Reference growth curves for normal appendiceal diameter in childhood.

The aim of this study was to investigate the relationship between the appendiceal diameter (AD) and age, sex, height, and body weight in children and to verify how does the normal AD grows in childhood. We evaluated the AD of patients younger than 16 years of age who underwent laparoscopic surgery at our hospital. We statistically examined the relationship between the AD and the age, sex, height, and weight. A final cohort of 188 patients participated in the study. The median AD for the sample population was 5 mm (range, 3.2-8.1). There was no significant difference in the AD between males and females in the multivariate analysis (P = 0.500). There was a positive correlation between the age and the AD (R = 0.396, P < 0.001). The AD had a significant positive correlation with the height and weight (P < 0.001, P < 0.001, respectively). The reference curve with regard to the AD can be useful in clinical situations, although it should be kept in mind that the range of individual differences in AD is large, and the growth degree by age is not uniform during childhood.

Neuroblastoma.

Neuroblastoma is one of the most common solid tumors in children and has a diverse clinical behavior that largely depends on the tumor biology. Neuroblastoma exhibits unique features, such as early age of onset, high frequency of metastatic disease at diagnosis in patients over 1 year of age and the tendency for spontaneous regression of tumors in infants. The high-risk tumors frequently have amplification of the MYCN oncogene as well as segmental chromosome alterations with poor survival. Recent advanced genomic sequencing technology has revealed that mutation of ALK, which is present in ~10% of primary tumors, often causes familial neuroblastoma with germline mutation. However, the frequency of gene mutations is relatively small and other aberrations, such as epigenetic abnormalities, have also been proposed. The risk-stratified therapy was introduced by the Japan Neuroblastoma Study Group (JNBSG), which is now moving to the Neuroblastoma Committee of Japan Children's Cancer Group (JCCG). Several clinical studies have facilitated the reduction of therapy for children with low-risk neuroblastoma disease and the significant improvement of cure rates for patients with intermediate-risk as well as high-risk disease. Therapy for patients with high-risk disease includes intensive induction chemotherapy and myeloablative chemotherapy, followed by the treatment of minimal residual disease using differentiation therapy and immunotherapy. The JCCG aims for better cures and long-term quality of life for children with cancer by facilitating new approaches targeting novel driver proteins, genetic pathways and the tumor microenvironment.

High aromatase activity and overexpression of epidermal growth factor receptor in fibrolamellar hepatocellular carcinoma in a child.

An 11-year-old boy was admitted with a liver tumor and underwent right trisegmentectomy for a diagnosis of fibrolamellar hepatocellular carcinoma. He had suffered from bilateral gynecomastia for a year, which improved after complete resection of the tumor. The tumor cells had significant aromatase activity (8.03 pmol/g/h) and contained high levels of estradiol (82.1 pg/mL), which contributed to gynecomastia. Furthermore, overexpression of epidermal growth factor receptor was determined in the tumor cells, which suggests that antitumor strategies using epidermal growth factor receptor antagonists may be effective.

Abdominal lymphangioma in children: report of three cases.

Most cases of abdominal lymphangioma are asymptomatic. However, patients may occasionally present with acute abdomen because of an intestinal obstruction or peritonitis caused by infected cysts, hemorrhaging, and/or torsion. These conditions may differ based on the location of the lymphangioma and do not always require emergency surgery. This report presents two cases of abdominal lymphangioma derived from the omentum, which required emergency surgery because of severe abdominal distension and peritonitis caused by torsion, respectively. In contrast, another case of retroperitoneal lymphangioma presented with acute abdomen and underwent elective surgery after conservative therapy for peritonitis. Elective surgery is acceptable if the symptoms are controllable under conservative therapy. However, it is important not to overlook the possible lethal complications such as intestinal and/or urological obstruction, aggressive peritonitis, and torsion of the cyst, which require emergency surgery.

Subcutaneous emphysema, pneumomediastinum, pneumothorax, pneumoperitoneum, and pneumoretroperitoneum by insufflation of compressed air at the external genitalia in a child.

A 7-year-old girl with concurrent subcutaneous emphysema, pneumomediastinum, pneumothorax, pneumoperitoneum, and pneumoretroperitoneum arrived at our facility. Compressed air at 5 atm of pressure was insufflated through the nozzle of a spray gun over her external genitalia. She was admitted for a small amount of genital bleeding and significant subcutaneous emphysema extending from the cheek to the upper body. Radiographic examination of the abdomen was suggestive of a visceral perforation, but she was managed conservatively and discharged in satisfactory condition without surgical intervention. The female genitalia possibly served as the entry point for air into the retroperitoneum and peritoneal cavity, with subsequent migration of air through the esophageal hiatus that resulted in pneumomediastinum, pneumothorax, and extensive subcutaneous emphysema.

Infantile epididymitis with calcification.

A 1-month-old infant presented with a case of calcifying chronic epididymitis. Differential diagnosis was made from a testicular torsion and neoplasm. Serial ultrasound examination revealed a calcified lesion adjacent to the normal testis, thereby avoiding an unnecessary orchiectomy. Infantile epididymitis has been thought to be rare; however, it is occasionally encountered in the literature and calcification with chronic epididymitis in an infant has not been previously reported. On the other hand, an infant with scrotal calcification should be suspected of neoplasm. However, the tumor markers alpha-fetoprotein (AFP) and Human Chorionic Gonadotropin beta (HCG beta) were within the physiological range. Therefore, a diagnosis must be carefully made to avoid an unnecessary orchiectomy.

Ultrasonographic serial measurements of the morphologic resolution of the pylorus after Ramstedt pyloromyotomy for infantile hypertrophic pyloric stenosis.

OBJECTIVE: The purposes of this study were to evaluate the accuracy of the ultrasonographic measurements used during diagnosis of hypertrophic pyloric stenosis (HPS), to examine the relationship between the palpability and the morphologic features of the pyloric mass, and to show the morphologic resolution of the pylorus after pyloromyotomy. METHODS: One hundred three infants with the diagnosis of HPS by clinical findings and ultrasonographic measurements were treated surgically by Ramstedt pyloromyotomy. The measurement parameters were the muscle thickness, diameter, and channel length of the pylorus. The serial measurements were performed postoperatively for 1 year after surgery. RESULTS: The preoperative ultrasonographic measurements of the pylorus were significantly correlated with the actual measurements obtained by surgery (P<.05). The diameter correlated most significantly with the palpability of the pylorus. In contrast to the prompt improvement of clinical symptoms, the muscle thickness did not reach the normal range (approximately 2 mm) until 8 months after surgery. The diameter had not decreased to its normal range (approximately 10 mm) by the end of the 1-year postoperative follow up period. The channel length decreased more rapidly than the other 2 parameters. CONCLUSIONS: The preoperative ultrasonographic measurements of the pylorus reliably evaluated the pyloric muscle thickness and diameter in HPS. The actual pyloric diameter correlated with the palpability of the pyloric mass. The morphologic resolution of the pylorus after surgery did not correlate with the prompt improvement of symptoms.