Ductal stenting with bilateral pulmonary artery banding as a life-saving management for hypoplastic left heart syndrome with congenital esophageal atresia: A case series.

We present three cases of hypoplastic left heart syndrome (HLHS) complicated by congenital esophageal atresia and trachea-esophageal fistula (EA/TEF). The standard treatment for HLHS involves a staged surgical approach, eventually reaching Fontan completion. There is no report of patients with both HLHS and EA/TEF, and no established treatment strategy exists for such cases. Given the significant risk of simultaneously operating on HLHS and EA/TEF, we elected to pursue staged repair for each condition separately. Initially, soon after birth, we performed gastrostomy to secure the nutritional pathway for EA/TEF and stabilize breathing. Subsequently, we conducted bilateral pulmonary artery banding (bil-PAB) and ductal stenting for HLHS, as the Norwood operation carried an unacceptably high risk in these patients. Two of these patients were able to transition to home care, while the other patient died during hospitalization due to complications after EA repair. A combination of bil-PAB with ductal stenting for HLHS and staged repair for EA/TEF may provide effective management for patients with both conditions. Learning objective: Hypoplastic left heart syndrome (HLHS) and congenital esophageal atresia (EA) are both life-threatening conditions that require early intervention after birth. There are few reports of patients with both conditions, and no treatment strategy is established. Although the procedure carries a high risk, we successfully performed ductal stenting with bilateral pulmonary artery banding for HLHS, as well as staged repair procedures for EA. Our approach may be a viable strategy for these conditions.

Diagnose and treatment for Type D congenital esophageal atresia with tracheoesophageal fistula.

Importance: Type D esophageal atresia (EA) with tracheoesophageal fistula (TEF) is characterized by EA with both proximal and distal TEFs. It is a rare congenital anomaly with a very low incidence. Objective: To investigate diagnostic and treatment strategies for this rare condition. Methods: We retrospectively reviewed the clinicopathological features of patients with EA/TEF treated at our institution between January 2007 and September 2021. Results: Among 386 patients with EA/TEF, 14 (3.6%) had type D EA/TEF. Only two patients were diagnosed with proximal TEF preoperatively. Seven patients were diagnosed intraoperatively. Five patients were missed for diagnosis during the initial surgery but was later confirmed by bronchoscopy. During the neonatal period, seven patients underwent a one-stage repair of proximal and distal TEF via thoracoscopy or thoracotomy. Due to missed diagnosis and other reasons, the other 7 patients underwent two-stage surgery for repair of the proximal TEF, including cervical incision and thoracoscopy. Ten of the 14 patients experienced postoperative complications including anastomotic leakage, pneumothorax, esophageal stricture, and recurrence. Patients who underwent one-stage repair of distal and proximal TEF during the neonatal period showed a higher incidence of anastomotic leak (4/7). In contrast, only one of seven patients with two-stage repair of the proximal TEF developed an anastomotic leak. Interpretation: Type D EA/TEF is a rare condition, and proximal TEFs are easily missed. Bronchoscopy may aim to diagnose and determine the correct surgical approach. A cervical approach may be more suitable for repairing the proximal TEF.

Simultaneous type III congenital esophageal atresia and patent ductus arteriosus in a low-weight patient: A case report.

BACKGROUND: We report a low-birth-weight child (1.8 kg) with neonatal type III congenital esophageal atresia (CEA) combined with symptomatic patent ductus arteriosus (PDA). After comprehensive evaluation, esophageal anastomosis was performed on postnatal day 11 after excluding surgical contraindications, and arterial catheter ligation was performed at the same time. Concurrent surgery for CEA combined with PDA has not been clearly reported in the literature. CASE SUMMARY: We report a 6-day-old female child with type III CEA and PDA. The patient presented with foam at the mouth after birth, cough and shortness of breath after feeding. At another hospital, she was considered to have neonatal pneumonia, neonatal jaundice and congenital heart disease and transferred to our hospital. After iodine oil radiography of the esophagus and echocardiography we confirmed diagnosis of CEA and PDA. The diameter of the PDA was 8 mm, with obvious left to right shunting. We performed right rear extrapleural orificium fistula ligation and esophageal anastomosis, and ligation of PDA via left axilla straight incision after 5 d of hospitalization. The operations were successful, and the incision healed after 12 d, and the patient was discharged. We re-examined the patient 1 mo after surgery. She did not vomit when she ate rice flour. Esophageal angiography showed no stricture of the anastomotic stoma. The patient weighed 3.2 kg. CONCLUSION: For CEA patients with multiple risk factors, comprehensive, timely and accurate diagnosis and evaluation, and early treatment may improve prognosis.

Safety, efficacy and pharmacokinetics of palivizumab in off-label neonates, infants, and young children at risk for serious respiratory syncytial virus infection: a multicenter phase II clinical trial.

Background: Pediatric patients with certain rare diseases are at increased risk of severe respiratory syncytial virus (RSV) infection. However, the prophylactic use of anti-RSV antibody (palivizumab) in these patients is not indicated at present in Japan. Methods: This first-in-the-world multicenter, uncontrolled, open-label, phase II clinical trial was carried out between 28 July 2019 and 24 September 2021 at seven medical institutions in Japan to investigate the efficacy, safety, and pharmacokinetics of palivizumab in 23 subjects recruited from among neonates, infants, or children aged 24 months or younger who had any of the following conditions: pulmonary hypoplasia, airway stenosis, congenital esophageal atresia, inherited metabolic disease, or neuromuscular disease. At least four continuous doses of palivizumab were administered intramuscularly at 15 mg/kg at intervals of 30 days. Findings: Twenty-three enrolled subjects completed the study. No subject required hospitalization for RSV. Adverse events (AE) did not notably differ from the event terms described in the latest interview form. Five severe AEs required unplanned hospitalization, but resolved without RSV infection. Therapeutically effective concentrations of palivizumab were maintained throughout the study period. Interpretation: Palivizumab might be well tolerated and effective in preventing serious respiratory symptoms and hospitalization due to severe RSV infection, indicating the prophylactic use in the pediatric patients included in this study. Funding: Japan Agency for Medical Research and Development (AMED), grant numbers 19lk0201097h0001 (to MM), 20lk0201097h0002 (to MM), 21lk0201097h0003 (to MM), and 22lk0201097h0004 (to MM). AMED did not have any role in the execution of this study, analysis and interpretation of the data, or the decision to submit the results.

Effects of primary posterior tracheopexy in thoracoscopic repair of esophageal atresia.

Background: This study aimed to evaluate the effectiveness of primary posterior tracheopexy (PPT) in reducing ventilator dependence after repair of esophageal atresia (EA), and the risk of respiratory tract infections (RTI) requiring readmissions within one year. Methods: This retrospective cohort study recruited patients with EA admitted to our hospital between June 2020 and December 2021. Results: In the PPT group (n = 17), the time to extubation after surgery was 86.7 h for 12 patients, with one patient (8.3%) requiring repeated postoperation intubation; six-in-sixteen patients (37.5%) experience at least one RTI requiring hospitalization in one year. In the non-PPT group (n = 17), the time to extubation was 127.0 h for 14 patients, with six-in-fourteen patients (42.9%) requiring repeated intubation; twelve-in-seventeen patients (70.6%) experienced at least one RTI requiring hospitalization in one year. Conclusions: Although the differences did not reach statistical significance due to limited number of participants, patients underwent PPT during EA repair had lower chance of repeated intubation and decreased risk of RTI requiring admissions within one year.

Evaluation of safety and efficacy of autologous oral mucosa-derived epithelial cell sheet transplantation for prevention of anastomotic restenosis in congenital esophageal atresia and congenital esophageal stenosis.

BACKGROUND: We performed the first autologous oral mucosa-derived epithelial cell sheet transplantation therapy in a patient with refractory postoperative anastomotic stricture in congenital esophageal atresia (CEA) and confirmed its safety. In this study, patients with CEA and congenital esophageal stenosis were newly added as subjects to further evaluate the safety and efficacy of cell sheet transplantation therapy. METHODS: Epithelial cell sheets were prepared from the oral mucosa of the subjects and transplanted into esophageal tears created by endoscopic balloon dilatation (EBD). The safety of the cell sheets was confirmed by quality control testing, and the safety of the transplantation treatment was confirmed by 48-week follow-up examinations. RESULTS: Subject 1 had a stenosis resected because the frequency of EBD did not decrease after the second transplantation. Histopathological examination of the resected stenosis revealed marked thickening of the submucosal layer. Subjects 2 and 3 did not require EBD for 48 weeks after transplantation, during which time they were able to maintain a normal diet by mouth. CONCLUSIONS: Subjects 2 and 3 were free of EBD for a long period of time after transplantation, confirming that cell sheet transplantation therapy is clearly effective in some cases. In the future, it is necessary to study more cases; develop new technologies such as an objective index to evaluate the efficacy of cell sheet transplantation therapy and a device to achieve more accurate transplantation; identify cases in which the current therapy is effective; and find the optimal timing of transplantation; and clarify the mechanism by which the current therapy improves stenosis. TRIAL REGISTRATION: UMIN, UMIN000034566, registered 19 October 2018, https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000039393 .

Isoperistaltic gastric tube: a versatile, length-gaining option for esophageal replacement in children and adolescents.

Several substitutes, including gastric transposition, colonic interposition, reverse gastric tube (RGT), etc., have been described for esophageal replacement in children and adolescents. However, the search for the ideal esophageal substitute continues due to adverse events associated with these procedures. This report presents our technique for creating an isoperistaltic gastric tube (IGT). We believe that the IGT is a versatile option for esophageal replacement as it is never length-limiting, and additional length can be gained by making minor adjustments.

Clinical features of five cases of congenital esophageal atresia complicated with gastric perforation / 中国小儿急救医学

Objective:To investigate the clinical characteristics of congenital esophageal atresia with gastric perforation, and to improve pediatricians′ understanding of this disease.Methods:The clinical data of five neonates with congenital esophageal atresia and gastric perforation treated in the neonatal intensive care unit of the Affiliated Hospital of Qingdao University from 2012 to 2022 were analyzed retrospectively.Results:Among the five neonates, four were boys and one was girl.The gestational age was 28 + 5 to 37 + 6 weeks, the birth weight was 1 100~2 350 g. All of them had dyspnea and feeding difficulties after birth.Gastric perforation occurred in three cases during invasive mechanical ventilation, one case during non-invasive ventilation, and one case during nasal catheter oxygen inhalation.Emergency primary gastric repair was performed, followed by secondary esophageal anastomosis.All the patients were cured and discharged from hospital. Conclusion:Gastric perforation is a rare complication of congenital esophageal atresia, which is more common in premature infants and low birth weight infants.Mechanical ventilation may promote the occurrence of gastric perforation.If gastric perforation is complicated, repair should be performed as soon as possible, and esophageal anastomosis surgery should be performed early after stability to improve the final outcome.

Primary repair of esophageal atresia gross type C via thoracoscopic magnetic compression anastomosis: A case report.

BACKGROUND: Esophageal atresia (EA) is a life-threatening congenital malformation in newborns, and the traditional repair approaches pose technical challenges and are extremely invasive. Therefore, surgeons have been actively investigating new minimally invasive techniques to address this issue. Magnetic compression anastomosis has been reported in several studies for its potential in repairing EA. In this paper, the primary repair of EA with magnetic compression anastomosis under thoracoscopy was reported. CASE SUMMARY: A full-term male weighing 3500 g was diagnosed with EA gross type C. The magnetic devices used in this procedure consisted of two magnetic rings and several catheters. Tracheoesophageal fistula ligation and two purse strings were performed. The magnetic compression anastomosis was then completed thoracoscopically. After the primary repair, no additional operation was conducted. A patent anastomosis was observed on the 15th day postoperatively, and the magnets were removed on the 23rd day. No leakage existed when the transoral feeding started. CONCLUSION: Thoracoscopic magnetic compression anastomosis may be a promising minimally invasive approach for repairing EA.

Nuss procedure for combined pectus excavatum and carinatum in a patient with a history of congenital esophageal atresia repair surgery.

Cardiothoracic surgery usually causes tissue adhesion on the operation site which increases the risk of complications in the subsequent thoracic surgery including Nuss procedure. Disorders that require cardiothoracic surgery include chest wall deformities such as pectus excavatum, congenital heart diseases, lung diseases such as congenital cystic adenomatiod malformation and bronchopulmonary dysplasia, and congenital diaphragmatic hernia. Recently, we encountered a rare case of combined pectus excavatum and carinatum in a patient with a history of congenital esophageal atresia repair surgery. Commendably, despite tissue adhesion from the previous surgery, a modified Nuss procedure was performed successfully with no complications. We agree that the Nuss procedure is feasible for thoracic deformities in patients with a surgical history of cardiothoracic surgery.

First-in-human autologous oral mucosal epithelial sheet transplantation to prevent anastomotic re-stenosis in congenital esophageal atresia.

BACKGROUND: Congenital esophageal atresia postoperative anastomotic stricture occurs in 30-50% of cases. Patients with severe dysphagia are treated with endoscopic balloon dilatation (EBD) and/or local injection of steroids, but many patients continue to experience frequent stricture. In this study, we investigated the transplantation of autologous oral mucosa-derived cell sheets (epithelial cell sheets) as a prophylactic treatment for congenital esophageal atresia postoperative anastomotic stricture. METHODS: Epithelial cell sheets were fabricated from a patient's oral epithelial tissue, and their safety was confirmed by quality control tests. The epithelial cell sheets were transported under controlled conditions from the fabrication facility to the transplantation facility and successfully transplanted onto the lacerations caused by EBD using a newly developed transplantation device for pediatric patients. The safety of the transplantation was confirmed by follow-up examinations over 48 weeks. RESULTS: The dates that EBD was performed were recorded for one year before and after epithelial cell sheet transplantation, and the intervals (in days) were evaluated. For about 6 months after transplantation, the intervals between EBDs were longer than in the year before transplantation. The patients were also aware of a reduction in dysphagia after transplantation. CONCLUSIONS: These results suggest that cell sheet transplantation may be effective in preventing anastomotic stricture after surgery for congenital esophageal atresia, but the effect was temporary and limited in this case. Although we chose a very severe case for the first human clinical study, it may be possible to obtain a more definitive effect if the transplantation is performed before the disease becomes so severe. Future studies are needed to identify cases in which cell sheet transplantation is most effective and to determine the appropriate timeframes for transplantation. TRIAL REGISTRATION: UMIN, UMIN000034566, registered 19 October 2018, https://upload.umin.ac.jp/cgi-open-bin/ctr_e/ctr_view.cgi?recptno=R000039393 .

Research progress in diagnosis and treatment of long-term complications in patients with congenital esophageal atresia / 中华实用儿科临床杂志

Congenital esophageal atresia with or without tracheoesophageal fistula is a relatively common and serious congenital gastrointestinal anomaly, which can be surgically repaired to achieve long-term survival in the majority of patients.However, various consequent long-term complications would affect the physical and mental health of patients, thus inducing a decline in their quality of life.In this study, the present status and advances of diagnosis and treatment of these complications were reviewed, in an attempt to further explore the long-term complications after congenital esophageal atresia.

Anesthetic management of thoracoscopic procedures in neonates: a retrospective analysis of 45 cases.

BACKGROUND: Advances in medical techniques and equipment have enabled the thoracoscopic repair of certain congenital abnormalities in neonates including congenital esophageal atresia/tracheoesophageal fistula (EA/TEF) and congenital diaphragmatic hernia (CDH). A retrospective analysis was conducted to examine the anesthetic management of neonates (7 days or younger) undergoing thoracoscopic surgery in our hospital department, and to determine the efficacy of anesthetic management in neonates. METHODS: Clinical data from 45 neonates who underwent thoracoscopic surgery in our hospital from December 2015 to March 2020 were retrospectively analyzed. A total of 25 patients underwent repair of CDH and 20 underwent repair of an EA/TEF. RESULTS: All patients received general anesthesia with endotracheal intubation, standard ASA monitoring, and arterial blood gas (ABG) analysis. All patients survived the surgery. A total of 14 patients experienced decreases in SpO2, pH, PaO2, and increases in PETCO2 and PaCO2 30 minutes after CO2 insufflation. Our anesthetic management protocols are outline and analyzed. CONCLUSIONS: Thorough preoperative preparation is critical for a desirable outcome in neonates undergoing a thoracoscopic repair of CDH or EA/TEF. In our cohort, intraoperative ventilation strategies included pressure control ventilation with peak airway pressure maintained at 15-25 cmH2O, a respiratory rate of 35-55 breaths/minute, a fraction of inspired oxygen (FiO2) of 60-80%, an inspiratory/expiratory ratio (I:E) of 1:1-1.5, and careful airway suctioning to clear secretions. Postoperatively, maintaining normovolemia and hemodynamic stability are critical for successful weaning of ventilatory support and extubation.

Study protocol: a multicenter, uncontrolled, open-label study of palivizumab in neonates, infants, and preschool children at high risk of severe respiratory syncytial virus infection.

BACKGROUND: The prophylactic use of anti-respiratory syncytial virus (RSV) antibody (palivizumab) for severe RSV infection is not approved in Japan in specified groups of infants with neuromuscular diseases or other rare diseases associated with reduced ventilation competence or difficulty in expectoration, which increase the risk of exacerbation of severe RSV infection. The objective of this study is to investigate the efficacy, safety, and pharmacokinetics of palivizumab in pediatric patients with those rare diseases for which palivizumab is not indicated at present. METHODS/DESIGN: This study is a multicenter, uncontrolled, open-label study planned to be carried out between July 1, 2019 and June 30, 2022 at 7 medical institutions in Japan. The study population will be recruited from among neonates, infants, or children aged 24 months or younger with a condition falling under any of the following 5 disease groups: pulmonary hypoplasia, airway stenosis, congenital esophageal atresia, inherited metabolic disease, or neuromuscular disease. The planned sample size is 18 subjects, including at least 3 subjects per disease group. Throughout the RSV season, at least 4 continuous doses of palivizumab will be administered intramuscularly at 15 mg/kg at intervals of 30 days. The efficacy and safety of palivizumab will be comprehensively evaluated based on the incidence of RSV-related hospitalization, and serum palivizumab concentration, serum anti-palivizumab antibody concentration, and the occurrence of adverse events/reactions after the start of palivizumab treatment. DISCUSSION: This study will evaluate the efficacy and safety of palivizumab in pediatric patients with rare diseases which place them at high risk of severe RSV infection, but which fall outside the current indications for palivizumab prophylaxis. The generated data will have implications for the regulatory approval of prophylactic palivizumab treatment in this patient group. TRIAL REGISTRATION: This study has been prospectively registered in Japic Clinical Trials Information, which is managed and administered by the Japan Pharmaceutical Information Center (registration number: JapicCTI-194946 , registration date: September 10, 2019).

Therapeutic strategy for thoracoscopic repair of esophageal atresia and its outcome.

PURPOSE: Thoracoscopic repair can be safely performed in most types of congenital esophageal atresia (EA), including in patients with long gap EA or very low birth weight. Accordingly, we performed single- or multistage thoracoscopic repair for various EA types. We aimed to report our therapeutic strategy for thoracoscopic radical surgery for treating EA and its outcome. METHODS: Outcomes of radical surgeries for treating congenital EA at our institute from 2013 to 2018 were retrospectively evaluated. RESULTS: Thirty-eight radical surgeries were evaluated: 3 Gross type-A, 1 type-B, 30 type-C, 1 type-D, and 3 type-E. The cervical approach was performed in 5 cases and thoracoscopic esophageal anastomosis in 33, including 26 single-stage (all type-C) and 7 multistage surgeries (3 type-A, 3 type-C, and 1 type-D). There were no cases of thoracotomies or intraoperative thoracoscopic surgery complications. Three cases of minor leakage were conservatively resolved. Three postoperative chylothorax surgeries (9%) and seven balloon dilatations (21%) for anastomotic stenosis were performed. CONCLUSION: Thoracoscopic radical surgery for treating EA, including single- and multistage procedures, can be performed, except in type-E cases or when the end of the proximal esophagus is located higher than the clavicle.

Clinical efficacy of magnetic compression anastomosis for congenital esophageal atresia and stenosis / 中华消化外科杂志

Objective To investigate the clinical efficacy of magnetic compression anastomosis for congenital esophageal atresia and stenosis.Methods The retrospective and descriptive study was conducted.The clinical data of 4 children who underwent magnetic compression anastomosis for congenital esophageal atresia and stenosis in the Northwest Women and Children's Hospital from December 2017 and February 2019 were collected.There were 2 males and 2 females.The children were aged 11 days,7 days,5 days,and 3 years,respectively.The children underwent magnetic compression anastomosis.Observation indicators:(1) surgical and postoperative situations;(2) follow-up.Follow-up using outpatient examination and telephone interview was performed to detect food intake and complications of children up to May 2019.Measurement data with normal distribution were represented as Mean±SD,and measurement data with skewed distribution were represented as M (range).Results (1) Surgical and postoperative situations:four children underwent magnetic compression anastomosis successfully.Of the 4 children,3 with esophageal atresia underwent open tracheoesophageal fistula repair and endoscopeassisted magnetic compression anastomosis,and 1 with congenital esophageal stenosis underwent endoscopic gastrostomy combined with magnetic compression anastomosis.The operation time of 4 children was (2.3±0.9) hours.The length of esophageal blind ending in the 3 children with esophageal atresia and length of esophageal stenosis were in the children with esophageal stenosis 30-35 mm and 8 mm.Four children has good magnet apposition,and time of postoperative magnet removal was (29± 10)days.Three children with esophageal atresia had oral removal of magnet,and 1 with esophageal stenosis had magnet removed by gastrostomy.One child complicated with postoperative fistula and anastomotic stenosis was cured by unobstructed drainage and nutritional support treatment.The duration of postoperative hospital stay was (39± 10)days.(2) Follow-up:4 patients were followed up for 3-17 months,with a median time of 10 months,and restored to oral intake after oral removal of magnet and removal of magnet by gastrostomy on the days 14-36 postoperatively.One child was detected anastomotic stenosis by esophagography at the postoperative 3 months,and was improved after esophageal dilatation.The other 3 children recovered to normal connectivity of esophagus postoperatively and maintain unobstructed.Four children had normal eating,without dysphagia or other serious complications.Conclusion Magnetic compression anastomosis is safe and feasible for congenital esophageal atresia and stenosis,with good short-term efficacy.

Application of MSCT in the diagnosis of neonatal type (Ⅲ) congenital esophageal atresia / 实用放射学杂志

Objective To study the value of MSCT in the diagnosis of neonatal type (Ⅲ) congenital esophageal atresia.Methods MSCT data of 25 cases with neonatal type (Ⅲ) congenital esophageal atresia confirmed by surgery and pathology were analyzed retrospectively.The full preparation before examination and the image post-processing function were checked,and MSCT data were compared with the findings of surgery.Results By full preparation and application of M PR,SSD technology after scanning,the esophageal proximal blind end,and the distance between the two esophageal blind ends were clearly displayed,furthermore,the opening position,morphology and diameter of the distal tracheoesophageal fistula were also showed,and bronchial tree morphology and inflammation in the lung can be visible.Conclusion MSCT full preparation and application of image post processing function are potential to the clinical diagnosis of congenital esophageal atresia and tracheoesophageal fistula.

Clinical value of sagittal CT scanning in neonates with congenital esophageal atresia and tracheoesophageal fistula / 实用放射学杂志

Objective To explore the clinical value of sagittal CT scan in the diagnosis of congenital esophageal atresia and tracheoesophageal fistula(CEA-TEF).Methods Sagittal CT scanning was performed in 1 7 neonates with clinically suspected CEA-TEF preoperatively. Post-processing methods included multiple planar reconstruction(MPR)and volume rendering(VR).The CT manifestations were compared with the surgical findings.Paired-samples t test and Kappa identity analysis were used to analyze the consistency between two esophageal pouches and position of fistula.Results Among 1 7 cases,sagittal CT images clearly showed the distance between two esophageal pouches and position of fistula.Based on Gross classification,2 cases were included in type ⅢA(11.8%),and 15 cases in type ⅢB(88.2%).The results were satisfactory with the surgical findings with 100% accuracy.The distance between two esophageal pouches determined by VR and MPR had no remarkable differences(t=0.991,P>0.05).The position of fistula determined by VR and MPR had high consistency with the surgical findings (Kappa≥0.75).Conclusion Sagittal CT scanning can significantly limit scanning range and reduce the radiation dose as well as improve image quality,and accurately display and assess the distance between two esophageal pouches and position of fistula in CEA-TEF.It has important application value in diagnosing CEA-TEF in neonates.

Fibronectin glomerulopathy complicated with persistent cloaca and congenital esophageal atresia: a case report and literature review.

BACKGROUND: Fibronectin glomerulopathy is a rare, inherited, autosomal dominant, glomerular disease characterized by proteinuria, microscopic hematuria, hypertension, massive glomerular deposits of fibronectin, and slow progression to end-stage renal failure. Because the incident of fibronectin glomerulopathy is extremely low, the pathophysiology, genetic abnormalities, epidemiology, and mechanisms remain to be elucidated. CASE PRESENTATION: We report a 21-year-old woman with fibronectin glomerulopathy, who had been diagnosed with persistent cloaca and congenital esophageal atresia at birth. She developed proteinuria and hematuria 7 months before admission. Urinary protein and serum creatinine levels were 3.38 g/gCr and 0.73 mg/dL. Renal biopsy showed severe mesangial widening due to massive deposits, which was positive periodic acid-Schiff and negative methenamine silver. Immunostaining was negative for immunoglobulin but positive for fibronectin. Electron microscopy showed diffuse mesangial granular deposits. Thus she was diagnosed with fibronectin glomerulopathy, despite a negative family history of kidney disease and lack of any known missense mutations of fibronectin 1 gene. CONCLUSION: We report a patient who developed fibronectin glomerulopathy during the clinical course of extremely rare congenital malformations, including persistent cloaca and congenital esophageal atresia. We describe a case of this condition in detail and summarize the 75 case reports of fibronectin glomerulopathy.