A pediatric case of Burkitt's lymphoma with bile duct obstruction requiring surgical biliary reconstruction.

BACKGROUND: Biliary obstruction due to compression by a B-cell solid tumor occurs rarely. A few reports have described biliary reconstruction surgery for obstructive jaundice caused by Burkitt's lymphoma. However, there are no detailed reports on pediatric cases. We report a pediatric case of obstructive jaundice due to malignant lymphoma treated with biliary reconstruction surgery. CASE PRESENTATION: A 5-year-old girl presented to our hospital with a massive abdominal tumor that caused biliary stricture. Chemotherapy was initiated after an open tumor biopsy. However, endoscopic biliary stent placement was performed owing to elevated bilirubin levels. We treated the patient with chemotherapy for 9 months while endoscopically replacing the biliary stent every few months. She achieved complete tumor remission. However, sclerotic lymph nodes were persistent on the dorsal side of the cholecystic duct junction, and biliary stricture at the same site had changed to stent-dependent biliary obstruction. Therefore, we performed choledochojejunostomy and retrocolic Roux-en-Y reconstruction 15 months after initial admission. There were no postoperative complications or tumor recurrences, and the bilirubin level remained low. Histopathologically, the resected bile duct wall was fibrotic and thick, and the bile duct lumen narrowed. CONCLUSIONS: Biliary reconstruction is effective to achieve long-term biliary patency in pediatric patients with stent-dependent biliary obstruction due to malignant lymphoma. However, the decision on when to stop biliary stent replacement and proceed to biliary reconstruction surgery is a matter of debate. Further case studies are required to address this issue.

Impact of the novel coronavirus infection on pediatric surgery: an analysis of data from the National Clinical Database.

PURPOSE: The coronavirus disease 2019 (COVID-19) pandemic limited the delivery of medical resources. Although surgeries are triaged according to disease severity and urgency, a delay in diagnosis and surgery can be detrimental. We conducted this study to analyze data on the impact of the COVID-19 pandemic on pediatric surgery for different diseases or disorders. METHODS: We compiled and compared data on pediatric surgical cases from 2018 to 2020, using the National Clinical Database. The number of diseases, severity, complication rates, mortality rates by disease/disorder, and the COVID-19 pandemic areas were analyzed. RESULTS: The total number of cases of pediatric surgery in 2018, 2019, and 2020 was 50,026, 49,794, and 45,621, respectively, reflecting an 8.8% decrease in 2020 from 2018 and an 8.4% decrease in 2020 from 2019. A decrease was observed when the number of patients with COVID-19 was high and was greater in areas with a low infection rate. There was a marked decrease in the number of inguinal hernia cases. The number of emergency room visits and emergency surgeries decreased, but their relative proportions increased. CONCLUSIONS: The COVID-19 pandemic decreased the number of pediatric surgeries, reflecting the limitations of scheduled surgeries and infection control measures.

Laparoscopic Heller myotomy with Dor fundoplication for esophageal achalasia treatment after distal gastrectomy and Billroth-II reconstruction.

Laparoscopic Heller myotomy with Dor fundoplication is the standard surgical treatment for esophageal achalasia. However, there are few reports on the use of this method after gastric surgery. We report a case of a 78-year-old man who underwent laparoscopic Heller myotomy with Dor fundoplication for achalasia after distal gastrectomy and Billroth-II reconstruction. After the intraabdominal adhesion was sharply dissected using an ultrasonic coagulation incision device (UCID), Heller myotomy was performed 5 cm above and 2 cm below the esophagogastric junction using the UCID. To prevent postoperative gastroesophageal reflux (GER), Dor fundoplication was performed without cutting the short gastric artery and vein. The postoperative course was uneventful, and the patient is in good health without symptoms of dysphagia or GER. Although per-oral endoscopic myotomy is becoming the mainstay of treatment for achalasia after gastric surgery, laparoscopic Heller myotomy with Dor fundoplication is also an effective strategy.

Successful combined laparoscopic and thoracoscopic surgery for esophago-mediastinal fistulae.

Minimally invasive surgeries have been developed, not only for gastrointestinal cancer, but also for benign or emergency cases. We report the case of a 62-year-old male who underwent laparoscopic and thoracoscopic combined surgery for an esophago-mediastinal fistula caused by a press-through package. In the initial laparoscopic phase, transhiatal dissection of the lower thoracic esophagus and harvesting of the greater omentum were performed. In the thoracoscopic phase, resection of the fistula and esophageal wall closure were performed. Thereafter, the greater omentum was lifted via the esophageal hiatus and wrapped around the repaired part of the esophagus for reinforcement. The total operative time was 371 min, with 163 and 208 min for the laparoscopic and thoracoscopic phases, respectively. In total, 20 ml of blood was lost. No perioperative complications or recurrences were observed. Laparoscopic and thoracoscopic combined omentoplasty was effective for refractory esophago-mediastinal fistula.

Extensive Subcutaneous Abscess due to Panton-Valentine Leucocidin-Positive Community-Associated Methicillin-Resistant Staphylococcus aureus in an Infant.

Community-associated methicillin-resistant Staphylococcus aureus (CA-MRSA) infections have increased worldwide in people without underlying diseases. CA-MRSA can often cause serious bacterial infections, especially skin and soft tissue infections (SSTI). Here, we describe a case of severe subcutaneous abscess due to Panton-Valentine leucocidin (PVL)-positive CA-MRSA in an infant without underlying diseases. A 4-month-old girl presented with a 4-day history of fever, with extensive redness and swelling of the lumbar region and buttocks. She was diagnosed with extensive subcutaneous abscess of the lumbar region and buttocks. Surgical drainage was performed, and a substantial volume of pus was drained. MRSA was detected in the pus on culture. Antibiotic therapy that covered MRSA was also administered for 3 weeks, and the abscess healed. As it was a severe SSTI due to MRSA, analysis of MRSA revealed PVL-positive MRSA. This patient had no underlying disease or history of antibiotic administration, and as MRSA was present in the nasopharyngeal cavity, it was considered a case of CA-MRSA. Furthermore, the prevalence of PVL-positive CA-MRSA in MRSA isolated from patients with SSTI has also increased in Japan. The Infectious Diseases Society of America recommends surgical intervention and empirical antibiotic therapy for MRSA-complicated SSTI cases in an era of CA-MRSA. Pediatricians must strongly consider the possibility of MRSA in children with severe SSTIs.

Prenatal diagnosis of pyriform sinus fistula using ultrasonography by detecting the communication to the pharynx.

OBJECTIVE: Pyriform sinus fistula (PSF) is a congenital anomaly which originates from the pharyngeal pouch. PSF is initially recognized as a cyst around the fetal neck, but accurate prenatal diagnosis of the disease is challenging. We aimed to report the key findings and tips in accurately distinguishing PSF from other differential diagnosis by which enables detection of the communication of the nuchal cyst and the pharynx. CASE REPORT: We report a case in which we were able to diagnose PSF as early as 18 weeks of gestation with ultrasonography. We used epiglottis as a landmark, and detected an unilobular cyst arising from the pharynx. CONCLUSION: Ultrasonography is a powerful tool in prenatal diagnosis of PSF especially at early stage of pregnancy. By detecting the epiglottis, it can locate the communication of the nuchal cyst and the pharynx, and thereby enables an accurate diagnosis of PSF.

Ectopic gallbladder with congenital biliary dilatation: a pediatric case report.

BACKGROUND: An ectopic gallbladder is a rare anomaly and can result in the misinterpretation of imaging findings and clinical confusion. Knowledge of such anomalies facilitates accurate diagnoses and prompt management. We report a pediatric case of an ectopic gallbladder concomitant with congenital biliary dilatation (CBD). CASE PRESENTATION: A 9-year-old girl was referred to our hospital for elevated liver enzyme levels. Following physical examination and a review of medical imaging findings, she was diagnosed with Todani type IV-A CBD. We could not visualize the gallbladder by abdominal ultrasonography, CT, and MRI scans; therefore, we suspected gallbladder agenesis. A laparoscopic excision of the extrahepatic bile duct was performed to treat the CBD. Neither a gallbladder nor a cystic duct were revealed on the liver undersurface. Therefore, gallbladder agenesis was considered as a diagnosis based on preoperative imaging and intraoperative findings. However, during dissection of the hepatic hilum, a cyst-like structure was found on the ventral side of the common hepatic duct, slightly to the right, and a small additional duct that originated from the cystic structure was found. Upon incision, a small amount of bile was drained from the small duct. Thus, the cystic structure was diagnosed as an ectopic gallbladder with hypoplasia. Following the removal of the ectopic gallbladder, the extrahepatic bile duct was excised. Subsequently, laparoscopic Roux-en-Y hepaticojejunostomy was performed without any complications. Postoperative histopathological evaluations of the resected specimen revealed Rokitansky-Aschoff sinuses in the resected cystic lesion. The pathological investigations confirmed the diagnosis of an ectopic gallbladder. Following an uneventful postoperative course, the patient was discharged on day nine. CONCLUSIONS: To our knowledge, this is the first pediatric case report describing an ectopic gallbladder concomitant with CBD. If the gallbladder cannot be detected in a preoperative imaging examination, it is important to consider the possibility of an ectopic gallbladder.

A Fetus with Imperforate Anus Developing Pulmonary Hypoplasia Triggered by Transient Urethral Obstruction.

Pulmonary hypoplasia is a rare entity in a fetus with imperforate anus. The fetus was diagnosed with high-type imperforate anus with rectourethral fistula based on the dilated fetal bowel and the presence of bowel calcification at 19 weeks of gestation. As gestation advanced, fetal ultrasonography demonstrated development of pulmonary hypoplasia, progressive bowel dilation, and persistent oligohydramnios from 28 weeks of gestation despite a fluid-filled bladder without hydroureter or hydronephrosis. To prevent further worsening of pulmonary hypoplasia caused by thoracic compression due to bowel dilation and oligohydramnios, a male neonate was delivered by cesarean section at 32 weeks of gestation. The neonate showed respiratory failure requiring full respiratory support. Although a catheter did not pass through the urethra into the bladder at birth, cystourethrography revealed the patency of fistula and stenosed lower urinary tract. Prenatal and postnatal findings strongly suggested that the meconium in the colon might have passed into the urethra in the penis, resulting in the physical blockage of urine outflow to the amniotic space which leads urine flow from the bladder to the colon through the fistula, which resulted in subsequent oligohydramnios and bowel dilation. To the best of our knowledge, this is the first case report of a fetus with imperforate anus developing pulmonary hypoplasia possibly due to urethral obstruction.

Optimal treatment of pneumothorax in adolescents with Marfan syndrome.

PURPOSE: Pneumothorax often develops in patients with Marfan syndrome (MFS). Here, we examined the effects of conservative and surgical pneumothorax treatments in children with MFS. METHODS: In this study, 23 patients, less than 20 years old, diagnosed with both MFS and pneumothorax between 1999 and 2019 were included. All data were collected retrospectively from patients' medical records. RESULTS: In total, 18 of 23 patients (78%) had relapsed pneumothorax either on the ipsilateral or contralateral side. Among these 18 patients, 6 (26%) patients had multiple relapses. Conservative and surgical treatments of pneumothorax were attempted in 33 and 29 lungs, respectively. The conservative treatment was attempted as a definitive therapy in 21 lungs. Twelve conservative treatments (57%) failed, which required surgical intervention. In 9 lungs (43%) with successful conservative treatment, 6 (67%) had ipsilateral relapses. In contrast to the above findings, only 4 (13%) ipsilateral relapses were observed in 29 surgical treatments. CONCLUSIONS: Our study revealed a low response and high relapse rate when MFS adolescents who diagnosed pneumothorax were subjected to the conservative treatment modality. Thus, we recommend surgical intervention as the first line of therapy to treat pneumothorax in adolescents diagnosed with MFS. LEVEL OF EVIDENCE: Ⅲ (Treatment Study).

A new method of primary engineering of esophagus using orthotopic in-body tissue architecture.

PURPOSE: Tissue engineering of esophagus is required for management of long-gap esophageal atresia (LGEA). Collagenous connective tissue membranes fabricated by in-body tissue architecture (iBTA), called biosheets, can repair esophageal defects and generate tissues similar to native esophagus. However, iBTA requires second-stage surgery because of heterotopic preparation of biosheets. Our aim was to develop orthotopic iBTA for primary engineering of the esophagus by interposing a tubular mold to the esophageal defect. METHOD: The cervical esophagus of six rats was transected. An acrylic tube (internal diameter 2.6 mm, length 7.0 mm) was inserted and fixed between the ends of the upper and lower esophagus, and a 3 mm-long esophageal defect was created. Four weeks later, the rats were sacrificed for histological analysis. RESULTS: Postoperatively the rats could intake liquid food. After four weeks, the esophageal defects were filled with regenerated tissues. Histologically the new esophageal walls stained positive for collagen type I. The inner surfaces were covered with stratified squamous epithelium that expressed pan-cytokeratin. In only one of six rats, regeneration of muscular-like tissue was suggested by positive immunohistochemical staining for desmin. CONCLUSION: Orthotopic iBTA can regenerate a substitute esophagus with esophageal epithelium and collagenous wall. This technique may be a novel treatment for esophageal atresia with gaps of various lengths including LGEA.

Prophylactic innominate artery transection to prevent tracheoinnominate artery fistula: a retrospective review of single institution experiences.

PURPOSE: This study aimed to investigate the optimal indication and availability of prophylactic innominate artery transection (PIAT). METHODS: We retrospectively analyzed the medical records of the patients with neurological or neuromuscular disorders (NMDs) who underwent PIAT. Meanwhile, we originally defined the tracheal flatting ratio (TFR) and mediastinum-thoracic anteroposterior ratio (MTR) from preoperative chest computed tomography imaging and compared these parameters between non-PIAT and PIAT group. RESULTS: There were 13 patients who underwent PIAT. The median age was 22 years. PIAT was planned before in one, simultaneously in five, and after tracheostomy or laryngotracheal separation in seven patients. Image evaluations of the brain to assess circle of Willis were performed in all patients. Appropriate skin incisions with sternotomy to expose the innominate artery were made in four patients. All patients are still alive except one late death without any association with PIAT. No neurological complications occurred in any patients. As significant differences (p < 0.01) between two groups were observed for TFR and MTR, objective validity of the indication of PIAT was found. CONCLUSIONS: PIAT is safe and tolerable in case of innominate artery compression of the trachea with NMDs. TFR and MTR are useful objective indexes to judge the indication of PIAT.

Discovery of Candidate Stool Biomarker Proteins for Biliary Atresia Using Proteome Analysis by Data-Independent Acquisition Mass Spectrometry.

Biliary atresia (BA) is a destructive inflammatory obliterative cholangiopathy of the neonate that affects various parts of the bile duct. If early diagnosis followed by Kasai portoenterostomy is not performed, progressive liver cirrhosis frequently leads to liver transplantation in the early stage of life. Therefore, prompt diagnosis is necessary for the rescue of BA patients. However, the prompt diagnosis of BA remains challenging because specific and reliable biomarkers for BA are currently unavailable. In this study, we discovered potential biomarkers for BA using deep proteome analysis by data-independent acquisition mass spectrometry (DIA-MS). Four patients with BA and three patients with neonatal cholestasis of other etiologies (non-BA) were recruited for stool proteome analysis. Among the 2110 host-derived proteins detected in their stools, 49 proteins were significantly higher in patients with BA and 54 proteins were significantly lower. These varying stool protein levels in infants with BA can provide potential biomarkers for BA. As demonstrated in this study, the deep proteome analysis of stools has great potential not only in detecting new stool biomarkers for BA but also in elucidating the pathophysiology of BA and other pediatric diseases, especially in the field of pediatric gastroenterology.

Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis.

PURPOSE: Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis. METHODS: A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason. RESULTS: We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23-12.89, p < 0.00001; odds ratio: 25.88, 95% CI 2.83-236.84, p < 0.004; odds ratio: 15.05, 95% CI 2.70-83.93, p = 0.002, respectively). CONCLUSION: The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.

Mosapride for gastroesophageal reflux disease in neurologically impaired patients.

BACKGROUND: The prokinetic agent cisapride is effective for the treatment of gastroesophageal reflux disease (GERD) in infants and children, but is no longer used for this purpose because of safety concerns. Therefore, other pharmacological agents need to be investigated for efficacy in GERD treatment. In this study, we examined the effectiveness and safety of mosapride for the treatment of neurologically impaired children and adolescents with GERD. METHODS: Mosapride (0.3 mg/kg/day) was administered to 11 neurologically impaired patients with GERD (five male; median age, 12.3 years). Esophageal acid exposure was measured using esophageal pH monitoring before and at >5 days after the start of mosapride treatment. The pressure and length of the lower esophageal sphincter were compared before and after mosapride treatment. RESULTS: In the 11 patients, median reflux index (percentage of the total monitoring period during which recorded pH was <4.0) was 17.5% (range, 4.4-59%) before and 8.2% (range, 2.8-20.7%) after mosapride treatment (P = 0.02). Median esophageal clearance was 1.0 min/reflux (range, 0.5-2.1 min/reflux) before and 0.7 min/reflux (range, 0.4-1.2 min/reflux) after treatment with mosapride (P = 0.02). The median number of reflux episodes before (219) and after (122) drug treatment did not differ significantly. CONCLUSION: The decreased reflux index in neurologically impaired patients with GERD is due to mosapride, therefore mosapride may be a candidate for GERD treatment.

[Vesico-appendiceal fistula caused by appendiceal cancer: a case report].

A case of vesico-appendiceal fistula caused by appendiceal cancer is reported. A 37-year-old male was admitted with the chief complaint of suspended dust in the urine. Under cystoscopy, a tumor (1 cm diameter) was found in the right posterior wall of the bladder. Transurethral resection of the bladder tumor was performed. The pathological outcome was intestinal metaplasia without malignancy. Preoperative abdominal computed tomography suggested vesico-appendiceal fistula, retrospectively. Therefore, appendectomy with partial cystectomy was attempted. However, the appendix was adhered to the sigmoid mesocolon, therefore, appendectomy, partial cystectomy, and sigmoid colectomy were performed. We diagnosed the tumor as mucinous adenocarcinoma. The patient has been receiving adjuvant chemotherapy with tegafur-gimeracil-oteracil potassium for 17 months, because he refused right hemicolectomy. There was no evidence of recurrence after 58 months of follow-up. Vesico-appendiceal fistula caused by appendiceal cancer is very rare. Our case is the 21st case reported in Japan.

[A case of percutaneous BCG perfusion therapy for CIS of upper urinary tract CIS after radical cystectomy with ileal neobladder].

We report a case of percutaneous bacillus Calmette-Guérin (BCG) perfusion therapy for carcinoma in situ (CIS) of upper urinary tract after radical cystectomy with ileal neobladder. A 42-year-old man underwent radical cystectomy and ileal neobladder diversion due to the recurrence of CIS in prostatic urethra after transurethral resection of bladder tumor 3 times and 2 courses of intravesical BCG therapy. Final pathological findings showed the presence of CIS in the right distal ureteral margin. After the radical cystectomy, our diagnosis was CIS in the right residual ureter, because of positive urine cytology and negative radiographic findings in the upper urinary tract. We performed the percutaneous BCG perfusion therapy for CIS of the right upper urinary tract after the construction of the percutaneous nephrostomy by intentionally inducing hydronephrosis. No recurrence was found after 3 years of BCG perfusion therapy.

Molecular epidemiology of cases of Mycoplasma californicum infection in Japan.

Bovine mastitis due to Mycoplasma californicum is often accompanied by huge economic losses, and the disease spreads very quickly. An appropriate molecular epidemiological analysis is needed to prevent and control infectious disease, but molecular epidemiological analysis methods for M. californicum have not yet been reported. Here we developed a combination of multiple-locus variable-number tandem repeat analysis (MLVA) and pulsed-field gel electrophoresis (PFGE) methods, which are common genotyping methods for various bacteria, for M. californicum. The MLVA is based on four interspersed repeat units that were found in the M. californicum genome data. The MLVA using these repeat units showed sufficient discriminatory power for a molecular epidemiological analysis; i.e., a Hunter-Gaston diversity index (HGDI) of 0.949, against M. californicum strains in Japan and M. californicum strain ATCC 33461. The PFGE for M. californicum also showed sufficient discriminatory power, with an HGDI of 0.985. Strain ATCC 33461 showed MLVA profiles and pulsotypes that differed greatly from those of strains from Japan. These results indicate that MLVA and PFGE are good tools for identifying M. californicum transmission events more accurately. Our combined MLVA and PFGE analysis suggests the persistence of M. californicum infection among herds in a specific area for a long period of time, as well as the movement of cows and heifers accompanying the expansion of M. californicum infection. Failure to identify asymptomatic infected cows is suspected as one of the central causes of the present M. californicum infection scenario in Japan.

[Condyloma acuminatum which developed in the neourethra post-urethroplasty for hypospasias; a case report].

We report here on a case of condyloma acuminatum in the neourethra after urethroplasty for hypospadias. A 37-year-old male visited an other clinic with the chief complaint of a meatal tumor. He had undergone a urethroplasty for hypospadias in his childhood. He was diagnosed as having condyloma acuminatum of the urethral meatus, and was referred to our hospital. Some other lesions in the neourethra were recognized under urethroscopy. We firstly resected the meatal tumor, and diagnosed it as condyloma acuminatum, pathologically. Thereafter, we performed transurethral resection of the urethral condyloma acuminatum. Recurrence of the condyloma acuminatum was noted after 6 months, postoperatively, so we performed TUR again. No recurrence has been seen to date.