Patricia M. Davidson

Liver tumours in children

Since 1955, 56 children (29 M: 27 F) with primary liver tumours have been treated at the Royal Childrens Hospital. Ranging in age from neonates to 14 years, they most frequently presented with an abdominal mass. The position, size, and vascularity of the tumour were assessed preoperatively by ultrasound +/− angiography. An elevated serum alpha-feto-protein was confirmatory evidence of a hepatoblastoma. In the malignant group (33), 70% of the children had tumour resection with 5 “surgical” deaths. The long-term survival in the resected group was 15/23 (65%). No children survived without resection. In the benign group (23), 8 children had an arteriovenous malformation (7 neonates). Six are alive and well following hepatic lobectomy with 1 dying of cardiac failure before the diagnosis was established. The other benign tumours were either removed by local excision (10), lobectomy (3), or biopsied (2). All children are alive and well. In those children requiring hepatic lobectomy, hypothermia and hypotension were used as adjuncts to anaesthesia. The commonest operative complication was blood loss (50%). The technique of total vascular isolation was introduced in 1975 and reduced the incidence to 16%. The use of chemotherapy enabled subsequent tumour resection in 4 of 5 tumours that were initially unresectable. Chemotherapy was also given postoperatively to 8 of 17 patients surviving resection.

Diagnostic delay in Crohn's disease

The interval between the onset of symptoms and confirmation of the diagnosis of Crohns disease in children is often many months or years. A number of factors contribute to this diagnostic delay, but the most significant appear to be delay in the patient seeking initial medical help and failure of the medical practitioner to consider the diagnosis at the time of the first medical contact. In this review of 55 children with Crohns disease the intervals between onset of symptoms, first presentation and diagnosis were examined and the causes of diagnostic delay determined. The main reason a diagnosis was not made at first presentation was because of ignorance of the fact that Crohns disease could occur in children. In older children, the combination of tiredness, lethargy and iron deficiency anaemia with vague or intermittent abdominal pain was a frequent cause of diagnostic difficulty. However, even in the absence of major gastrointestinal symptoms, the significance of growth failure and delayed puberty was usually recognised if the patient was referred to a paediatrician. Diagnostic delay could well be reduced if medical practitioners involved in the primary care of children were educated to recognise the various ways in which Crohns disease presents in this age group.

Pancreatic pseudocysts in children

Pancreatic pseudocyst (PPC) is an uncommon condition in childhood and is almost always associated with blunt abdominal trauma. Additional disease within the pancreas is rare, unlike adult experience. Prior to the advent of ultrasonography (US) assessment of the cyst was difficult. Subsequently, it has become apparent that PPC may develop but still undergo spontaneous regression. We have reviewed eight children with PPC seen at the Royal Childrens Hospital between 1967 and 1987: six males and 2 females ranging in age from 2 to 12 years. The aetiology was blunt abdominal trauma in seven cases and idiopathic pancreatitis in one. Four children presented acutely and the diagnosis of PPC was made by US within 10 days of admission. One of these children had a diagnostic laparotomy for traumatic pancreatitis and his PPC was identified subsequently on serial US. The PPC in this child and one other resolved spontaneously after a period of total parenteral nutrition and intestinal rest. In two cases, the PPC failed to resolve and drainage was necessary. Late presentation (5–16 weeks) after recognised injury occured in four children; all required surgery within 1 week of admission. No surgical complications occured. Conservative management of PPC has been condemned because of the risk of complications occurring during the waiting period. This applies to adults who have developed a PPC in a pancreas diseased due to alcohol and/or cholelithiasis; the natural history of PPC is quite different in children. We thus recommend a trial of conservative treatment for all children who develop a PPC early, but consider that surgery is the best choice for those who present later.

Contemporary surgical treatment for choledochal cysts

Congenital choledochal cyst is an uncommon anomaly of the common bile duct, and the pathogenesis is uncertain. Although various surgical approaches have been used, cyst excision with a Roux-en-Y anastomosis has proved to be the most successful. A total of 21 patients (17F:4M) have been treated by this method with minimal surgical morbidity at the Royal Childrens Hospital, Melbourne. Diagnosis proved difficult in some cases, and in 2 patients was delayed for some years because of misinterpretation of radiological and pathological findings.

Crohn's disease : the Melbourne experience

During the years 1979–1988, 63 children (38 male: 25 female) were first diagnosed as having Crohns disease on the basis of histological findings. Twenty-nine per cent were less than 10 years old at the onset of symptoms; 40% of these cases took more than 1 year to diagnose. Most patients (59%) presented with abdominal pain and diarrhoea, but 40% presented with: a non-specific illness (14%); upper gastro-intestinal tract symptoms (14%); growth failure (6%); systemic disease (5%), or peri-anal disease (1%). There were signs of abdominal tenderness, distension, or an abdominal mass in over 50% of cases. Peri-anal Crohns disease was present in 41% and a height less than the 10th centile was recorded at diagnosis in 45%. Children were treated with a combination of prednisolone and sulphasalazine. In 16 cases a bowel resection was performed. Surgery was indicated for localised stricture formation causing obstruction and for persistence of severe disease poorly responsive to steroids, particularly in the prepubertal child with growth failure. The extent and distribution of disease influenced outcome. Twenty-four of 39 children followed for more than 3 years are either well-controlled or disease-free at present.

Ileal perforation: a late surgical complication of Henoch-Schönlein purpura

Henoch-Schönlein purpura is a systemic vasculitis which presents with a triad of rash, abdominal pain, and joint pain. Surgical complications are uncommon but include severe abdominal pain mimicking the ‘acute surgical abdomen’, intussusception, perforation of the intestine, and rarely, stricture formation. This is a case report of a child who developed a stricture with perforation some weeks after the acute episode of Henoch-Schönlein purpura.

Diagnosis and treatment of splenic torsion in a child

A case of splenic torsion in a 4-month-old child is reported. He was extensively investigated but the diagnosis was not made until laparotomy was performed. Retrospectively, the diagnosis of splenic torsion could have been made by ultrasonography and radio-isotope liver/spleen scan. It is possible that the spleen may be able to recover function and consideration should be given to detorsion rather than splenectomy.

Perianal disease in childhood Crohn's disease: frequency, characteristics, and prognostic significance

Perianal disease (PAD) occurs in 36% of children with Crohns disease. Its development may precede other manifestations of the disease (3 of 26 patients). PAD ranges in severity from anal fissures and fistulae and localised perianal abscesses to extensive ischiorectal suppuration. Skin tags also occur, and although asymptomatic, may give a valuable clue to the diagnosis of Crohns disease. Recurrent perianal or ischiorectal sepsis produce the greatest morbidity. PAD may undergo spontaneous resolution without surgical intervention. In contrast, treating patients with severe and chronic PAD may prove frustrating and result in time lost from school.

The spectrum of cervical cystic hygroma

Cystic hygromas (benign tumours of the lymphatic system or lymphangiomas) occur predominantly in the head and neck region of infants and children. They can be grouped into three prognostic categories: (1) simple cystic hygroma; (2) cystic hygroma with oropharyngeal involvement; and (3) cystic hygroma with mediastinal involvement. At the Royal Childrens Hospital, Melbourne, during a 16 year period (1973–1988) 47 of 122 (39%) patients admitted with a lymphangioma were classified as cervical cystic hygroma. Simple cystic hygromas, presenting as a lump, occurred in 33 children. In 28, uncomplicated excision was possible. Nine children presented at birth with cystic hygroma with oro-pharyngeal involvement, 5 of whom had severe respiratory distress. All 9 required multiple excisions; death occurred in 1. Cervicomediastinal cystic hygroma occurred in 5 children presenting between birth and 2 years. Mediastinal involvement was confirmed by chest X-ray. All children had thoracocervical excision without early complications; 2 had cervical recurrence.

Management of hydrohaematometrocolpos syndromes

Hydrohaematometrocolpos may occur secondary to isolated imperforate hymen, genital tract duplications, and complex urogenital sinus or cloacal abnormalities. The presenting symptoms and relation to menarche provide clues to the diagnosis. Examination of the abdomen, introitus, and vagina may confirm the diagnosis and assist in clarification of the anatomy. In genital duplications ultrasonography further delineates the pelvic anatomy and demonstrates unilateral renal agenesis. We have reviewed our 10-year experience and present an approach for the diagnosis and investigation of these patients.