Noritoshi Handa

Changing profile of abdominal wall defects in Japan: Results of a national survey

BACKGROUND/PURPOSE The incidence of gastroschisis has increased over the past 3 decades in a number of countries. To elucidate the Japanese status of anterior abdominal wall defects, the Japanese Society of Pediatric Surgeons conducted a national survey in Japan. METHODS Information was obtained by sending out a questionnaire to 192 University Hospitals, Childrens hospitals, and general hospitals that each had more than 200 beds. The characteristics of the patients including the birth date, birth weight, gestations, rate of associated anomalies, rate of antenatal diagnosis and prognosis, maternal age, gravidity, history of smoking, and drug use were analyzed. RESULTS The authors obtained answers from 149 institutions, including 1,785 cases of omphalocele and 970 cases of gastroschisis, which were treated between 1975 to 1997. There was a significant increase in the incidence of gastroschisis, from 0.131 in 1975 to 1980, 0.269 in 1981 to 1985, 0.337 in 1986 to 1990, 0.461 in 1991 to 1995 to 0.467 per 10,000 births in 1996 to 1997. The incidence of omphalocele was 0.322 in 1975 to 1980, 0.567 in 1981 to 1985, 0.657 in 1986 to 1990, 0.741 in 1991 to 1995 to 0.626 per 10,000 births in 1996 to 1997, respectively. In the omphalocele group, 43.1% of the mothers were between 25 to 29 years of age, whereas in the gastroschisis group 42.6% of the mothers were 20 to 24 years of age. In the gastroschisis group, the number of primipara mothers was larger than that of multipara mothers. In the omphalocele group, approximately 10% of the mothers smoked during each period, whereas in the gastroschisis group, the percentage of smoking mothers increased chronologically from 12.9% in 1981 to 1985, 18.7% in 1986 to 1990, 23.5% in 1991 to 1995 and 29.3% in 1996 to 1997. A history of drug use by the mother was approximately 10% for both the omphalocele and gastroschisis groups. In the omphalocele group, 55.9% had associated anomalies against 21.8% in the gastroschisis group. Approximately 10% in the omphalocele group and less than 3% in the gastroschisis group showed chromosomal abnormalities. From 1986, a significant number of cases detected antenatally has been observed. CONCLUSIONS There have been substantial changes in the incidence of anterior abdominal wall defects, particularly regarding gastroschisis in Japan. The reasons for such changes are most likely multifactorial, further epidemiological monitoring is thus called for.

Immunohistochemical distribution of surfactant apoprotein-A in congenital diaphragmatic hernia

The high mortality for congenital diaphragmatic hernia (CDH) has been attributed to a combination of pulmonary hypoplasia and persistent pulmonary hypertension. Recent experimental studies suggest that surfactant deficiency may also contribute to CDH pathophysiology. In this report, the authors immunohistochemically and morphometrically examined whether or not the hypoplastic lungs of CDH are associated with the immaturity of the surfactant system, especially alveolar type II cell function. Nine autopsy cases with CDH were immunohistochemically examined for the expression of surfactant apoprotein, using anti-IgG against human surfactant apoprotein A (SP-A), and the findings were compared with those in a gestational and postnatal age-matched control group. The lung/body weight ratio in the CDH was less than that in the controls (0.010 +/- 0.005 versus 0.021 +/- 0.013, P < .01). The radial alveolar count (RAC) of the CDH cases were also significantly less than that of the control cases (2.10 +/- 0.52 versus 3.48 +/- 0.39, P < .01). In the CDH cases, the RAC of the lung on the affected side were also significantly less than that of the lung on the unaffected side (1.71 +/- 0.34 versus 2.50 +/- 0.26, P < .01). In the immunohistochemical distribution of SP-A, compared with the control cases, the number of SP-A-positive cells on the alveolar septa of the CDH cases decreased in number, and this immunohistochemical reaction was weak even in positive type II cells. In addition, the immunoreaction observed in the alveolar type II cells of the unaffected side lungs in the four CDH cases was stronger than that of the unaffected side lungs. These results suggest that in the lungs of the CDH cases, especially on the affected side, there is a possible delay in both the structural growth and functional maturation or development of SP-A synthesis by alveolar type II cells, and this retardation of the functional maturation in alveolar type II cells is also considered to play a role in postnatal respiratory insufficiency in CDH patients.

Motility function of the esophagus before primary anastomosis in esophageal atresia

Two patients with a long gap esophageal atresia without tracheoesophageal fistula underwent an esophageal manometric study before primary anastomosis and one of them underwent an identical study after operation. Before operation both patients showed peristaltic contraction in the proximal esophagus when swallowing which was always followed by a coordinated contraction of the distal esophagus the same as in that of the normal esophagus. These peristaltic contractions induced a reflex relaxation of the lower esophageal sphincter (LES). After operation the manometric studies of the repaired esophagus demonstrated an abnormal esophageal motility, with simultaneous contractions occurring when swallowing in the proximal to the distal esophagus. In addition, the reflex relaxation of LES was incomplete. These data suggest that even if the intraluminal continuity is defective, the esophageal motility function is not disturbed in the proximal to distal esophagus in preoperative esophageal atresia, and that intraoperative mobilization and denervation may be suggested to be an important factor in esophageal dysmotility even when esophageal atresia is successfully repaired.

Yolk Sac Tumor in a Case of Testicular Feminization Syndrome

A 17 month old who had been diagnosed as having testicular feminization syndrome (noted during inguinal herniorrhaphy) was operated on because of an abdominal mass that had a high serum level of alpha-fetoprotein. Histologically, the lesion was a yolk sac tumor. The alpha-fetoprotein level normalized within 2 months of the surgery, through the administration of adjunctive chemotherapy containing cisplatinum. The patient is disease-free 4 years postoperatively. When performing inguinal herniorrhaphy in a girl, the surgeon should be prepared to deal with testicular feminization syndrome. If gonadal neoplasm is deniable at the time of diagnosis, careful follow-up examinations are needed until completion of the development of secondary sex characteristics.

Immunohistochemical distribution of bombesin-positive pulmonary neuroendocrine cells in a congenital diaphragmatic hernia

Morphometrical analyses of the immunohistochemical expression of bombesin, which is one of the peptides produced by pulmonary neuroendocrine (PNE) cells, were carried out on the bronchioles of human congenital diaphragmatic hernia (CDH) neonates, and the findings were then compared with those in a gestational and postnatal age-matched control group. As a result, no difference was found in the number of bombesin-positive cells between the lungs of the control group and the unaffected side lungs in the CDH group except for the ratio of the bombesin-positive cells per unit of the bronchiolar surface area (P<0.05). However, compared with the lungs in the control group, the affected side of the lungs in the CDH group showed a significant increase in the expression of bombesin, namely, the ratio of the bombesin-positive cells per bronchiole (P<0.05), the ratio of the bombesin-positive cells per unit perimeter of the bronchioles (P<0.05), and the ratio of the bombesin-positive cells per unit of the bronchiolar surface area (P<0.01). These results thus suggest that hyperplasia of the PNE-cell system in the lungs of the CDH cases, especially on the affected side, exists in human fetuses. We also further speculate that PNE cells may thus play a role in the problems associated with CDH during intrauterine life in human beings.

Right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis

A case of right-sided diaphragmatic hernia following group B streptococcal pneumonia and sepsis is reported herein. The clinical course was characteristic. The position of the right hemidiaphragm was initially normal. After an antecedent group B streptococcal infection, an abnormal shadow indicating either pneumonia or a pleural effusion on the chest x-ray was recognized and an elevation of the bowel and liver into the right hemithorax gradually appeared. Repair of the hernia was indicated and the postoperative result was excellent. The relationship between a delayed-onset diaphragmatic hernia and a group B streptococcal infection is still unknown. Increased intrathoracic pressure caused by mechanical ventilation coupled with an abnormal lung compliance due to inflammation may have resulted in the delayed herniation. Among various methods for diagnosis applied, chest x-ray and ultrasonography were noninvasive and useful.

Infant Cholelithiasis: Report of a Case

Cholelithiasis is an extremely unusual finding in infancy. The following article describes the case of a 2-month-old male with a VACTER association who presented with persistent and progressive obstructive jaundice and acholic stool due to cholelithiasis. Thirty cases under the age of 1, including our case, have previously been reported in the Japanese literature. Twenty-eight cases had predisposing factors. The calculi were present only in the gallbladder in 18 cases, in the common bile duct or cystic duct or both in 7 cases, and in the gallbladder and common bile duct in 3 cases. Ten cases of stones were radiopaque, which thus made the plain abdominal roentgenogram findings very valuable. Nine cases underwent operation including cholecystectomy in 3 cases, choledocholithotomy in 3 cases, and cholecystolithotomy in 1 case, while the procedure was unknown in 2 cases. Recently, the number of reported cases of cholelithiasis in infants has gradually increased and today these cases are most often diagnosed by ultrasonography, because the examination is easy to perform and not invasive.

Expression of clara cell 10-kDa protein (CC10) in congenital diaphragmatic hernia.

Abstract Clara cell 10 kDa protein (CC10) has been thought to be fairly specific to Clara cells and a major secretory protein that is both synthesized and released from Clara cells. In the present study, morphometric analyses of the immunohistochemical expression of CC10 were carried out on the bronchioles of human neonates with congenital diaphragmatic hernia (CDH) and then compared with morphometric analyses from a gestationally and postnatally age-matched control group in order to clarify the immaturity of Clara cells in CDH lungs. No difference was found in CC10 expression between the affected side and the unaffected side of the lungs in the CDH group. However, compared with the lungs of the control group, the CDH group showed a significant decrease in CC10 expression, namely, the ratio of CC10-positive cells per bronchiole, per unit perimeter of bronchiole, and per unit bronchiolar surface area. These results suggest that in the lungs of CDH cases, a possible delay in either functional maturation or the development of CC10 synthesis by the bronchioles may exist, and this retardation of functional maturation of the airway is also considered to play a role in the postnatal respiratory insufficiency observed in CDH patients.

A case of congenital intraspinal neuroblastoma

A case of congenital intraspinal neuroblastoma with paraplegia in a newborn is described, and a review of previously reported cases is presented. Calcification within the spinal canal and abdominal mass was noted. At age 75 days, the patient underwent a resection of almost the entire retroperitonial tumor. A magnetic resonance scan showed hepatic metastasis, and chemotherapy was changed to a more intensive regimen. The liver metastasis disappeared; however, neurological signs persisted. A literature review of 38 cases showed 26 patients had a laminectomy and 12 patients did not. Treatment with or without laminectomy was associated with a poor prognosis for neurological recovery, suggesting cord compression must occur antenatally, and irreversible damage occurs before birth. Congenital intraspinal neuroblastoma has a relatively good prognosis (survival rate, 91.9%), although complete neurological recovery is highly unusual. Based on these findings, the authors conclude that a laminectomy is not indicated for those patients.

Zinc and Copper Requirements During Parenteral Nutrition in the Newborn

Zinc and copper requirements and metabolism in surgical neonates on parenteral nutrition were monitored. Without a supplementation of zinc, the plasma zinc level decreased in the second week and became severe with time. In full-term babies, for up to 4 weeks of parenteral nutrition, 40 micrograms/kg/d was adequate to maintain the plasma zinc level within a normal range. For extended periods of parenteral nutrition, the dose of zinc can be reduced without excess loss of this mineral. However, premature babies in the zinc-supplemented group showed a decreased zinc level in the fourth week of parenteral nutrition, indicating that 40 micrograms/kg/d of zinc supplementation was inadequate for premature surgical babies. Infants with enterostomy require increased amounts of zinc, even up to 300 micrograms/kg/d. Copper levels revealed little change, up to 4 weeks of this feeding. In the presence of increased fluid loss through gastrointestinal fistulas, deficiency of copper occurred and the copper requirements increased. Careful monitoring is necessary to ensure an adequate supply of these minerals to surgical neonates.