Craniofacial and dental dysmorphology in patients with median facial dysplasia: long-term follow-up.

Median facial dysplasia affects a subset of patients with cleft lip and palate exhibiting certain characteristics of median facial structure deficiencies without definable gross abnormalities of the brain. The aim of this study was to describe the craniofacial and dental morphology of almost skeletally mature patients with median facial dysplasia. Patients were selected for this retrospective study if they were diagnosed with median facial dysplasia and ≥15 years old. The craniofacial and dental morphology was evaluated by analysing cephalometric and panoramic radiographs. This sample of median facial dysplasia patients (9 males and 11 females; 6 unilateral and 14 bilateral clefts) had a mean age of 16.7 ± 1.9 years. Controls were age-, sex-, cleft type-matched, and nonsyndromic patients. The results showed that in patients with median facial dysplasia, the anterior cranial base and midface were shorter than in controls. The median facial dysplasia inter-orbital distance was shorter and the nasal bone was more retrusive than in controls. All patients with median facial dysplasia had several missing permanent teeth. These features require extensive surgical, orthodontic, and dental rehabilitation procedures.

Hepatopathy-thrombocytopenia vs infection-induced hemophagocytic syndrome in Wilms' tumor: a case report.

A 3-year-old boy with Wilms' tumor, post operative left nephrectomy stage, had HTS on day 99 of the combined chemotherapy which lasted for more than 20 days. He had severe respiratory distress due to a moderate amount of ascites and marked pleural effusion. Because of high fever, thrombocytopenia and marked hemphagocytosis in the bone marrow, he received IVIG for 2 days. Normal platelet count and markedly decreased pleural fluid were attained within 3 days. He subsequently tolerated full doses of combined chemotherapeutic agents with an additional one (doxorubicin). In cases of HTS, IAHS should be suspected. The bone marrow should be done and treatment accordingly so that there is no need to decrease, the dosage of chemotherapeutic agents afterwards.

Wilms' tumor: analysis of thirty cases.

Thirty cases of Wilms' tumor who had been treated at the Ramathibodi Hospital from January 1970 to December 1982 were analysed retrospectively. There were 14 boys and 16 girls, aged 6 months to 7 years (mean age was 2 years). The right kidney was involved in 12 cases, the left side involvement in 18 cases. Other than the abdominal mass, the common signs and symptoms were hematuria (30%) and hypertension (13.3%). The congenital anomalies were found in 2 cases. There was an increase in VMA in three of the six cases determined for VMA:creatine ratio and VMA in 24 hours urine. Seven cases (23.3%) had nephrectomy done in other hospitals. Ninety percent of the patients came in with the stage II-IV, only 10% had stage I. The treatment consisted of surgery, radiation therapy, actinomycin-D, vincristine and adriamycin. Eight patients (26.6%) were lost to follow-up. The cure rate in stage II, III and IV were 71.4%, 50% and 29% respectively. The serious surgical complications include a case of shock due to excessive bleeding and another case of sudden death during the operation due to the tumor emboli from the inferior vena cava to the main pulmonary and both bronchial arteries.

Challenge of the two neural tumors: neuroblastoma and retinoblastoma.

Fifty cases of neuroblastoma and 29 retinoblastoma patients who had additional chemotherapy were analyzed retrospectively. Male:Female ratio were 1:1 and 0.8:1, the ages ranged from birth to 14 years (4 +/- 3.42 year) for neuroblastoma, and one month to 5.5 years (2.4 +/- 1.4 year) for retinoblastoma. More than 70%-90% of them came in advanced stages. The treatment consisted of surgical resection, enucleation or exenteration if feasible. Radiation therapy and chemotherapy were given as specific and palliative measures. All of the neuroblastoma who were younger than 8 months old survived long-term. Various chemotherapeutic agents did not seem to effect the outcome of the advanced cases of these two diseases. The retinoblasts did not seem to be sensitive to MTX and Ara-C. Thio-tepa intrathecally seems to be worth trying. Since these two tumors are not sensitive to treatment and are still a great challenge to the developed countries, awareness of the diseases, early diagnosis and early treatment are appropriate approaches for the developing countries.