Treatment of cystic hygroma by intralesional bleomycin injection: experience in 70 patients.

BACKGROUND/AIM: Cystic hygroma is a congenital malformation of the lymphatic system. Surgical excision is the treatment of choice, but injection of a sclerosing agent into the cyst is an alternative procedure. The aim of this study was to review the results after cystic hygroma treatment using intralesional bleomycin injection over a 16-year period at a tertiary hospital for pediatric patients in Thailand. MATERIALS AND METHODS: Medical records of patients with cystic hygroma treated by intralesional bleomycin injection between 1992 and 2007 were reviewed. Bleomycin at dosages of 0.3-0.6 mg/kg was injected into the cysts and repeated injections were performed at an interval of 2-6 weeks. Clinical outcomes were reviewed and analyzed. RESULTS: Seventy patients, 42 males and 28 females aged between one month and 14 years were treated with intralesional bleomycin injections. An excellent response (complete clinical resolution) was obtained in 33 cases (47.1%). A good response (partial reduction >50% of the mass) was achieved in 25 cases (35.8%). A poor result (no clinical response or mass reduction <50%) was noted in (17.1%). Adverse reactions including fever, local swelling, redness and pain at the site of injection occurred in 30 cases (42.9%). These reactions persisted for only a few days. Three cases died. Two girls in the poor results group died from pneumonia, respiratory failure and septicemia after bleomycin injection and partial surgical removal. The third one in the excellent results group developed thyroid carcinoma with metastasis at the opposite side to the first cystic hygroma. She died at another hospital from intracarotid artery injury with uncontrolled bleeding during left radical neck dissection. CONCLUSION: Intralesional bleomycin injection is useful for the treatment of cystic hygroma. It should be used in patients with large cystic masses and extensive invasion to reduce the risk of injury to vital organs.

Quality of life of patients with Hirschsprung's disease at 5 - 20 years post pull-through operations.

PURPOSE: The aim of this study was to evaluate the bowel habits and quality of life with respect to faecal continence of patients with Hirschsprung's disease (HD) who had undergone pull-through operations more than 5 years previously. MATERIALS AND METHODS: Four hundred and sixty-seven patients who underwent pull-through operations for HD during the period of 1987 - 1999 were followed up for evaluation. A questionnaire including demographic data and a qualitative clinical scoring method as described by Holschneider was used for the evaluation of faecal continence. The scoring system did not require a physical examination. Outcomes of the 3 major procedures (Swenson, Duhamel and Soave technique) were analysed. The research was undertaken from October 2004 to September 2006. Patients with neurological defects and total colonic aganglionosis (TCA) were excluded from the analysis. Only patients with typical HD were evaluated, and they were divided into 3 groups based on the length of the period since surgery: 5 - 10 years in Group A; 10 - 15 years in Group B; and 15 - 20 years in Group C. RESULTS: Only 204 patients (male to female ratio: 169 : 35) returned to the Queen Sirikit National Institute of Child Health for evaluation. Twenty-six patients were excluded because of TCA in 13, Down's syndrome in 9 and cerebral palsy in 4. The remaining 178 patients were evaluated and divided into Group A (n = 67), Group B (n = 75) and Group C (n = 36). Excellent results (14 points), good results (10 - 13 points) and fair results (5 - 9 points) were noted in Group A in 52.2 %, 34.3 % and 7.5 % of cases respectively, in Group B in 68 %, 28 % and 4 % of cases, respectively, and in Group C in 88.9 %, 11.1 % and 0 % of cases, respectively. Five cases (7.5 %) in Group A and 3 cases (4 %) in Group B with fair results still had problems such as constant soiling and an inability to hold back defecation. They experienced marked limitations in their social life because of their dependence on diapers and frustration because of teasing by their friends. CONCLUSION: Faecal incontinence still remains a problem in some patients with HD at 5 - 15 years after surgical correction. However, nearly all patients who were operated on more than 15 years previously had nearly normal faecal continence and a normal social life.

Wilms' tumor with metastasis to duodenum - the first reported case in Thailand.

A case of Wilms' tumor with gastrointestinal tract metastasis of the duodenum was documented in a 22 month - old - child. She had been diagnosed with Wilms' tumor stage III, treated surgically and by radiation and chemotherapy about 1 year previously. While she was still on chemotherapy, she developed hematemesis and clinical signs of partial gut obstruction. Gastroscopy and upper gastrointestinal series showed an intraluminal vascular mass in the duodenal bulb and histologically proved to be Wilms' tumor. We believe this is the first report in the world of Wilms' tumor with gastrointestinal tract metastasis.

Meconium peritonitis.

Meconium peritonitis is an unusual and often fatal form of neonatal peritonitis characterized by intraperitoneal calcification, numerous fibrosis with or without pseudocyst formation due to antenatal extravasation of meconium. This report is a retrospective study of 32 pediatric patients who were treated at the Surgical Department of the Children's Hospital from 1987 to 1996. The purposes of this study emphasize clinical manifestations, radiological findings, operative procedures and results of treatment. Twenty seven were neonates and five were older infants. The most common clinical presentation was abdominal distension at birth. The abdominal X-rays showed abnormal calcification and mass lesion in the peritoneal cavity in 71.9 and 46.9 per cent respectively. Only one patient was not treated surgically because he had no evidence of gut obstruction and inflammation. Thirty-one patients were operated on. At laparotomy, all of them had numerous inflammatory adhesion bands and matted bowel loops. Giant pseudocysts and intestinal perforations were noted in 64.5 and 54.8 per cent respectively. The obvious causes of meconium peritonitis were ileal atresia in 4, jejunal atresia in 3 and appendiceal perforation in 1. In the other 23 patients, no apparent cause of perforation was noted. Only lysis of the adhesion with or without drainage was done in 9 patients and one of these died. Partial resection of pseudocysts and exteriorization of the perforated bowel were done in 10 patients and 2 of these babies died. Primary anastomosis after resection of the perforated bowel was done in 12 patients and 5 of these cases died. The overall survival rate was 75 per cent. Our data from this study suggested that partial resection of the pseudocyst and temporary enterostomy should be done in cases with bowel perforation and severe meconium contamination. Early diagnosis, proper operative procedure and meticulous postoperative care offer the best opportunity for survival of patients with meconium peritonitis.

Choledochal cyst: review of 74 pediatric cases.

Seventy-four cases of CDC were treated at the Children's Hospital from 1977 to 1995. Female to male ratio was 5:1. Forty per cent of the patients developed symptoms within one year after birth and 75 per cent within 5 years of age. About one third of the cases were treated surgically within one year of age. Only 5 patients or 6.8 per cent had all the clinical triads of jaundice, abdominal pain and mass. Jaundice was the most common symptom in infants below one year of age but abdominal pain was the most common symptom in older children. Three newborn infants had associated biliary atresia. Established liver cirrhosis was noted during surgery in 9 patients who were operated upon within one year of age but none of the older children. All but one had either type I or type IV CDC, while the remaining one had Caroli's disease or type V CDC. Excision was the preferred treatment. Overall operative mortality rate after excision was 6.5 per cent. None of the patients who had internal drainage procedures succumbed. Infants below one year of age appeared to have high morbidity and mortality rates after surgery. Internal drainage procedure may be considered in high risk patients with advanced cirrhosis. Available information suggests that the occurrence of this disease in Thailand is probably more common than in Western countries and etiology of CDC in infants may be different from that in older children or adults.