Fine needle aspiration cytology of hepatoblastoma. A report of two cases.

BACKGROUND: Hepatoblastoma is the most common primary hepatic tumor in children. The literature contains few examples of fine needle aspiration (FNA) cytology of these tumors. CASES: A 5-month-old and 4-month-old underwent ultrasonography-guided FNA for the preoperative investigation of hepatic masses. The smears were stained with May-Grünwald-Giemsa and Papanicolaou stain. Alcohol-fixed smears were used for immunocytochemistry. All smears revealed cells with round/oval nuclei, prominent nucleoli and vacuolated cytoplasm, arranged in groups and acinar structures. The groups were embedded in a myxoid stroma. alpha-Fetoprotein was positive in all, and vimentin was positive in some tumor cells. The cytologic findings resembled the histologic counterpart in one case, and the other case agreed with the clinical/radiologic prediagnosis. Immunocytochemistry was supportive. CONCLUSION: FNA cytology can be diagnostic in many other childhood tumors as well as hepatoblastomas. Detailed descriptions of cytomorphologic features of hepatoblastoma will help FNA to be used confidently on these tumors.

A case of ambiguous genitalia with unilateral amelia and unilateral peromelia of the upper limbs.

A 7-year-old patient is reported with a 46,XY karyotype, ambiguous genitalia and unilateral amelia and unilateral peromelia of the upper limbs. The external genitalia had essentially a female configuration with labia majora, large clitoris, and narrow vaginal opening. Gonadal tissue was not palpable on either side. The levels of 17-OH progesterone dehydroepiandrosterone sulfate (DHEA-S), androstenedione and luteinizing hormone (LH) were normal, but the level of follicle stimulating hormone (FSH) was elevated minimally. Abdominal ultrasonography (USG) was normal. On pelvic USG, neither uterus nor ovaries were seen. Genitography showed a blind vagina. Gonads, Müllerian and/or Wolffian structures were not observed at laparotomy. Clitoral recession and cut-back vaginoplasty were performed. The occurrence of these findings suggests embryonic testicular regression syndrome with bilateral transverse defect of the upper limbs. The case has been presented because the pattern of the birth defects, including both ambiguous genitalia and unilateral amelia on one side of the upper limbs and unilateral peromelia on the other, have not been described previously.

Tubular duplication of the hindgut with genitourinary anomalies.

Hindgut duplication including the colon and rectum as well as the genital and urinary organs are extremely rare. Only a few cases are noted in the medical literature. In this report, a newborn with exstrophy of the urinary bladder, double vagina and uterus, double anus, and complete duplication of rectum and colon with malrotation is presented.

Neonatal peritonitis.

A retrospective study was undertaken over an eight-year period to assess the mortality rate of 66 newborns who had undergone surgery in our clinic because of peritonitis. The mortality rate for the patients admitted in poor condition was 95.2 percent, whereas it was 45.5 percent for those in good condition. There was a 100 percent mortality associated with newborns that had hypothermia and with those that had severe respiratory difficulties, whereas it was 92.3 percent for low-birth-weight infants and 86.5 percent for dehydrated infants. All babies with sclerema neonatorum died. The newborns with white blood cell counts under 5,000/mm3 also did not survive (83.3%). Etiologically, congenital megacolon, meconium ileus and spontaneous gastrointestinal perforations were the most frequent anomalies leading to death (100%). In the newborns with gastrointestinal perforations, most deaths occurred in patients with perforations of the cecum, duodenum and stomach (100%). Mortality seemed to be greater in patients with complications (77.3%), and it rose to 83.3 percent for patients who had to undergo a repeat operation due to complications. The overall mortality rate was found to be 71.2 percent.

The effect of steroid treatment on corrosive oesophageal burns in children.

A retrospective study was done on corrosive oesophageal burns to ascertain the preventive effect of corticosteroids on stricture development. Within the last 12 years 351 children were admitted to our medical centre with a history of corrosive agent ingestion. In 235 of these the diagnosis of oesophageal burn was confirmed by means of oesophagoscopy. Children admitted within the first 48 hours received steroid, antibiotic and fluid therapy while fluid and antibiotics were given, if needed, in the rest. Forty-six of the children were lost to follow-up. The type of corrosive agent, the admission period, the degree of the burn and the stricture development were used as parameters. Stricture development was found statistically significant in late admitted patients vs. early admissions. This retrospective study suggests the effectiveness of corticosteroid treatment in preventing the stricture development.

[Hydatid cysts in children]. / Les kystes hydatiques des enfants.

84 cases of hydatid cysts in children are reported. Presenting sings were non-specific in more than 1/2 the children. Pulmonary localisation predominated and the chest X-ray alone gave the diagnosis in 47 cases with pulmonary cysts. Several organs were involved in 17 cases. Abdominal ultrasound was routinely performed for pulmonary presentations. CT scan was reserved for parenchymal lesions in order to quantify the exact number of cysts present. 107 procedures were performed. In the majority of cases conservative surgery was involved; endocystectomy for pulmonary cysts and partial cystectomy with capitonnage for abdominal cysts. Mebendazole treatment was not considered to be an alternative to surgical treatment and was only used in specific cases.

Injuries of the vulva and vagina in childhood.

Injuries of the vulva and vagina are relatively rare in children. Over a seven-year period, we treated 45 girls. The most common etiologic factor in our study group was trauma. While 28 had only vulvar lesions, the rest had injuries of both the vulva and vagina. Thirty-two children were treated surgically for only vulvar and vaginal injuries. However, additional organ treatment was mandatory for 13 children.

A symmetric double monstrosity.

A newborn was admitted to the hospital with a set of accessory lower limbs attached to the distal sternum in addition to omphalocele, intestinal malrotation and interventricular defect. The parasitic twin was removed successfully and the omphalocele was left for conservative treatment.

Idiopathic postoperative intussusception.

During the last 11 years idiopathic postoperative intussusception developed in ten children. The incidence was 5.5% of all the intussusceptions. The age ranged from three months to ten years and the interval between two surgical procedures differed from three to eight days. Only one of the patients presented the classical signs of intussusception. In nine of them, the obstructions were in the small bowel. Nine of the ten intussusceptions needed simple manual reduction. Postoperative recovery was uneventful.

Mortality due to trauma in childhood.

Mortality rates and factors affecting the mortality rates were studied retrospectively in 390 children with abdominal and/or thoracic trauma. Age groups, admission time, additional system injuries, and mortality rate were used as parameters. The severity of trauma injury was evaluated according to the Modified Injury Severity Scale. The mean mortality rate in all the groups was 7.9%. The rate was 6.7% for thoracic, 7.5% for abdominal, and 14.7% for combined trauma. This study demonstrates that early admitted children, children with additional organ injuries, and MISS scores greater than 25 have much more risk of mortality, and the mortality rate in preschool children is not different from the other groups.

Complete duplication of the colon.

A case with complete tubular duplication of the colon is presented. The patient was a 1-year-old girl who had had defecation problems for 3 months. Successful surgical treatment was achieved by closing the vaginal fistula and creating an oval window on the common wall of the duplicated colon.