Purpose: We reviewed the cases of patients who underwent umbilicoplasty (VY flap) with a reverse Y-shaped incision at our hospital and evaluated its therapeutic results and usefulness.
Methods: We retrospectively evaluated sex, age at surgery, birth weight, gestational age, comorbidities, operation time, and postoperative complications in 191 patients who underwent umbilicoplasty during the 15-year period from April 2004 to November 2018. In addition, we conducted a questionnaire survey about patient satisfaction.
Results: Umbilicoplasty was performed in 183 patients before they entered school. The most common complication was inguinal hernia (n = 18; 9.4%). Periumbilical dermatitis was the most common postoperative complication (n = 9; 13.8%). Only three patients (4.6%; two with exudate and one with dermatitis) required ointment treatment. Most of the patients with complications recovered without treatment. Among the 68 patients who completed the questionnaire survey, 48 described their umbilical appearance as “satisfactory”. In the comparison between the satisfied group and the unsatisfied group, there were significant differences in age at surgery and sex. The patients in the satisfied group were older and the group had a higher proportion of boys. Five children (average age, 7.6 years) responded that they themselves wanted to undergo the operation.
Conclusions: Satisfaction with the surgical procedure at our hospital was fully evaluated. Currently, there are various procedures for umbilical hernia. However, it seems that the appropriate timing of operation may be different for each operative procedure.
Purpose: Children undergoing surgery develop intense anxiety and fear when they are separated from their parents at the operating room. Preoperative anxiety has been associated with difficulty in the induction of anesthesia. In our hospital, we allow the parents to be present during the induction of anesthesia to treat preoperative anxiety in children. Parents and children were asked to complete a questionnaire to determine the effect of parental presence on preoperative anxiety.
Methods: Participants were 100 consecutive pediatric patients who underwent scheduled surgery under general anesthesia from April to October in 2017 and were accompanied by their parents in the operating room during the induction of anesthesia. The children and parents’ anxiety was evaluated by one pediatric surgeon. The questionnaire was administered one week after their surgery.
Results: The average age was 4.7 years (1 month to 15 years). Of these children, 37% undergoing surgery developed anxiety, among which 18% developed intense anxiety upon entering the operating room. Children aged 1–5 years were most likely to develop anxiety; 8% of them cried and 26% cried and tried to escape from the operating room during the induction of anesthesia. Parental anxiety was associated with concomitant increase in the child’s anxiety. The postoperative questionnaire collection rate was 89%, and 99% of parents answered that they were happy to enter the operating room with their children. Eighty-one percent of children over the age of five answered they were relieved to be accompanied by a guardian.
Conclusions: Parental presence relieved children’s anxiety, but parental anxiety had a significant effect on the child’s preoperative anxiety.
Purpose: We investigated the clinical characteristics of congenital esophageal stenosis and whether balloon dilation is useful as first-line therapy for all types of congenital esophageal stenosis.
Methods: We retrospectively analyzed the medical records of patients with congenital esophageal stenosis, who were treated at three hospitals including the Osaka Women’s and Children’s Hospital between January 2001 and December 2018. We reviewed esophagographic findings, treatments administered and clinical outcomes.
Results: This study included 31 patients (21 men and 10 women). Associated anomalies were found in 23 patients, among whom 19 presented with tracheoesophageal fistula. Initial therapies included balloon dilation in 20 patients, primary radical surgery in two, and endoscopic radial incision in one. Among the 20 patients who underwent balloon dilation, 19 were successfully treated; however, one required subsequent radical surgery. Angles formed by the esophageal wall were smaller in patients with tracheoesophageal fistula than in those without this anomaly. Esophageal perforation secondary to balloon dilation occurred in 10 patients (50%); however, all patients were successfully treated using conservative therapy.
Conclusions: Balloon dilation is useful as first-line therapy for congenital esophageal stenosis regardless of the disease types, clinical characteristics and association with tracheoesophageal fistula.
Sex cord tumor with annular tubules (SCTAT) is a rare borderline malignant tumor compared with other sex cord-stromal tumors. This tumor often produces female sex hormones and is known to complicate Peutz-Jeghers syndrome (PJS). The patient was a girl aged 9 years and 6 months who was brought to our hospital presenting with chief complaints of breast enlargement and atypical genital bleeding. An approximately 11-cm serous cystic lesion was present in the left ovary and the serum estradiol (E2) level was high. The lesion temporarily shrank and the E2 level also decreased during the course of observation, but the lesion re-enlarged thereafter. Suspecting a reactive ovarian cyst, we performed partial ovarian cystectomy and the lesion was histopathologically diagnosed as SCTAT. No standard treatment for SCTAT has been established. In this patient, to preserve fertility, adnexectomy was performed only on the affected side seven months after partial ovarian cystectomy. Since the patient’s case was not complicated by PJS and it has been reported that about 20% of noncomplicated cases follow a malignant course, a strict course of observation will be necessary hereafter.
Progressive familial intrahepatic cholestasis (PFIC), a rare cause of hepatic failure, is characterized by progressive cholestasis that begins in infancy. We report two cases of PFIC in patients who underwent living donor liver transplantation (LDLT) to treat this condition. This is the first report on LDLT performed for PFIC type 4 in Japan. [Case 1] An 11-year-old male adolescent underwent LDLT for PFIC type 1 secondary to deterioration of liver function following infectious enteritis. Although he underwent LDLT with complete biliary diversion, biliary secretion was inadequate, and he died 6 months after LDLT. [Case 2] A 4-year-old girl with PFIC type 4, who was initially clinically diagnosed with PFIC type 2 associated with the absence of bile salt export pump activity despite no significant genetic abnormality in PFIC2, underwent LDLT to treat her liver failure. Her postoperative course was uneventful, and she showed normal growth without any complaints over 6 years after LDLT. Although she was initially clinically diagnosed with PFIC type 2, she was finally diagnosed with PFIC type 4 on the basis of results of repeat genetic tests after LDLT, which showed a significant mutation in TJP2. The genetic diagnosis of PFIC is important in determining the type of disease for liver transplantation and other treatments.
We performed the excision of the entire extrahepatic duct and the gallbladder with hepaticojejunostomy for congenital choledochal cyst with an aberrant right hepatic artery (ARHA), which was a unique right hepatic artery arising from the gastroduodenal artery. Our patient was a two-year-old boy presenting with abdominal pain and postprandial vomiting. He was admitted because an abdominal ultrasound scan revealed acute cholecystitis. Contrast-enhanced computed tomography (CT) showed congenital choledochal cyst (type IV-A) with an ARHA, the accessory right hepatic artery, arising from the gastroduodenal artery and passing through the front of the dilated bile duct. The elective excision of the entire extrahepatic duct and the gallbladder with hepaticojejunostomy was performed and the ARHA was preserved successfully. His postoperative course was uneventful and he was discharged seven days after the surgery. The preoperative evaluation of vascular transit and careful surgical maneuvering enable the preservation of the ARHA.
The subglottic cyst is relatively rare. A boy born at 28 weeks of gestation had a birth weight of 1,202 g and required five days of endotracheal intubation during the neonatal period. At one year and two months of age, he developed a severe respiratory obstruction resulting in cardiopulmonary arrest, which required resuscitation. Flexible bronchoscopy under general anesthesia with a laryngeal mask revealed a small cyst on the right side of the subglottic space, but it was not considered to have been responsible for the critical airway obstruction. He showed frequent episodes of croup-like symptoms and was referred to us again at three years of age. Enhanced computed tomography showed a 5-mm cystic mass close to or inside the subglottic portion of the trachea, indicative of a subglottic cyst inside the trachea or a bronchogenic cyst compressing from the outside. We intended to excise the cyst via a cervical incision. As the cyst was not located outside the trachea, we performed meticulous unroofing of the subglottic cyst via laryngofissure with distal tracheotomy. During the follow-up period of one year, there was no recurrence of the cyst. However, another small cyst was found in the left subglottic area, but it did not increase in size during the subsequent six months of follow-up. One year and five months after tracheotomy, we cauterized the cyst using a diode laser and successfully decannulated the tracheostomy. We herein report our experience with the diagnosis and treatment of this patient with a subglottic cyst who had a somewhat unusual presentation of airway obstruction.
Some children with mild congenital tracheal stenosis (CTS) who are treated conservatively eventually develop exercise intolerance. We present the cases of two children with exertional dyspnea in mild CTS. The six-minute walk test (6MWT) was useful for pre- and postoperative evaluations of exercise capacity in these children. Case 1. A 6-year-old boy with CTS without a life-threatening event complained of exertional dyspnea while playing with other healthy friends. 6MWT showed that he was able to walk 351 m, which is significantly short for his age. He underwent tracheoplasty. His postoperative course was uneventful. 6MWT showed that he was able to walk 451 m on postoperative day (POD) 26 and 541 m after seven months. Case 2. An 8-year-old boy with CTS required ECMO owing to RSV-induced respiratory distress at three years of age. Although he had not been on mechanical ventilation since then, he had exercise intolerance at school, which affected his social life. 6MWT showed that he was able to walk a distance of 390 m, which is short for his age. He underwent tracheoplasty. His postoperative course was uneventful. His 6MWT result improved to 554 m on POD 26. There was no significant decrease in SpO2 after walking pre- and postoperatively in both cases. The 6MWT may be safe and useful for the evaluation of exercise capacity in children with CTS.
We encountered a case of a boy who had been diagnosed with jejunal atresia at two days of age and received jejuno-jejunostomy on the same day. Although his postoperative course was uneventful, he had acholic stools and cholestasis from 17 days of age and was started on cholagogues. The patient was discharged from the hospital at 22 days of age, but his cholestasis persisted. Therefore, he was readmitted to the hospital for further examination, but biliary atresia could not be ruled out. Test laparotomy performed at 59 days of age revealed biliary atresia (III-b1-v), and portoenterostomy (Kasai procedure) was performed. Postoperatively, his cholestasis improved and has been well maintained until the present age of one year. Because cholestasis after surgery for small bowel obstruction often occurs, cholestasis in cases of biliary atresia may be masked, and the timing of an appropriate examination and therapeutic intervention tends to be delayed. In the present study, we reviewed this case and cases of small bowel atresia at our institution. We found that the presence of biliary atresia should be confirmed in patients with cholestasis persisting for more than one month after birth.
We report the case of a four-year-old girl with left congenital diaphragmatic hernia (CDH) incidentally detected on left upper arm fracture. She was brought to our department owing to abnormal findings of chest X-ray that was carried out as preoperative assessment for the fracture surgery. Chest CT showed the gut, spleen, and pancreas prolapsed through the dorsal left diaphragm defect (Bochdalek) into the thoracic cavity. Upper and lower gastrointestinal series showed a jejunal to descending colon in the left thoracic cavity. In this case, a left upper arm surgery to repair dislocated fractured bones preceded a wait-and-see diaphragmatic repair. In surgery for CDH, it is difficult to return the spleen to the abdominal cavity because of a narrow hiatus causing edema of the intestinal wall and congestion of the spleen. It is important to be prepared for intraoperative accidental injury even in asymptomatic patients during radical surgery for late-presenting CDH.
We report a neonatal case of congenital biliary dilatation (CBD) with a very narrow left hepatic duct. A female neonate prenatally diagnosed as having CBD by fetal ultrasonography was born at 37 weeks and 4 days with a birth weight of 3,016 g. Abdominal ultrasonography revealed that the common bile duct was cystically dilated to 50 mm in diameter with irregularly dilated intrahepatic bile ducts, and CBD (Todani Type VI-A) was diagnosed. Her meconium was dark green and her general condition was good, but within a few days, her stool color became white. Because the obstruction of bile drainage was a concern, a radical operation was performed at 10 days after birth. Intraoperative cholangioscopy and cholangiography revealed that the common hepatic duct had cystic dilatation in the hepatic hilum, the right hepatic duct had membranous narrowing, and the left hepatic duct had long segmental stenosis. After total excision of the extrahepatic bile duct, bilateral hepatic ductoplasties were carried out for the biliary reconstruction with wide anastomosis at the hepatic hilum. At that time, hepaticojejunostomy was possible on the right side, but hepatic portojejunostomy (Kasai’s Procedure) was required because the lumen was too narrow on the left side. Her postoperative course was uneventful, she was discharged from the hospital 13 days after surgery, and no complications have been observed over the next 11 years.
The patient was a two-year-old girl who had been treated for recurrent vomiting for three days. She was brought to our hospital after her previous doctor suspected gastrointestinal perforation due to sudden abdominal distension. When she arrived at our hospital, her face was pale, her vitality was poor, and she had a peripheral cold sensation. Her pulse rate was 200/min, her blood pressure was 60/42 mmHg, her respiratory rate was 43/min, and she was in shock. Contrast-enhanced computed tomography (CT) of the abdomen showed a large amount of free air and ascites along with findings of gastric volvulus. Emergency laparoscopic surgery was performed. The gastric volvulus was corrected laparoscopically. Because the site of perforation was challenging to identify, we changed the procedure to open laparotomy. A pinhole perforation was found at the upper gastric corpus due to detachment of the omentum. Wedge-shaped resection was performed around the perforation, and the stomach was fixed to the abdominal wall. No migrating spleen was observed. The postoperative recovery from disseminated intravascular coagulation and improvement of gastric peristalsis took a few days, but she gradually improved. She was discharged on the 19th postoperative day. Since her discharge from the hospital, there has been no recurrence of volvulus. There have been several reports on gastric perforation associated with acute gastric volvulus. Since there have been some reports of death due to the rapid and severe onset of symptoms, a prompt diagnosis and treatment are required.
Congenital dermoid fistula (CDF) of the anterior chest region is a fistula composed of the squamous epithelium and cutaneous appendages, and it frequently occurs at the left sternoclavicular joint. CDF has not been well-known. A 10-month-old boy and a one-year-old girl had congenital fistula in the left sternoclavicular joint and were brought to our hospital owing to inflammation of the fistula. Ultrasonography revealed a cystic region under the fistula. The fistula was removed as guided by a pigment injected into it. The fistula ended in a cyst located 1 cm deep in the subcutaneous tissue. Histopathological analysis revealed that the tract was composed of the squamous epithelium, hair follicles, sebaceous glands and eccrine glands. A review of 52 reported cases including our two cases revealed that 69% of cases were due to infections, infections easily recurred, and a fistula can recur after operation. A complete excision of the fistula and cutaneous appendages is necessary to prevent recurrence with a caution that CDF forms a cyst or a nodule deep in the subcutaneous tissue. In our cases, the injection of a pigment was useful.
We report the case of a girl with biliary atresia. Portoenterostomy was performed at 57 days of age. The portal vein seemed to show trifurcation with division of the main portal vein into the left, right anterior and right posterior. The main fibrous cone was normally attached to the middle portion of the portal fissure between the left portal branch and the right anterior branch, but fibrous tissue to the posterior segment branched off early from the main fibrous cord and ran along the right posterior portal vein. It was as if the south-turning right posterior bile duct became a fibrous cord. The fibrous cone on the hepatic hilum between the left portal branch and the right anterior branch was dissected and transected at the level immediately outside of the liver capsule. The fibrous tissue running to the posterior segment was also transected at the level immediately outside of the liver capsule of the medial portion of the right posterior portal vein. Two small incisions were made on the antimesenteric border of the roux limb of the jejunum. Two anastomoses were also performed to cover the surface of the transected tissue between the left portal branch and the right anterior branch and between the right anterior branch and the right posterior branch. Her jaundice resolved promptly. Although it is a short-term follow-up result, her postoperative course has been good. Portoenterostomy with double anastomoses can be an option in cases of biliary atresia with anatomical variance in portal structures.
The patient was a 35-year-old male who underwent Kasai operation (reconstructed by hepatic portoduodenostomy interposed with 20 cm of backflow prevention valve-added jejunum) for biliary atresia (BA) 69 days after birth. His postoperative course was fairly good, but he self-interrupted his outpatient visits at the age of 22. When he was 34 years old, he was referred to the Department of Gastroenterology at our hospital because of jaundice. Abdominal CT scans showed intrahepatic bile duct dilatation, and ERC was attempted with the diagnosis of anastomotic stenosis, but the anastomotic site could not be identified. Owing to the worsening of his jaundice, he was referred to another hospital for liver transplantation. PTBD was successfully carried out at the referral hospital and his jaundice improved. His PTBD tube was managed at our hospital, but he repeatedly had fever and liver dysfunction. He later complained of vomiting, so we performed an upper gastrointestinal endoscopy, which revealed a tumor in the duodenum. CT scans showed multiple liver tumors and pleural/ascitic effusions. He was diagnosed as having intrahepatic cholangiocarcinoma by cytological analysis of bile, pleural fluid and ascites and by EUS-guided needle biopsy. He received chemotherapy, but his response was poor. He passed away 10 months after his second visit at the age of 34. The prognosis of intrahepatic cholangiocarcinoma after surgery for BA is poor. In the follow up of long-term survivors with autologous liver, the possibility of carcinogenesis should be considered.