemic disease of early childhood and is characterized by an acute vasculitis that may involve the skin, joints, kidneys and gastrointestinal tract. The diagnosis is clinical and aided by the appearance of a characteristic purpuric rash that classically involves the buttocks, perineum and lower limbs. Complications involving the male genital system are unusual and were first described in the 1960’s. They include oedema and haematoma of the scrotal wall and spermatic cord, testicular haemorrhage and subcapsular testicular haematoma, epidydimitis, orchitis and penile swelling. The signs often mimic conditions that require surgical intervention, especially torsion of the spermatic cord, and a number of children have scrotal explorations. In this study we reviewed our experience with the management of children with scrotal involvement in HSAPS.

All the available records, of male patients admitted to Alder Hey Children’s Hospital, Liverpool, with a diagnosis of HSAPS in the 10-year period between 1987 and 1997, were reviewed. Records of patients with complications of the male genital system were examined in detail.

Ninety-three patients were identified. Their age at presentation ranged from 8 months to 16 years (median 69 months). Twenty-seven boys (29 %) had involvement of the male genital system. Twenty-two boys (24 %) had a swollen and tender scrotum; their ages ranged from 17 months to 12 years (median 68 months). The remaining 5 boys (5 %) had swelling of the penis. In this last group, one boy was treated with antibiotics for balanitis because of the degree of preputial and glandular swelling, before the onset of the characteristic purpuric rash.

In the group with scrotal involvement (n=22) the clinical presentations were similar. On examination, all the children had varying degrees of scrotal pain with swelling, discolouration and tenderness. In two boys, the characteristic rash was not present when the scrotal symptoms developed. In a further boy, the rash was noted by the attending surgeon but its significance was over-looked. In all the other patients, the diagnosis of Henoch-Schonlein syndrome was established before the development of scrotal symptoms. In 10 boys, the symptoms were bilateral and in 12 unilateral. Twelve boys were either reviewed by surgeons or initially referred to the paediatric surgical service because of the severity of their symptoms. Three boys (3/22=14%) were investigated with ultrasound that revealed good Doppler flow and were managed conservatively. Eight boys (8/22=36%) had surgical exploration of the scrotum because testicular torsion could not be excluded on clinical grounds. These included the three in which the diagnosis of Henoch-Schonlein syndrome had not been made. The findings at surgical exploration included epidydimitis (three), haemorrhagic fluid in the tunica vaginalis (two), testicular haematoma (one), scrotal wall oedema and haematoma (one) and oedema of the scrotal wall and spermatic cord (one). There were no cases of torsion.

There have been a number of reports of children with scrotal involvement as part of HSAPS and only one reported case of testicular torsion associated with haemorrhagic vasculitis. The reported incidence of scrotal involvement ranges from 2 to 38% and in a small minority of children the acute scrotum is the presenting manifestation of the syndrome. Our own incidence of scrotal involvement (24%) is within the range previously reported. The incidence of surgical exploration (8/22=36%) is high and represents the majority of children referred for assessment to the Paediatric Surgical service.

Clinical examination of the scrotum and localization of signs to specific intrascrotal structures is difficult in children due to inflammation and tenderness. Urgent surgical exploration is widely considered as the only sure and safe way to exclude torsion of the spermatic cord and consequently the threshold for such explorations is low. The limited usefulness of conventional sonographic images in the assessment of the paediatric acute scrotum has further contributed to this practice.⁶ High resolution colour Doppler sonography is a much better diagnostic tool as it allows visualization of intra-testicular vascular anatomy and can distinguish between testicular blood flow and flow in the scrotal wall itself.^7,8,9However, even its use is not without pitfalls.¹⁰ In this context, the relatively high incidence of explorations in our series is understandable. However, it is likely that lack of awareness of HSAPS amongst paediatric surgical staff contributed to this incidence. The underuse of radiology in the assessment of these cases may have also been a contributing factor.

We believe that whilst surgery remains the only foolproof way to exclude torsion, the adoption of a conservative approach is acceptable in some patients with HSAPS and an acute scrotum under certain conditions. This is the group with an unequivocal clinical diagnosis of HSAPS and this invariably means the presence of the characteristic rash. If these patients are first referred to the paediatric surgeons they should be familiar with the syndrome and should confirm the diagnosis after consultation with their paediatric colleagues. The decision to treat expectantly should be supported by high resolution colour Doppler sonography confirming increased testicular blood flow in support of the diagnosis of vasculitis. It may be that this service should be available at all hours for this policy to work and its role should be as an adjunct to the clinical diagnosis. Another very important issue that has to be addressed is that of fully informed and documented parental consent for the decision not to operate. The adoption of conservative management would have potential medicolegal implications in cases of missed torsion of the spermatic cord; it could be argued that failure to follow the established surgical practice of urgent exploration could amount to negligence. We believe that whilst it should be made clear that testicular survival is indeed compromised if a torted testis is managed conservatively, it should be explained to parents that the scrotal signs can be part of the systemic syndrome and are caused by vasculitis, a process different from torsion, and that on the clinical and radiological evidence torsion is exceedingly rare.

In a small group of patients with HSAPS, the scrotal signs will appear before the characteristic rash but may be associated with other more subtle signs of the syndrome including haematuria and joint swelling. In their presence, a surgeon aware of these clinical manifestations of the syndrome should look for evidence of a developing purpuric rash. However, in the absence of the rash, the management of an acutely tender and swollen hemi-scrotum would have to be urgent surgical exploration. Finally, in another small group of patients with HSAPS, an acute scrotum develops before any other clinical manifestations of the disease. In this group, and in the presence of severe scrotal symptoms and signs, there is little choice but to explore the scrotum with urgency.

Our practice should aim to avoid testicular loss due to failure to recognize torsion. On the other hand, we should not fail to recognize that a systemic condition can account for the scrotal symptoms and signs. We believe that it is acceptable to manage children with an unequivocal clinical diagnosis of HSAPS and an acute scrotum conservatively, provided this decision is supported by high quality Doppler ultrasound scans and a fully informed and documented parental consent. If the diagnosis of HSAPS is not beyond doubt and if the diagnosis of torsion of the cord cannot be excluded on clinical grounds, the only safe approach is to explore the scrotum with urgency.

Correspondence: Mr Rick Turnock, Consultant Paediatric Surgeon, Department of Paediatric, Surgery, Alder Hey Children’s Hospital, Eaton Road, Liverpool L12 2AP, U.K.

AUDIT

An audit of the management of the acute scrotum in children with Henoch-Schonlein purpura