Purpose: We evaluated the benefit of early infantile laryngotracheal separation in spinal muscular atrophy (SMA) type I following diagnosis.

Methods: We examined 5 patients (3 males, 2 females) with SMA type I, who underwent laryngotracheal separation between April 2008 and April 2015, with all operations performed using a modified Lindeman technique in infancy. We retrospectively examined the background, status at time of surgery, and postoperative developments.

Results: The mean monthly incidence rate was 1.6 ± 1.0 months. Four patients were diagnosed as having SMA type I by genetic analysis, and 1 patient was diagnosed on the basis of family history and clinical developments. All the patients were admitted with the visualization of atelectasis upon imaging before surgery. The mean age was 5.6 ± 1.0 months and the mean weight was 6.4 ± 1.4 kg at the time of surgery. All the patients had no postoperative complications, and the frequency of their hospitalization due to respiratory infection was 0.77 times/year on average. Currently, all the patients are continuing with home management. In addition, 2 patients use a communication device in order to interact with their surroundings.

Conclusions: We were able to safely perform laryngotracheal separation in infancy for 5 patients with SMA type I, as well as prevent lung degradation due to repeated respiratory infections. It is considered beneficial for patients and families living at home to undergo laryngotracheal separation in infancy for SMA type I.

Purpose: To discuss indications for delayed excision of urachal remnants.

Methods: Twenty-five patients underwent excision of possible urachal remnants. Medical records including operative findings, histopathological findings, and final diagnosis were examined.

Results: The ages of the 25 patients, i.e., 21 male and 4 female patients, ranged from 20 days to 19 years (median, 9 years). Operation was performed 3 weeks to 41 months (median, 3 months) after initial treatments in these patients, of whom 2 patients underwent operation 16 and 41 months after repeated infections. The operative findings were urachal remnants in 10 (40%) patients, i.e., urachal sinus in 5, cyst in 3, and vesicourachal diverticulum in 2 patients. Histopathological examination showed lumen and epithelia compatible with urachal remnants in 9 patients. In 15 (60%) patients, the operative findings were not conclusive for the presence of urachal remnants. The median umbilical ligament with or without scar tissues was excised in 2 patients with scar tissues and in 13 patients without scar tissues. Histopathological evidence of urachal remnants was present in 7 patients. Owing to the absence of such evidence, the final diagnosis was omphalitis unrelated to urachal remnants in the remaining 8 patients. There were more children with omphalitis among those younger than 3 years.

Conclusions: It is relevant to perform urachal remnant excision in patients older than 3 years of age. Conservative treatments are recommended in patients younger than 3 years except those with repeated infections because urachal remnants are unlikely to be related to umbilical infections.

Purpose: Although the usefulness of compression therapy with a sponge has recently been reported, the efficacy of this therapy has not been examined. Therefore, we retrospectively investigated its efficacy.

Methods: We examined the cases of umbilical hernia at our hospital between January 1, 2004 and December 31, 2016, and followed up the patients after the therapy. The patients were classified into two groups on the basis of therapy efficacy, namely, the closed group and unclosed group. We examined the patients’ gender, hernia type, gestational age, birth weight, age at start of therapy, umbilical hernia maximum diameter, hernia orifice maximum diameter, therapy period, and age at hernia closure. Data were analyzed using the JMP^® Pro 13.0.0 software program, and P < 0.05 was considered significant.

Results: Among the 1,866 patients diagnosed with umbilical hernia, 1,286 were able to be followed up after the therapy. The closed group comprised 1,134 patients (88.2%), and the unclosed group comprised 152 patients (11.8%). Significant differences were found between the groups in terms of the hernia type, gestational age, birth weight, age at start of therapy, therapy period, and age at hernia closure. In addition, the cut-off values for therapy period and age at hernia closure were 144 days and 321 days old, respectively.

Conclusions: The lower the gestational age, the higher the rate of hernia orifice closure. Similarly, the smaller the birth weight, the higher the rate. The supraumbilical type has a lower rate than the typical middle type. When the compression therapy is performed, the rate decreases after the age of 11 months. When the compression therapy period exceeds 4.8 months, the rate decreases.

A female baby weighing 1,150 g was born at 29 weeks and 6 days of gestation and was strongly suspected of having type C esophageal atresia soon after birth. Because her birth weight was very low, we selected abdominal esophageal banding and gastrostomy for staged operation. After the repair of the esophageal atresia, debanding was performed 121 days after the banding operation. Subsequently, stenosis of the abdominal esophagus developed, and we suspected that it was caused by the retained abdominal esophageal banding tape. We tried endoscopic balloon dilation, which revealed the banding tape at the site of stenosis. Repeated balloon dilation enabled the removal of the retained banding tape from the inside of the esophageal lumen. Although abdominal esophageal banding for high-risk esophageal atresia is useful, efforts should be made to prevent surgical complications associated with esophageal banding.

The patient was a 3-day-old male infant. He was born by normal vaginal delivery following an uncomplicated term gestation. Because of bilious vomiting at 3 days of age, he was transferred to our hospital. He underwent exploratory laparotomy for obstruction of the small intestine. Operative findings revealed a perforation at the tip of Meckel’s diverticulum (MD) located 20 cm proximal to the ileocecal valve, and the adhesion between the perforated site and the ileocecal valve caused intestinal obstruction. There was a cystic lesion close to the perforated site, which was not connected to the diverticulum. A portion of the small intestine including MD was resected and ileostomy was constructed. The cystic lesion was also excised. Histopathological analysis revealed that the inflammation and necrosis of MD were localized around the perforated site without any evidence of ectopic gastric mucosa in the resected specimen, and the cystic lesion was composed of necrotic intestinal tissue. From these findings, the cystic lesion was considered to be a dismembered part of the diverticulum formed owing to the torsion of MD. Although some pediatric cases with the torsion of MD have been reported, there has been no report on a case of neonatal perforation of MD.

Solitary fibrous tumor (SFT) is a rare tumor of soft tissues, which mostly arises from the pleura. Although extrathoracic SFT has been commonly described, that of the vulva is extremely rare. We report the case of a full-term female neonate who was referred to our institution with a vulvar mass suspected as inguinal hernia. CT showed a 18 × 14 × 14 mm soft tissue tumor without continuity from the abdominal cavity, and revealed multiple pulmonary metastases. MRI indicated that the vulvar mass was probably rhabdomyosarcoma. Total resection of the left vulvar mass was performed on day 11 of life. The pathological diagnosis was solitary fibrous tumor of the vulva. To the best of our knowledge, this is the first report of SFT in a neonate.

We report herein two cases of hypertrophic pyloric stenosis (HPS) in patients presenting with prenatal polyhydramnios. Patient 1 suddenly showed polyhydramnios and dilated stomach with active peristalsis at 36 weeks of gestational age as determined by prenatal ultrasonography. A 3,210 g boy was delivered at 38 weeks of gestational age and immediately diagnosed as having HPS on the basis of findings of ultrasonography and upper gastrointestinal series. Patient 2 also showed polyhydramnios prenatally at 28 weeks of gestational age. A 2,398 g boy was delivered at 36 weeks by emergent Caesarean section. He had no symptoms after birth, but started vomiting from 15 days after birth. He was transferred to our hospital and diagnosed as having HPS. Both patients presented with polyhydramnios at different periods of pregnancy and showed different postnatal clinical courses. Patient 1 presented with a rapid worsening of hydramnios during the late pregnancy period and developed HPS soon after birth. In contrast, patient 2 presented with a gradual worsening of hydramnios from the second trimester of pregnancy and developed HPS three weeks after birth. The polyhydramnios in patient 1 was likely induced by the in utero onset of HPS. In cases of prenatal polyhydramnios, HPS should be suspected and diagnosed by carefully detecting stomach dilation and hyperperistalsis.

A 2-year-old boy was diagnosed as having ileocolic intussusception and reduced by pneumatic enema. He had five repeated recurrences after pneumatic reductions for five days, and underwent laparoscopic ileocolopexy. We fixed the cecum on the right abdominal wall and fixed the terminal ileum on the ascending colon. He was discharged on the third day after surgery. There has been no recurrence for 1 year and 7 months after surgery. Laparoscopic ileocolopexy should be considered in cases of repeated recurrences at short intervals.

Congenital bladder diverticulum (CBD) is a rare disease entity found in children, which can be treated surgically. In contrast, the type of CBD that occurs from the bladder neck is known to pose risks of surgical complications involving ureteral injury or bladder nerve damage. An infant was admitted to our hospital with a huge CBD occurring from the bladder neck. Laparoscopic examination revealed that the left ureter ran across the diverticular wall, and excision of the diverticulum may be associated with the previously mentioned risks. Thus, after the removal of the mucous membrane, we sutured the muscular layer of the CBD and performed ureterocystostomy to avoid the risk of vesicoureteral reflux. The infant was found to be doing well, and cystography confirmed the disappearance of the CBD and good elasticity of the bladder. No wall thickening was observed after the suturing of the muscular layer of the CBD. We conclude that this operative technique is useful for the management of such cases.

Asplenia syndrome is one of the multiple congenital anomalies, which has right atrial isomerism and complex cardiovascular anomalies. To increase survival, the management of this syndrome is important for not only the cardiovascular anomalies but also the associated gastrointestinal anomalies. We report the case of a boy with asplenia syndrome who had hiatal hernia and microgastria, whose stomach was completely displaced to the mediastinum, which was surgically managed with a Hunt-Lawrence pouch. The patient underwent palliative surgery for the single ventricle at 1 month of age, and then underwent repair for hiatal hernia and gastrostomy at 3 months of age. Because of the small capacity of his stomach, he still had severe gastroesophageal reflux. After undergoing the Fontan procedure, he had cardiovascular radical surgery at 2 years of age. Gastric augmentation was accomplished at 2.5 years of age, with the construction of a Hunt-Lawrence pouch in order to increase the gastric volume. He is now making satisfactory progress. The associated anomalies in asplenia syndrome are generally complicated for both cardiovascular and gastrointestinal systems. In the management of this syndrome, it is important for each medical team to be in close collaboration with each other, select the best timing and method for the treatment, and plan a multidisciplinary therapeutic strategy.

The patient was a 23-year-old female, who underwent a radical operation of anal canal duplication at the age of 8 months. Although she had no complications associated with the disease during her childhood, she felt discomfort during defecation and lower abdominal pain for half a year. Abdominal CT revealed a cystic tumor lesion in the pelvis and she was referred to the Department of Pediatric Surgery. Further examination showed a 4-cm-diameter lesion located between the rectum and the sacrum, and rectal duplication or remnant of anal canal duplication was suspected. Surgical treatment was performed; the tumor and rectum did not share a common wall and the lesion was extracted without rectal wall resection. Furthermore, histopathological analysis showed that the wall of the lesion was lined by granulation tissue and an anal gland, which contained pus fluid, and the final diagnosis was residual abscess of anal canal duplication. Although to the best of our knowledge there has been no case report of residual abscess of gastric tract duplication, the complete resection of the mucosa of the duplication tissue is considered necessary as the initial surgical approach.

We report a surgical case of a 5-month-old female infant with a fistula of the left lumbar region. The fistula with hair was detected at 2 months of age. It became red at 3 months of age, but treatment with an antibacterial agent did not improve it, and she was referred to our hospital. We found the fistula at the center of a subcutaneous abscess with 1 cm diameter. Ultrasonography showed that the fistula was 15 mm in length and ran to her back side and came to a blind end above the thoracolumbar fascia at the level of the bottom of her left kidney. After resolving the inflammation, magnetic resonance imaging revealed that the 17-mm-long blind fistula was completely within the subcutaneous tissue, and had no connection to the spinal canal. She underwent surgery to remove the fistula at 5 months of age to prevent recurrent infections. The fistula was completely resected at the level of the subcutaneous tissue. Histopathological findings showed that the wall was covered with a stratified squamous epithelium and had hair follicles inside. In general, congenital dermal sinuses are small tracts or dimples that open in the midline of the posterior of the body from the occiput to the lumbosacral region. Although the opening of the lesion was in the lateroabdominal area, that is, nonmiddle, the fistula was diagnosed as a congenital dermal sinus on the basis of her age at disease onset.

A CVR (CPAM volume ratio) of >1.6 is considered to be associated with a high risk of fetal hydrops and poor prognosis. We experienced treating two cases of CCLD with fetal hydrops or cavity effusion (CVR > 1.6), for which thoracoamniotic shunting was performed in the prenatal period. Both cases were rescued by a multidisciplinary approach that included emergency surgery after birth. Patient 1 underwent thoracoamniotic shunting at 25 weeks of gestation. After birth, we performed chest drainage and cyst drainage because of pneumothorax and the expansion of the cyst. At 17 days of age, we performed right upper lobectomy. Patient 2 underwent thoracoamniotic shunting at 22 weeks of gestation. We performed emergency cystectomy immediately after birth owing to poor cardiac hemodynamics. At 8 months of age, we performed right middle lobectomy and segmental resection of the lower lobe. Thoracoamniotic shunting for CCLD with fetal hydrops can markedly improve the prognosis. However, multidisciplinary therapy is often required in the neonatal period, and medical support is needed after hospital discharge. In addition to the prognosis, the patient’s quality of life (QOL) should be considered when deciding the treatment for CCLD with fetal hydrops.

The patient was a 5-year-old boy who had no medical history. He was referred to us with suspected acute appendicitis and complaints of fever and lower abdominal pain. Although his abdominal pain was intense, rebound tenderness and muscular protection were negative. There were no symptoms of problems in the digestive tract such as vomiting or diarrhea. His blood examination revealed an increase in inflammatory reactions, as indicated by the increased white blood cell count and C-reactive protein levels. Computed tomography (CT) revealed wall thickening of the ascending colon; therefore, we started antibiotic administration after acute enterocolitis was diagnosed. After admission, we performed CT again and found a fatty and dense mass with a swirling structure inside the mass; therefore, we diagnosed the patient as having idiopathic omental torsion. Because the symptoms were relieved, conservative management was continued and the patient was discharged on the fourth day after hospitalization. Omental torsion is a rare condition, particularly seen in children; its classical treatment option is surgery. Although conservative management was successful in this case, there are few reports on it. There are many cases where surgery is performed after an initial diagnosis of acute appendicitis is made owing to the presence of similar symptoms. However, in recent years, because of advances in imaging diagnostic techniques such as CT, more patients are being accurately diagnosed. We believe that if patients are accurately diagnosed, surgery will always be required; however, conservative management is sometimes possible.