Long-term outcome of anorectal malformations: the patient perspective.

To assess the long-term outcome of surgery for anorectal malformations (ARM) from the patient's perspective. One hundred and sixty seven children were operated for ARM between 1982 and 2000. Disease impact questionnaires to assess both clinical and psychological outcomes were sent to 137 eligible families. Statistical analysis was performed using SPSS version 11.5. The response rate was 61% (n=84). Sixty six percent had soiling. There was no significant difference in the incidence of soiling between genders or between the younger child and adolescent. Soiling was significantly increased in high (86%) and intermediate (79%) compared to low (43%) malformation (P=0.001). Constipation was seen in 62% and abdominal pain in 49%, with no significant difference between malformation levels. Overall, 71% had associated anomalies. Although 44% had a documented urological abnormality, clinical significant problems were seen in only 30%. Eighty percent of the children had one or more behavioural problems and 15% expressed suicidal thoughts. ARM had a negative impact on the social life of the child in 52% and on family functioning in 50%. Soiling (P=0.000), presence of associated anomalies (P=0.001), constipation (P=0.005), level of ARM (P=0.015) and abdominal pain (P=0.039) correlated significantly with psychosocial morbidity. Despite these findings, 62% of adolescents and 71% of children below 12 years with their parents reported above average global hopefulness score, remaining hopeful for the future. Children with ARM have ongoing physical and social morbidity, indicating the need for continuing multidisciplinary review and support to optimise their quality of life.

Laser Doppler imaging of paediatric burns: burn wound outcome can be predicted independent of clinical examination.

This study sought to assess the validity of independent, blinded reporting of Laser Doppler Imaging (LDI) prediction of burn wound outcome in children. Two experienced paediatric burn consultants were invited to report on LDI scans performed routinely within 3 days of burn. They were provided with the LDI flux image, a low-resolution colour digital photograph of the burnt area and a basic history. Report predictions were correlated with outcome. Reports were compiled on 50 scans performed on 31 patients at a mean of 54h post burn. Of the 100 reports generated, mean correlation with outcome was 97%. If the LDI predicted a deep burn, it was always correct. Non-correlations were due to a number of factors including inadequate scanning of the affected area, excessive movement and residual wound debris. Accurate prediction of burn wound outcome could be made via the standard information generated by LDI and appeared more reliable than clinical prediction. The correlation improved with increasing experience with LDI.

Colonic duplication: diagnostic dilemma.

Colonic duplication is a rare congenital anomaly which presents as a diagnostic and therapeutic challenge. We report a 5-year-old boy with colonic duplication who required multiple hospital admissions, multiple diagnostic tests, and evaluation by various clinical specialists before the diagnosis was made intraoperatively. He was known to have left renal hypoplasia, low spinal abnormalities, hypoplasia of the left hemipelvis, and mild hypoplasia of the left lower limb, all of which were considered to be a variant of caudal regression syndrome.

Recurrent parotitis of childhood.

BACKGROUND: Recurrent parotitis (RP) of childhood is a rare condition of unknown aetiology, probably immunologically mediated. OBJECTIVE: To review the clinical presentation, diagnosis and management of RP of childhood. METHODS: Retrospective study from 1983 to 2004 of children diagnosed with RP of childhood at a tertiary children's hospital. RESULTS: We identified 53 children, 37 (70%) male and 16 (30%) female. The age of onset was biphasic, with peaks at 2-5 years of age and at 10 years. The commonest symptoms were swelling (100%), pain (92.5%) and fever (41.5%). Symptoms usually lasted 2-7 days with a median of 3 days. The mean frequency was 8 episodes per year. The diagnosis was often delayed, >1 year in 70% of patients, maximum 8 years. The most common diagnoses, before the definitive diagnosis of RP, were mumps (21%), 'infection' (15%) and stones (11%). Sialogram (57%) and/or ultrasound (41%) showed sialectasis in 81% of patients. Over half the patients (54%) were given antibiotics at least once to treat the parotitis. Two children had hypogammablobulinaemia, one child had human immunodeficiency virus infection, and one child had Sjogren's syndrome. Two children had high titre antinuclear antibodies. CONCLUSIONS: Recurrent parotitis had a biphasic age distribution. The major clinical features that distinguish it from other causes of parotid swelling are the lack of pus and recurrent episodes. A clinical diagnosis can often be confirmed by ultrasound. Antibiotics do not have a role in treatment. Affected children should be screened for Sjogren's syndrome and immune deficiency.

Biliary dyskinesia: is the problem with Oddi?

We report our experience of cholecystectomy for treating symptoms suggestive of biliary disease in association with a decreased gallbladder ejection fraction (GBEF) but without evidence of cholelithiasis. Five children with normal biliary ultrasounds were evaluated between January 1990 and December 2000 for recurrent upper abdominal pain. Based on a cholecystokinin (CCK)-provoked GBEF of less than 50% and the absence of any other gastrointestinal pathology, patients underwent cholecystectomy with operative cholangiography for presumed biliary dyskinesia. Pathological examination demonstrated chronic inflammation in all cases. Two patients had complete resolution of their symptoms, but three had persistent pain following surgery. Biliary dyskinesia seems an uncommon cause of persistent abdominal pain in childhood. Cholecystectomy was not always effective in relieving symptoms. Biliary scintigraphy with CCK provocation should not be used as the sole criterion for cholecystectomy. Sphincteric manometry may be valuable in the assessment of this small group of patients to avoid inappropriate intervention. The future perhaps lies in better understanding of the physiological action and pharmacological control of the sphincter of Oddi.

Small bowel perforation in the premature neonate: congenital or acquired?

To determine the potential aetiological factors of small bowel perforation in the premature neonate, we performed a retrospective chart review of those neonates with spontaneous intestinal perforation (SIP) of the small bowel seen in our tertiary paediatric hospital between January 1980 and December 2000. Data were collected on gestational feto-maternal health, medical interventions prior to perforation and the subsequent operative and laboratory findings. There were 23 patients with SIP of the small bowel over the 21-year review; 65% were male. There were 7 twin pregnancies but no cases linked to maternal drug abuse. The median gestational age was 27 weeks, the median birth weight 973 g, 19 neonates required ventilation, 15 steroids and 13 indomethacin. The median age at diagnosis was 7 days, heralded by rapid development of abdominal distension in 22 patients. Surgical intervention in addition to insertion of a peritoneal drain was required in 19 patients. Positive microbiological cultures of blood or peritoneal fluid at operation were documented in 8 patients; 5 grew Staphylococcus epidermidis and 4 Candida species. Perforations were located in the ileum in 20 and the jejunum in 1. Deficiency of the muscularis propria was found in 6 patients. Of the 6 deaths, 2 neonates had significant co-morbidity in addition to extreme prematurity. Small bowel SIP occurs in the premature neonate after the first week of life and usually presents with abdominal distension. Putative risk factors identified included twin gestation, neonatal ventilation, use of steroids and indomethacin, infection with Staphylococcus epidermidis and Candida species and deficiency of enteric smooth muscle.

Laser Doppler imaging prediction of burn wound outcome in children.

The ability of laser Doppler imaging (LDI) to evaluate burn depth in children was investigated. Fifty-seven patients were prospectively studied over a 10-month period. Each patient was clinically assessed, photographed and independently scanned between 36 and 72 h of the burn. Patients were reviewed until wound healing had occurred within 12 days or skin grafting had been performed. The median age was 1 year and 10 months (range 5 months to 15 years and 8 months). The median body surface area burnt was 7.0% (range 0.5-30%). In 30 patients, the burn did not heal within 12 days, 17 of which were grafted. Clinical examination correctly determined 66% of deep partial or full thickness burns between 36 and 72 h of injury compared to 90% using LDI. The LDI was also more specific; correctly diagnosing 96% of superficial partial thickness burns as opposed to 71% on clinical examination. Moderate degrees of movement did not appear to limit the accuracy of the scan.

Atypical mycobacterial disease in children: a personal series.

Atypical mycobacterial disease is common in children in Australia. Over 22 years, records were kept prospectively by the senior author. The diagnosis was confirmed in 118 patients, either by culture or by the combination of a positive skin test plus typical histology. There were 46 boys and 72 girls with a median age at diagnosis of 28 months. Most children (n = 56) presented with chronic lymphadenitis or abscess formation (n = 55). The duration of illness varied from 4 days to 18 months. The most common sites affected were the head and neck (n = 112), with the pre-auricular region and anterior end of the submandibular triangle being characteristic. Nine patients had multifocal disease. The aim of treatment is to excise as much of the infected tissue as possible: 47 children had node excision through a planned incision that was closed primarily, with only 4 needing a second operation; 42 had excision of a node through the base of the superficial part of a collar-stud abscess with 6 recurrences. However, of the 33 children who had only drainage/curettage of the cavity or node 10 had recurrences requiring re-operation. Only 1 patient required a third operation. Morbidity was extremely low, with 1 staphylococcal wound infection. No child suffered permanent paresis of the mandibular division of the facial nerve. It is our belief that surgical excision of both the macroscopically affected and adjacent macroscopically unaffected nodes is necessary to achieve cure in the majority of cases.