Enhanced lymphangiogenesis in the left lateral segment of a biopsied liver during portoenterostomy for biliary atresia.

PURPOSE: We investigate the histopathology of the portal vein branches and lymphatic vessels to elucidate the mechanism of atrophy of the left lateral segment (LLS) of the liver in biliary atresia (BA). METHODS: LLS and right anterior segment (RAS) liver biopsy samples obtained during Kasai portoenterostomy (KPE) from ten consecutive patients with BA underwent histopathological investigation of the portal vein and lymphatic vessels using double chromogenic immunostaining for CD31/D2-40 and the hepatitis-like findings (HLF) score. Each parameter and clinical data were compared between prognostic groups. RESULTS: HLF scores in the LLS were always higher than those in the RAS. There was no difference in portal vein and lymphatic vascular morphology, whereas the number of lymphatic vessels was correlated with the fibrotic area of all specimen areas. Left-to-right ratio of the number of lymphatic vessels was correlated with the age at KPE (r = 0.784, p = 0.007) and the pre-KPE CRP value (r = 0.723, p = 0.018). CONCLUSIONS: Lymphangiogenesis on the LLS compared to the RAS was significantly correlated with the degree of fibrosis and the age at KPE. Further investigation is warranted to clarify the causes of LLS atrophy and lymphangiogenesis relevant to immune dysregulation.

Inflammation patterns in early post-operative cholangitis predict long-term outcomes in biliary atresia: a potential role of non-suppurative cholangitis.

PURPOSE: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis. METHODS: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients. RESULTS: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001). CONCLUSIONS: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA.

Endosurgery workshop to improve the confidence of novice pediatric surgeons in performing laparoscopic hepaticojejunostomy.

PURPOSE: This study assessed the efficacy of a high-impact, short-term workshop in honing the laparoscopic hepaticojejunostomy technical skills and self-confidence of novice pediatric surgeons, focusing on vertical needle driving and knot tying. METHODS: Lectures, hands-on sessions, pre- and post-workshop evaluations, and training using porcine models were conducted to refine basic and advanced skills. The "hepaticojejunostomy simulator" was used for comparative analysis of precision in pre- and post-workshop vertical needle driving and knot tying. Participants self-evaluated their skills and confidence on a 5-point scale. RESULTS: After the workshop, eight inexperienced pediatric surgeons demonstrated a significant improvement in hepaticojejunostomy suturing task completion rates and needle-driving precision at the jejunum and hepatic duct. However, the A-Lap Mini Endoscopic Surgery Skill Assessment System indicated no significant improvements in most assessed parameters, except for the full-layer closure score (p = 0.03). However, a significant increase in participants' confidence levels in performing laparoscopic hepaticojejunostomy was observed. CONCLUSION: The workshop augmented technical proficiency and confidence in young pediatric surgeons. The combination of lectures, practical exposure, and model training is an effective educational strategy in pediatric surgical instruction.

Axillary skin crease incision versus conventional posterolateral incision in open repair of patent ductus arteriosus for extremely low birth weight infants: a retrospective study.

BACKGROUND: Thoracotomy with posterolateral incision (PLI) is commonly used for surgical repair of patent ductus arteriosus (PDA) in extremely low birth weight (ELBW) infants. Some reports have described thoracotomy for PDA using an axillary skin crease incision (ASCI) in consideration of cosmetic problems such as surgical wounds and thoracic deformities, but the details remain unclear. METHODS: In this study, we performed clipping ligation by thoracotomy with ASCI for ELBW infants with PDA from 2011 to 2015 for the purpose of improving cosmetic results, and retrospectively compared the results with those for conventional PLI cases performed from 2016 to 2020. RESULTS: ASCI was found to be associated with serious surgical complications and showed a significant difference in outcome parameters only for surgery time, suggesting a safety problem for ASCI. Considering these results, PLI allows clipping of the nearby PDA from the thoracotomy wound while looking straight ahead, whereas the PDA in ASCI is positioned deep and oblique to the thoracotomy wound, so the clipping angle is limited and accurate completion of the procedure is difficult. CONCLUSIONS: Regarding PDA repair in ELBW infants, ASCI shows a high risk of serious surgical complications. Conventional PLI remains preferable for safe and accurate results.

Contrast-enhanced ultrasonographic findings in torsion of the appendix testis or epididymis in children: a case series.

PURPOSE: Torsion of the appendix testis or epididymis is a cause of acute scrotum in children. Ultrasonography with color Doppler is the first-choice modality for diagnosis. However, this method requires skill and experience to make a diagnosis with confidence. Recently, contrast-enhanced ultrasonography for diagnosis in various fields has been reported. However, to our knowledge, there has been no report of this method being used to diagnose torsion of the appendix testis or epididymis. The purpose of this study was to retrospectively examine contrast-enhanced ultrasonographic findings in torsion of the appendix testis or epididymis. METHODS: Patients who underwent contrast-enhanced ultrasonography for torsion of the appendix testis or epididymis at our institution between April 2010 and April 2023 were enrolled in this study (n = 12). Contrast-enhanced ultrasonography findings of the affected appendage and the testis parenchyma were examined retrospectively. RESULTS: The parenchyma of the testes was notably enhanced in all the cases. However, 9 of the 12 cases showed that the appendage with torsion was not enhanced at all. In the remaining three cases, only slight enhancement was seen. Nevertheless, it was notably less than that of the parenchyma of the testis. CONCLUSION: Our findings indicated that contrast-enhanced ultrasonography may be an easy and reliable method for diagnosing torsion of the appendix testis or epididymis.

Contrast-enhanced ultrasound findings in testicular torsion and non-testicular torsion.

PURPOSE: Testicular torsion requires emergency surgery; thus, prompt and correct diagnosis is very important. Ultrasound with color Doppler is usually the first-choice modality for diagnosis; however, skill and experience are required for confident diagnosis. Recently, contrast-enhanced ultrasound for the diagnosis of testicular torsion has been reported, but there have been only a few reports. This study aimed to compare contrast-enhanced ultrasound findings in cases of testicular torsion and non-testicular torsion. METHODS: Patients who underwent contrast-enhanced ultrasound for acute scrotum at our institution between April 2010 and January 2023 were divided into testicular torsion (n = 17) and non-testicular torsion (n = 16) groups. The respective contrast-enhanced ultrasound findings were retrospectively examined and compared. RESULTS: In 16 out of 17 cases of testicular torsion, the parenchyma of the affected testis was not enhanced. In the remaining case, reduced contrast enhancement was observed; however, it was still notably less than that observed on the unaffected testis. On the other hand, in all cases of non-testicular torsion (n = 16), the parenchyma of the affected testis was notably enhanced. CONCLUSION: Contrast-enhanced ultrasound is considered an easy and accurate method for diagnosing testicular torsion.

A Case of an Extremely Preterm Infant with Intussusception Triggered by Acquired Cytomegalovirus Infection.

Intussusception is a common cause of intestinal obstruction in infants aged 6-18 months. However, intussusception in preterm neonates (IPN) is an exceedingly rare disorder. The etiology of IPN remains unclear, but common prenatal injuries, such as those causing intestinal hypoxia/hypoperfusion, dysmotility, and strictures, have been proposed as possible contributing factors. Diagnosis is often delayed because the symptoms closely resemble those of necrotizing enterocolitis (NEC). Given the divergent treatments for IPN and NEC, establishing an early and accurate diagnosis is crucial. IPN is predominantly located in the small intestine (91.6%), and ultrasonography proves useful in its diagnosis. We present a case of a very preterm infant who developed intussusception triggered by acquired cytomegalovirus (aCMV) infection, necessitating surgical treatment. The cause of intussusception in this case was diagnosed as aCMV enteritis because no organic lesions were observed in the advanced part of the intussusception. The presence of CMV was confirmed by CMV-DNA-PCR examination of the resected intestinal tract. Intestinal edema and decreased intestinal peristalsis due to aCMV enteritis are likely the primary causes of the intussusception.

Neonatal necrotizing fasciitis with gas gangrene due to peripherally inserted central catheter-related infection.

BACKGROUND: Necrotizing fasciitis in neonates is a rare and life-threatening infection involving necrosis of the skin, subcutaneous tissues, deep fascia, and sometimes underlying muscles, with a fulminant course and high mortality rate. Necrotizing fasciitis with gas gangrene related to infection of a peripherally inserted central catheter is very rare. CASE PRESENTATION: The patient was a full-term female neonate born by vaginal delivery. Following diagnosis of patent ductus arteriosus, indomethacin was administered from a peripherally inserted central catheter for 3 days. Four days after the termination of medical treatment for the patent ductus arteriosus, the patient developed fever and a severely elevated inflammatory response was identified from blood testing. Around the right anterior chest wall, corresponding to the site of the catheter tip, redness was increased and gas crepitus was felt under the skin. Computed tomography revealed emphysema in the anterior chest, in subcutaneous areas and between muscles. Emergency surgical debridement was performed under a diagnosis of necrotizing fasciitis with gas gangrene. With antibiotic treatment, we started to fill the wound with a dialkyl carbamoyl chloride-coated dressing and povidone-iodine sugar ointment after washing with saline once a day. The patient survived and after 3 weeks of treatment with the dressing, the wound had successfully resolved without motor impairments. CONCLUSIONS: In addition to medical treatment and prompt surgical debridement, we used dialkyl carbamoyl chloride-coated dressing and povidone-iodine sugar ointment for antiseptic dressings and successfully treated neonatal necrotizing fasciitis with gas gangrene caused by peripherally inserted central catheter infection with Citrobacter koseri.

Infantile type I pleuropulmonary blastoma presenting with dyspnea due to compression by pneumothorax and an occupying tumor: a case report.

BACKGROUND: Pleuropulmonary blastoma (PPB) is an extremely rare and malignant pediatric lung tumor. Purely cystic PPB has a more favorable prognosis than solid PPB, but may be difficult to distinguish from a certain type of "benign" congenital pulmonary airway malformation before and during surgery. The influence of tumor rupture on long life prognosis has not been clarified in detail. CASE PRESENTATION: A 5-month-old boy underwent emergency transfer from another hospital due to a left thoracic cystic lesion and left pneumothorax detected on chest radiography performed for persistent wheeze and cough. Contrast-enhanced computed tomography of the chest revealed marked deviation of the mediastinum to the right due to a giant cystic lesion and pneumothorax. Thoracotomy was performed on hospital day 2. A cystic lesion had developed from the distal alveolar region of lower lobe of the left lung and the tumor showed a tiny adhesion to the left diaphragm and a tiny rupture near the adhesion. Partial lung excision including the cyst and scraping of the adhesion were performed. Histopathological investigations revealed immature blast cell-like mesenchymal cells and differentiated striated muscle cells in a dense cambium layer were found under the epithelium of the cystic lesion. Type I PPB was diagnosed. CONCLUSIONS: Surgery should be performed with the possibility of type I PPB in mind when an extrapulmonary cystic lung lesion is found. Since issues such as the pathogenesis and long-term prognosis of ruptured cases remain unclear, continued careful follow-up of this case will be required.

Challenging management of a baby with congenital multiple intestinal atresia, trisomy 18 and extremely low birth weight: a case report.

BACKGROUND: Extremely low birth weight (< 1000 g) still influences postsurgical prognosis in the neonatal and infantile periods. Additionally, the life expectancy of neonates with trisomy 18 is extremely poor owing to various comorbidities. Therefore, it takes courage to perform laparotomy for the purpose of treatment of congenital multiple intestinal atresia in a baby with an unpredictable life prognosis. CASE PRESENTATION: Fetal ultrasonography revealed cardiac malformation, intestinal dilation, and physical characteristics suggestive of a chromosomal abnormality in this case. The patient was diagnosed with trisomy 18 after birth, with an extremely low birth weight. An atrial septal defect, ventricular septal defect, dilated jejunum, and a very thin collapsed small intestine were found on ultrasonography. With a diagnosis of congenital small intestinal atresia, a challenging laparotomy was done at 3 days of age, with jejunal atresia and multiple distal small intestinal atresia were observed. The jejunal end and distal small intestinal stump were separated into stomas at the wound edge. Hypertrophic pyloric stenosis developed at the age of 3 months and resolved with medication. The patient gained weight (2 kg) by daily stool injection into anal side of the intestine and decompression against poor peritonitis of dilated jejunum using enteral feeding tube for the long period. Finally, we could perform intestinal reconstruction safely and successfully at the age of 9 months. Tracheotomy was performed due to difficulty in extubation associated with chronic lung disease. The patient was discharged at the age of 1 year and 3 months, and no major problems were noted at the age of 2 years. CONCLUSIONS: We treat congenital intestinal atresia in extremely low birth weight infants with severe chromosomal abnormalities and severe cardiac malformations as follows: Stoma creation is performed quickly to avoid deterioration of the patient's hemodynamics. After that, while continuing enteric management, palliative cardiovascular surgery is performed as necessary, and the patient's body weight and intestinal tract status are determined to allow safe intestinal reconstruction.

Median sternotomy approach for the repair of esophageal atresia: a case report.

BACKGROUND: Repair of esophageal atresia is usually performed through the right thoracic cavity. However, when the upper pouch of the esophagus and tracheoesophageal fistula (TEF) is located in the thoracic inlet and completely on the left side of trachea, it is difficult to dissect and anastomose the esophagus through the right thoracic cavity. We present a case of esophageal atresia, with the esophageal upper pouch located high and completely on the left side of trachea, successfully repaired via the median sternotomy approach. CASE PRESENTATION: A male neonate with a birth weight of 1766 g was prematurely delivered via cesarean section at 34 weeks of gestation. Contrast-enhanced computed tomography (CT) showed that the upper pouch of the esophagus was located at the thoracic inlet and completely on the left side of the trachea; hence, a diagnosis of esophageal atresia was made. Moreover, a TEF was connected to the trachea at the level of the lower end of the upper esophageal pouch. An aberrant right subclavian artery and persistent left superior vena cava were also detected. Esophageal dissection and anastomosis were determined to be very difficult if approached from the right thoracic cavity. Therefore, we performed median sternotomy one day after the neonate was born. The upper pouch of the esophagus and TEF were easily dissected via the median sternotomy approach. Anastomosis of the esophagus was performed, with a good visual field, to the left of the trachea. The postoperative course was uneventful. CONCLUSIONS: This is the first reported case of a median sternotomy approach for esophageal atresia. This technique may be useful when a right thoracic approach is difficult, especially if the esophageal upper pouch is located completely to the left of the trachea or if it is higher than the normal position.