Prognosis and clinical issues of esophageal atresia in extremely low birth weight neonates: a case series.

BACKGROUND: Esophageal atresia (EA) in extremely low birth weight (ELBW) neonates is rare. This report aims to clarify EA's clinical courses and prognosis in ELBW neonates and the clinical issues of long-term survival cases. METHODS: A retrospective analysis was conducted for 8 neonates diagnosed with esophageal atresia. Medical records of ELBW EA neonates treated at our institution were reviewed to assess patient demographics, clinical courses, and outcomes. Transferred patient data was obtained from their local physicians through questionnaires. RESULTS: EA in ELBW neonates were included in 8 of EA infants (7%). Fatal respiratory and cardiovascular complications of trisomy 18 and complications related to immaturity such as liver failure and pulmonary hypertension were associated with poor prognosis. During primary operations, gastrostomy and esophageal banding were performed together in 50% of the cases, while gastrostomy was performed alone in 25%. The esophageal anastomosis was not performed during any primary operation. All causes of death, except for 1 case, were due to non-surgical causes. A long-term survival case of 17 years postoperatively was included. CONCLUSION: Although ELBW EA has a poor prognosis, long-term survival is possible in some cases, so aggressive therapeutic intervention is considered essential. It is important to share information about the prognosis with parents and multidisciplinary specialists and to select an appropriate treatment strategy for each case.

Association between the confluent form of pancreatic and bile duct and histopathological findings in pancreaticobiliary maljunction: A case series study.

INTRODUCTION: Pancreaticobiliary maljunction (PBM) is a congenital anomaly wherein the persistent reflux of the pancreatic juice into the biliary tract induces biliary tract cancer. The prediction criteria for gallbladder carcinogenesis have been reported previously through results obtained from examining carcinogenic and non-carcinogenic cases with the parameters that classified each confluent form in PBM. This study aimed to validate these previous study results and provide new recommendations for gallbladder carcinogenesis prevention. METHODS: Twenty-four patients with PBM underwent hepaticojejunostomy. The prediction criteria for gallbladder carcinogenesis consist of three elements. The confluent forms that satisfied none or one of the three criteria were defined as a low score group, and those that satisfied two or three were defined as the high score group. Immunohistology and pathological search were performed on the gallbladders' sections in both groups to evaluate chronic inflammation. RESULTS: The cases with dysplasia, positive Ki67 labeling index, and gallbladder cancer were more common in the high score group and tended to have more lymphocyte infiltration. These findings indicate that the degree of inflammation and cell proliferation might be more severe in the high score group than in the low score group. CONCLUSIONS: There is a close relationship between the confluent form and the histopathological findings of the gallbladder in patients with PBM. The confluent forms observed in the high score group might have an additional correlation with increased proliferation activity and subsequent malignant transformation of the gallbladder epithelium.

Segmental Atrophy of Explanted Livers in Biliary Atresia: Pathological Data From 63 Cases of Failed Portoenterostomy.

OBJECTIVES: Atrophy of the left lateral segment (LLS) is often encountered in liver transplantation (LT) for biliary atresia (BA). To clarify the meaning of the heterogeneous atrophy, we compared the pathological characteristics of the LLS with the right posterior segment (RPS) of BA livers obtained during LT. METHODS: Among the 116 patients with BA who underwent LT at our hospital between 2014 and 2018, 63 patients with persistent cholestasis after the Kasai portoenterostomy (KP) were selected. Three pathologists evaluated tissues from the LLS and RPS for 5 pathological parameters. Positive areas in whole-slide image observed as portal inflammation, fibrosis, cholestasis, and ductular reaction, were analyzed with automated image quantitation. Moreover, we examined the relationship between the pathological score and the Pediatric End-stage Liver Disease (PELD) score. RESULTS: The median age at LT was 7 months (range 4-26 months). Inflammation and fibrosis were significantly greater in the LLS than in the RPS (P < 0.001, for both); however, there were no differences in cholestasis, ductular reaction, and hepatocellular damage (P = 0.3, 0.3, and 0.82). The same results were obtained in automated image quantitation. Moreover, the sums of the 5 pathological scores in the LLS showed a significant positive correlation with the PELD score (P = 0.016, rs = 0.3). CONCLUSIONS: More severe inflammation and fibrosis without cholestasis were observed in the LLS. The segmental atrophy may not be associated with poor bile drainage, but with etiopathogenesis of BA. Moreover, the proper site for biopsy during KP could be the LLS.

Successful abdominal wall closure following collagen-based artificial dermis induced epithelialization for giant omphalocele: A case report.

INTRODUCTION: A giant omphalocele (GO) with marked viscero-abdominal disproportion is associated with surgical difficulty and higher morbidity and mortality rates. Despite various treatment strategies, no consensus exists on optimal GO management. We report the clinical course of a neonate with a GO who was successfully treated with abdominal-wall closure through the novel application of collagen-based artificial dermis (CAD) for epithelization. PRESENTATION OF CASE: A female neonate (estimated gestational age, 38 weeks; birthweight, 3.047 kg) with a GO where most viscera, including the liver, were completely herniated. G-band analysis showed no chromosomal abnormality and normal karyotype. Conventional silo formation was attempted, but incomplete silo was formed due to adhesion between the portal vein and fascia, and repatriation of the herniated viscera had not progressed. A new silo was formed using biomaterial, but it was infected and removed. Abdominal wall epithelialization using NPWT was attempted again but was interrupted by the occurrence of jejunal perforation. After incising the epithelialized part of the abdominal wall and repairing the perforated jejunum, the GO was covered and fixed using CAD. Epithelialization progressed well, and she was discharged on day 328. DISCUSSION: In this case, the major therapeutic challenges (including formation of an incomplete silo, silo infection, and jejunal perforation) were overcome with conventional treatment except for epithelialization of the abdominal wall, which was achieved by using CAD. CONCLUSION: The treatment with CAD for epithelialization can be considered in cases where it is extremely difficult to return the viscera in conventional management.

Successful temporary resection of a ruptured hepatoblastoma without preoperative chemotherapy: A case report presenting a novel surgical strategy.

INTRODUCTION: Although spontaneous tumor rupture is a serious complication of hepatoblastoma, there is no consensus regarding the treatment strategy in infants. We report a patient with lung metastases who had a ruptured hepatoblastoma prior to the start of the scheduled chemotherapy and was successfully treated with a combined treatment including liver resection, lung resection, and chemotherapy. PRESENTATION OF CASE: A 22-month-old boy with a ruptured hepatoblastoma and lung metastases underwent an emergency laparotomy with complete tumor resection, followed by chemotherapy. Moreover, a barely detectable metastatic lung lesion shown by a chest CT scan was resected after the fifth chemotherapy treatment. Both postoperative and chemotherapy courses were uneventful. The patient survived without any recurrent hepatoblastoma 2 years after the emergency surgery despite the poor prognosis indicated by distant metastases at the time of diagnosis. DISCUSSION: Because rupture itself can be the main cause of death in patients with hepatoblastoma, emergency tumor hemostasis is essential. However, there are no reports comparing the prognosis of the treatment method performing tumor hemostasis alone, tumor resection after chemotherapy, and tumor hemostasis and resection at the same time. The clinical course of the patient indicates that performing tumor hemostasis and resection simultaneously and lung resection after chemotherapy is an effective option to treat a ruptured hepatoblastoma with disseminated tumors and lung metastases if the patient's condition is stable. CONCLUSION: Aggressive treatment with surgery and chemotherapy is an effective option for ruptured hepatoblastoma with disseminated tumors and lung metastases in infants.

Long-term outcomes after biliary diversion operation for pancreaticobiliary maljunction in adult patients.

AIM: Pancreaticobiliary maljunction (PBM) with or without congenital biliary dilatation (CBD) is a risk factor for biliary tract cancer. We investigated long-term outcomes after biliary diversion operation with special reference to types of CBD. METHODS: Subjects comprised 40 adult patients who underwent biliary diversion operation for PBM without biliary tract cancer. Group A comprised 20 patients with type Ia or Ic CBD, or non-dilated bile ducts, while group B comprised 20 patients with type IV-A CBD. The clinical findings and postoperative outcomes were compared between groups. RESULTS: Of 40 patients, nine patients suffered from repeated cholangitis and eight of these nine patients suffered from hepatolithiasis after biliary diversion operation. Biliary tract cancer or pancreatic cancer was detected in four patients at 3 years and 2 months to 24 years after the operation. In three of these four patients, the serum concentration of carbohydrate antigen 19-9 increased before detection of carcinoma. One patient died of hepatic failure due to repeated cholangitis. The proportions of patients with repeated cholangitis, hepatolithiasis, and re-operation, and patients who died of biliary tract cancer, pancreatic cancer, or hepatic failure, were significantly higher in group B than in group A. The survival rate was significantly worse in group B than in group A. CONCLUSIONS: Careful long-term follow-up with measurement of serum tumor markers is necessary after biliary diversion operation for PBM, especially in patients with type IV-A CBD or repeated cholangitis.

Successful treatment of metastatic cerebral recurrence of pleuropulmonary blastoma.

Pleuropulmonary blastoma (PPB) is a rare pediatric tumor. The central nervous system (CNS) is the most common site of extrathoracic metastasis. The prognosis of PPB patients with CNS metastases is dismal: most patients die within one year after recurrence. Here, we describe two patients diagnosed with PPB who developed intracranial recurrences shortly after the end of their initial treatment and were successfully treated by gross total resection, radiotherapy, and chemotherapy. Both patients are in complete remission four and three years after recurrence. Although an optimal regimen remains to be determined, these cases demonstrate that PPB with CNS metastases is potentially curable.

A Case of Apparent Life-Threatening Event: Comorbid Gastric Volvulus Associated Gastroesophageal Reflux Disease and Epilepsy in a 4-Month-Old Boy.

Most isolated episodes of apparent life-threatening events (ALTEs) do not lead to the diagnosis of serious conditions, and their prognoses are generally benign. However, recurrent ALTEs are often associated with a risk of future serious adverse events and should be evaluated for appropriate management. Here we present ALTE case in which gastric volvulus associated gastroesophageal reflux disease was detected as an etiology initially, followed by the detection of epilepsy as another etiology. Clinicians should consider possibility of two or more etiologies in a single recurrent ALTE case.

Familial Currarino syndrome associated with Hirschsprung disease: two cases of a mother and daughter.

Currarino syndrome with Hirschsprung disease (CS-HD) is extremely rare. We present the first family with CS-HD. Case 1: A 28-year-old woman was admitted with severe abdominal distension and dyspnea. She was diagnosed with anal stenosis, hemisacrum, anterior sacral meningocele (ASM), tethered cord (TC), and short-segment aganglionosis. She underwent the modified Duhamel operation after meningocele repair and cord detethering. A bicornuate uterus, bilateral ovarian dermoid cysts, and small rectal duplication were also noted intraoperatively. Case 2: The daughter of case 1 was admitted for abdominal distension and anal stenosis at the age of 17 days. Studies revealed a hemisacrum, ASM, TC, presacral mass, atrial septal defect, polyp in the right nasal cavity, right vesicoureteral reflux, and short-segment aganglionosis. She underwent the modified Soave operation at the age of 1 year and 4 months after meningocele repair, cord detethering, and resection of the presacral mass (epidermoid cyst). In both cases, the aganglionic segments were confirmed by preoperative rectal suction biopsy and postoperative pathological examination on full-thickness rectal specimens. Some causal genes for Currarino syndrome (CS) and Hirschsprung disease (HD) are currently investigated. Thus far, 10 CS-HD cases have been reported, including 6 cases of familial CS. However, all the patients had sporadic HD. Recent reports suggest that anomalies of the enteric nerve system contribute to postoperative constipation in CS cases.

[A case of advanced gastric cancer responding to neoadjuvant chemotherapy with S-1 and CDDP combination].

The patient is a 75-year-old woman who received 2 courses of neoadjuvant chemotherapy (NAC) with S-1/CDDP for advanced gastric cancer and para-aortic lymph node metastasis. After completion of the second course, both the primary tumor and lymph node metastases practically disappeared. Later, total gastrectomy was performed. Histopathological examination revealed cancer cells remaining in just one part of subserosa (ss), and no lymph node metastases were detected. The postoperative course was favorable, and the patient is currently attending the outpatient clinic.