Purpose: Pathogenesis of umbilical granuloma is not well understood. We investigated blood flow of the umbilicus in umbilical granuloma and omphalitis cases in order to elucidate the pathogenesis of umbilical granuloma and mechanisms of normal umbilical formation after birth. Methods: Intraumbilical blood flows were observed by using color Doppler ultrasound in 38 cases with umbilical granuloma and 5 cases with omphalitis during the neonatal period and early infancy. Images were checked before and after treatment. Results: In the umbilical granuloma cases, 2 to 3 streaks of pulsating blood flows passing longitudinally through the umbilicus were observed. They ended mostly within the umbilicus, although some cases were connected with abdominal walls. There were no blood flows in the medial umbilical ligaments and ligamentum teres hepatis. In the omphalitis cases, low echogenic bloodless abscess cavities with increased blood flows in their walls were observed. At the healed stage of both umbilical granuloma and omphalitis, color Doppler images showed umbilical scars of about 10-mm width without blood flow. Conclusions: At the first stage of umbilical formation after birth, blood flows of umbilical arteries and vein at the very end of umbilical cord presumably persists, and bypasses systemic circulation of the abdominal wall. Slow gradual loss of this blood flow follows, leading to the formation of umbilical scars. In a couple of weeks thereafter, total shutdown of the blood supply leads to the completion of the umbilicus. Umbilical granulomas are not the result of umbilical infection, but of the residual blood flow in the umbilicus.

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Purpose: With the increase in the number of births of extremely low birth weight infants (ELBWI) and very low birth weight infants (VLBWI), the frequency of enterostomy for ELBWI/VLBWI has increased recently. We reviewed recent enterostomies in ELBWI/VLBWI at our institution. Methods: Enterostomies were performed in a total of 32 neonates from March 2007 to February 2012, and 18 of them were ELBWI/VLBWI. The patients' records were retrospectively reviewed. Results: Of the 18 ELBWI/VLBWI, meconium related ileus (MRI) was found in 10 cases, focal intestinal perforation (FIP) in 4, necrotizing enterocolitis (NEC) in 3, and ileus suspicious of Hirschsprung disease in 1. There were no cases of enteral perforation or diffused peritonitis among the patients with MRI. A second enterostomy was required in 2 patients. All patients, except 1 with NEC, have survived to date. Conclusion: From the survival rate (94.4%) of our cases, enterostomy performed at appropriate times for each disease may lead to improvement in survival rates of ELBWI/VLBWI.

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Purpose: We examined recurrent patients who underwent post-infantile inguinal hernia surgery, reviewed conventional infantile inguinal hernia surgery, and discussed future tasks in new endoscopic surgeries. Methods: Eighteen recurrent patients who underwent previous infantile inguinal hernia surgery in our hospital from July 1998 to December 2011 were examined. The subjects were divided into two groups: eight patients who showed recurrence in childhood, and ten patients who showed recurrence after they grew up. Their disease states were examined by focusing on recurrence mode. Results: Of the eight early-recurrence patients, recurrence was associated with technical error in seven, and associated with damage to the internal inguinal ring in one. Of the ten adult-recurrence patients, recurrence was of the direct type in five, indirect type in four, and femoral hernia in one. In two of the four indirect type subjects, there was no evidence of an opened inguinal canal. In one patient where high ligation was performed without the sac being dissected, an edema was formed within the distal sac. In the other patient, although there was a postoperative trace in the internal inguinal ring, the sac was patent. Conclusions: It has been considered that in infantile inguinal hernia surgery, high ligation is fundamental and strengthening of the posterior wall is not necessary. However, when performing high ligation of the sac, the internal spermatic fascia is also reefed and cut simultaneously, providing an equivalent act to the Marcy's procedure in adult surgery. In patients where the internal inguinal ring is widely dilated and damaged, it is necessary to add reefing to the internal inguinal ring. Not adhering to high ligation and proper attention ensure that the internal inguinal ring is not excessively separated and damaged to effective prevent recurrence. Furthermore, in the endoscopic surgical technique, ligation of the peritonea alone may be necessary to prevent the indirect type occurrence after patients have grown up.

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Purpose: The purpose of this study was to analyze the clinical parameters in selecting a proper bar-size for the Nuss procedure in the repair of pectus excavatum. Methods: From December 2004 to April 2008, preoperative CT scans of 205 cases were used in the analysis. Preoperative clinical parameters included the transverse thoracic diameter (distance between outside of the bilateral ribs of preoperative CT scans at the level of the lowest point of the sternum), length between bilateral mid-axillary line, and body height. Parameters and the bar-size actually adopted were analyzed with the correlation coefficient analysis. Results: The correlation coefficient between the bar-size and transverse thoracic diameter was 0.95, the length of bilateral mid-axillary line was 0.83, and the body height was 0.92. The relationship between the transverse thoracic diameter and bar-size is described by the following equation: bar-size (inch)=transverse thoracic diameter (cm)×0.4+1.7 Conclusions: Among the 3 parameters, the transverse thoracic diameter of preoperative CT scans was most significantly correlated with the adopted bar-size; we were able to establish a standard length of the transverse thoracic diameter for each bar-size. Using this information, we can select a proper bar-size in the Nuss procedure.

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A 0-day-old female patient was referred to our hospital because of fetal distress. She was diagnosed with a left diaphragmatic hernia. After recovering from persistent pulmonary hypertension, she underwent surgical repair for the diaphragmatic hernia at 6 days of age. At that time, an extralobar pulmonary sequestration was noted in the left pulmonary cavity, and she underwent an extraction. Because of poor postoperative oxygenation, weaning was not possible. X-ray findings showed cystic shadows in the lower left pulmonary area, and computed tomography revealed cystic lung disease. At postoperative day 35, she underwent a lower left lobectomy. She was pathologically diagnosed with Stocker's type-II congenital cystic adenomatoid malformation (CCAM). Congenital diaphragmatic hernia combined with pulmonary sequestration and CCAM is extremely rare, and this suggests a reciprocal embryological relationship in their pathogenesis.

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This is a case report of Hirschsprung's disease associated with congenital central hypoventilation syndrome (CCHS) suffered from cardiac arrest due to bradyarrythmia. A 9-day-old male was transferred to our hospital because of recurrent apnea, abdominal distention, and bilious vomiting probably due to a small intestinal obstruction. An emergency laparotomy performed revealed caliber change at 15 cm proximal from the cecum. Ileostomy and appendectomy were then performed. He was pathologically diagnosed with total colon aganglionosis. After the operation, his apnea did not improve. Genetic testing showed PHOX2B mutation and polyalanine expansions; the diagnosis of CCHS was confirmed. He had previously suffered from bradyarrythmia several times, and cardiac arrest occured at the age of 112 days. However, he recovered by cardiopulmonary resuscitation and therapeutic hypothermia without neurological damage. He is now 11 months old, and he has been staying in our hospital since admission.

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A 12-year-old boy underwent thoracoscopic upper right lobectomy in the left lateral decubitus position because of bronchial atresia. His right upper arm was placed on a paddled armboard in hyperabduction of 120 degrees, because a resected right upper lobe was taken out from an axillary incision. The procedure lasted for 330 minutes. On postoperative day 1, his right upper extremity was paralyzed, the motor power was graded as 1 by the muscle manual test, and sensation was also decreased along his C5-Th1 dermatome. He was diagnosed as having total arm-type brachial plexus injury. The intensity of his right brachial plexus increased on MRI T2-imaging. His neurological symptoms gradually improved after undergoing steroid therapy and the rehabilitation program. The motor power had resolved completely 12 weeks after treatment. The brachial plexus injury is a critical complication, which directly affects the patient's quality of life. In order to prevent perioperative nerve injuries, careful positioning of the patient's arm with avoidance of hyperabduction is essential.

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Although anastomotic biliary strictures are one of the most common complications after liver transplantation, non-anastomotic (intrahepatic) biliary strictures rarely develop. The authors report a pediatric case with a non-anastomotic biliary stricture following live liver transplantation with ABO incompatibility. The patient developed dilatation of the biliary tree 3 months after transplantation. Repeated balloon dilatations following percutaneous transhepatic bile drainage were successfully performed. The patient was asymptomatic with normal liver function 63 days after stenting, although slight dilatation of intrahepatic dilatation remained. Careful follow-up is required, because non-anastomotic biliary strictures are associated with therapy resistance, higher recurrence, graft failure and the need of retransplantation.

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A male baby weighing 2,520 g was born at 35 weeks of gestation. The prenatal sonography showed polyhydroamnion and the absence of a fetal gastric bubble, suggesting type-A esophageal atresia (EA). The radiographies after birth showed a coiled-up sign of a nasogastric tube and a gasless abdomen. On the basis of these findings, type-A EA was diagnosed followed by a tube gastrostomy on day 1 postpartum. At the age of 2 months, the gap of both ends of the esophagus was 1-cm long under fluoroscopy, and there were four dimples on the upper esophageal end via endoscopic examination. Contrast study showed no fistulous tract to the lower esophagus, and that the proximal and distal regions of the esophagus were located on the left side of the mediastinum. Left thoracotomy performed at 3 months of age showed that the both esophageal pouches were connected with a 2-cm-long atretic band without fistula. The atretic band was resected, and an end-to-end esophagoesophagostomy was completed with minimal tension. On histological examination, the band consisted of bunches of smooth muscles without a lumen. One year later, he fully tolerated oral feeding and indicated weight gain. This rare case corresponds to the subtype-II_3 in Kluth's atlas of esophageal atresia.

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A newborn girl was admitted to our department because of an abdominal wall defect located in the midline 2 cm below the xiphoid process. Its size was 1×2 cm, and a portion of the liver was exposed from the defect. Moreover, the left upper abdomen was bulging. The umbilical cord and its sides were normal. Clinical examination showed no other associated abnormalities. Diagnosed as having epigastric hernia with partial abdominal wall defect, she underwent surgery at age of 4 days. As the skin edge of the defect was adhered to the liver, we performed adhesiolysis around the skin edge. The thin muscle layer around the defect was observed under the skin; the left side was thinner than the right one. The defect was closed by two-layer suturing. Other than gastroschisis, omphalocele, body stalk anomaly, and prune belly syndrome, atypical abdominal wall defects have rarely been reported. To our knowledge, there are only six cases in the literature to date. Our present case may be caused by disorders of extension and fusion of the left lateral body wall fold, migration and differentiation of the myotome derived from paraxial mesoderm during the period of organogenesis.

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A 4-year-old boy was referred to our hospital with abdominal pain and vomiting. Physical examinations revealed tenderness in the right lower quadrant. As he was diagnosed as having enterocolitis, he was admitted to our hospital and received conservative treatment. The next day, physical examinations revealed progressive abdominal tenderness with muscle guarding and rebound tenderness. Computed tomography scan revealed a dilated small intestine and a fluid-filled cystic lesion with air in the right lower quadrant. Because of findings mentioned above, appendicitis with abscess formation or duplication cyst was suspected. An emergency operation revealed torsion of the Meckel's diverticulum which formed the cystic lesion, including nonpurulent dark-red fluid. The Meckel's diverticulum was gangrenous. Diverticulectomy was performed. Presentations of torsional Meckel's diverticulum were signs of an acute abdomen with computed tomography scan and ultrasound examination showing cystic lesions. The large and long Meckel's diverticulum with a narrow neck manifested risk of torsion, and asymptomatic Meckel's diverticulum with these features should be resected.

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A male infant was delivered by cesarean section at 29 weeks and 4 days of gestation because of placenta previa totalis. His birth weight was 1,348 g and Apgar score was 9/9. After birth, he showed signs of respiratory distress syndrome and was admitted to the neonatal intensive care unit. He had one meconium stool 24 hours after birth, and his abdomen became progressively distended before feeding. Plain abdominal X-ray on day 3 showed free intraperitoneal air, and he underwent emergency surgery for suspected isolated intestinal perforation. He was hemodynamically stable and laboratory data were in the normal range. At surgery, a 5-mm-diameter punched-out perforation was found 10 cm from the ileocecal valve. The perforated site protruded as a diverticulum. A mesodiverticular band was also found. He was diagnosed with a perforated Meckel's diverticulum. He underwent resection of the affected ileum with end-to-end anastomosis. Pathological findings showed thinning and a defect of the muscular layer with a localized inflammatory reaction around the perforation without ectopic mucosa, suggesting an isolated intestinal perforation. We report here a very rare case of perforated Meckel's diverticulum in a neonate with review of literature.

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In the head and neck area, a congenital fistula of the cheek is rare, while cervical and aural fistulae are often seen. We report a case of congenital accessory parotid gland duct fistula on the cheek successfully treated by drainage in the oral cavity. A 1-year-old boy presented with a fistula opening at the facial surface of the left cheek. The fistula was located at the center of the left cheek, 15 mm posterior to the left commissure of the lips, and discharged saliva-like exudate (which increased during meals). Three accessory ears of 3 mm in diameter were also found in front of the left ear. The pyelography of the left parotid duct revealed normal left parotid gland and duct within the normal position. Fistelography revealed that the glandular tissue existed at 5 mm from the opening. The fistula had no attachment with the normal parotid gland and duct. These findings led us to make a diagnosis of congenital accessory parotid gland duct fistula on the cheek. Surgical drainage of the fistula in the oral cavity was performed. The fistula translocated to the oral cavity was open with salivary flow one year after the operation.

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This report presents a Japanese neonate with testicular torsion. He showed a painless left testicular mass at the age of 3 days. Imaging studies with ultrasonography and computed tomography suggested a testicular neoplasm. Therefore, testicular resection was performed at the age of 5 days. The pathological diagnosis of the resected testis was a hemorrhagic infarction. The cause of testicular torsion in neonates is different from those in infants or in older children. Most cases of neonatal testicular torsion are caused by twisting of the scrotal contents together with tunica vaginalis before fixation to the scrotal subcutaneous tissue. Patients with antenatal testicular torsion do not have definitive symptoms of acute scrotum and local pain. Therefore, the correct preoperative diagnosis and salvage of the affected testis is difficult. Confirmation of the testicular blood supply by color Doppler may misinterpret viability of an affected testis because of extensive blood supplies to the testis. The salvage of an affected testis may be possible if ultrasonography reveals a homogeneous pattern in the testis. It is therefore important to check for the presence of any type of contralateral testicular disorder, because bilateral testicular torsion has been reported by some authors.

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