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 Children's 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. Offprint requests to: N. A. Myers     

Treatment of burns during the first trimester of pregnancy by sulfamylon and silver nitrate

A patient who suffered 30 per cent third-degree burns about one week after becoming pregnant was treated with Sulfamylon (mafenide) and silver nitrate for 20 weeks until abortion. Pathological examination of the fetus showed no abnormalities, suggesting that Sulfamylon and silver nitrate are safe drugs for the treatment of burns sustained in the first trimester of pregnancy.     

Combined anterior abdominal wall, sternal, diaphragmatic, pericardial, and intracardiac defects: a report of five cases and their management.

Five cases are described of an uncommon anomaly consisting of defects of the anterior abdominal wall, sternum, diaphragm, and heart. The anatomy, etiology, embryogenesis, associated noncardiac anomalies, and treatment of the condition are discussed. The occurrence of asphyxiating thoracic dystrophy with the syndrome, observed in one case, has not been previously documented. Three of the five infants in this series survived.     

Forearm fractures in children. Single bone fixation with elastic stable intramedullary nailing in 20 cases

We present our experience with elastic stable intramedullary nailing (ESIN) used in the single bone fixation of both bones forearm fractures in children. From May 2002 to July 2004, 20 children (14 boys and 6 girls), median age of 10 years (range 6–15 years) were treated with ESIN for 16 closed and 4 grade I open forearm fractures. All patients were reviewed clinically at a median follow-up of 20 months (range 6–30 months). All fractures were radiologically united at a median of 6.7 weeks (6–9 weeks). The median operating time was 35 min (range 25–60 min). The median hospital stay was 2 days (range 1–3 days). Removal of the nails was undertaken in all 20 children at a median of 19 weeks (range 16–24 weeks) post-operatively. At follow-up, a full range of elbow and wrist movements were found in all cases. There was no clinically significant rotational deformity in any case. ESIN seems to be a safe method in the treatment of single bone fixation of both bones forearm fractures in children between 6 and 15 years of age.     

The utilisation of intraosseous infusion in the resuscitation of paediatric major trauma patients

Intraosseous lines are a reliable and rapid tool for obtaining vascular access in emergency situations, particularly in children. Their use is recommended when intravenous access cannot be easily secured and there is a need for fluid or pharmacological resuscitation. Training in this technique is included in the Advanced Trauma Life Support (ATLS) and Advanced Paediatric Life Support course (APLS) provider courses. The objective of this study is to analyse the national use of intraosseous lines in paediatric trauma in England and Wales. Data has been collected from the Trauma Audit and Research Network (TARN) group longitudinally over 14 years from 1988 to 2002. From 23,489 paediatric trauma cases, intraosseous lines were used in only 129 patients. Compared with the remainder of the paediatric data, we found that these were the younger (1-6 years), more severely injured patients (higher ISS, lower GCS, higher head, thorax, and abdominal AIS). The mortality of these patients was high at 64% compared with 4% overall. IO line use was greater in general than in Paediatric hospitals, perhaps due to good intravenous access skills in paediatric centres. We recommend that intraosseous line use should be a skill available to everybody involved in paediatric trauma resuscitation, particularly those who may not have refined paediatric intravenous cannulation skills.     

Partial hypoxanthine-guanine phosphoribosyltransferase deficiency presenting as acute renal failure

Hyperuricemia and secondary urate nephropathy are uncommon in the paediatric setting outside of tumour lysis syndrome. We describe the case of a 12-year-old boy who presented at 3 years of age with acute renal failure. The cause of this remained unknown until the development of uric acid renal calculi 9 years later. This, and the availability of the previously unknown family history, provided the subsequent diagnosis of partial hypoxanthine-guanine phosphoribosyltransferase (HPRT) deficiency. Detailed family history is important for early detection of this heterogeneous group of disorders. Early treatment may minimise long-term renal morbidity and mortality from renal insufficiency.     

Problems and pitfalls of transition from paediatric to adult renal care

Transition is a generic issue for subspecialties dealing with chronic illness and has received little attention to date. Transfer to adult care occurs at the end of a transition process that must be individualised for each patient and takes into account all aspects of growth and development, which may be variably impaired. Good communication with the young person, family and adult nephrologist is essential so that the anxieties of all are properly addressed. Non-compliance with treatment, particularly prevalent in adolescents, requires attention to psychological and social issues as well as medical factors. The young person must have sufficient self-management skills (which should be assessed) and there should be plans for long-term social support before transfer. Transition should be a positive process and models need to be evaluated.     

Nasotracheal intubation in diphtheria

Nasotracheal intubation can be used effectively for the relief of upper airway obstruction in dephtheria. It has many advantages over tracheostomy; it avoids an operation and it eases anxiety of the parents. Of 57 patients so treated, 50 recovered without injury to the larynx in any patient.     

Colorectal cancer with neuroendocrine differentiation in a child

Colorectal cancer is extremely rare in children and presents with a poor prognosis because of the delay in diagnosis and lack of histological differentiation. We report a case of a sigmoid colon carcinoma with areas of neuroendocrine cells in a 12-year-old patient without familial occurrence of colorectal cancer. Symptoms at presentation were anaemia, anorexia, abdominal pain and weight loss. The patient was treated with radical resection and adjuvant chemotherapy. One year later, a local recurrence and hepatic metastases were diagnosed and she underwent chemotherapy and surgical resection. Twenty-six months from initial diagnosis she is alive with evidence of disease. The clinical presentation, diagnosis and treatment of the previously reported cases of colorectal cancer in children are also reviewed.     

Surgical complications and outcome of paediatric liver transplantation: the Singapore experience

The purpose of this study was to analyse the early and late results of paediatric liver transplantation (LT), with particular reference to complications that required surgical intervention. The charts of all children who underwent LT between 1990 and 2002 were reviewed retrospectively. Results were analysed with a minimum follow up of 9 months. Thirty-five children have undergone 38 LTs; 22 received grafts from their parents, 16 received cadaveric organs and three children had retransplantation. The ages of the children ranged from 12 to 168 months. Biliary atresia was the most frequent indication for transplant (n=27). Twenty-seven children had complications that required surgical or radiological interventional procedures. Vascular complications included hepatic artery thrombosis (n=2), hepatic vein (HV) thrombosis (n=1), and the majority being portal vein thrombosis (n=6). Bile leaks were observed in eight children. Other complications included intestinal perforation (n=2), intra-abdominal abscesses (n=1), wound dehiscence (n=2), post-operative bleed (n=2), intestinal obstruction (n=2), ventral hernia (n=1), and multiple abdominal wound sinuses (n=1). Three children underwent retransplantation, two for hepatic artery thrombosis with multiple episodes of cholangitis and intrahepatic biliomas and the third was done for hepatic vein thrombosis. Patient and graft survival at 1 year is 81.5 and 74.2%, respectively. Paediatric LT is associated with significant morbidity, the main complications being vascular and biliary. The article was presented at the 8th Congress of the Asian Society of Transplanatation (8th CAST) in September 2003 held in Kuala Lumpur, Malaysia.