Coronary arteriovenous fistula (CAVF) is a rare condition defined as an anomalous termination of the coronary arteries. The etiology of CAVF is either congenital or acquired, and iatrogenic CAVF is most commonly caused by cardiovascular surgery or percutaneous intervention. Most of the prenatally diagnosed CAVFs were related to complex heart disease, and only few cases of an isolated CAVF have been reported to date. We successfully diagnosed an isolated CAVF by fetal echocardiography at 25.3 weeks of gestation. Accurate prenatal diagnosis resulted in the prompt decision for postnatal surgical correction, and the neonate thrived well without any complications.
Arteriovenous Fistula ; Coronary Vessel Anomalies ; Coronary Vessels ; Echocardiography ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Pregnancy ; Prenatal Diagnosis

PURPOSE: The congenital H-type rectovestibular fistula, a fistula between the anorectum and genital tract besides a normal anus is a rare variant of anorectal deformities. This disease needs proper treatment but there are no standard of diagnosis and treatment. The purpose of this report is to review a 13-year experience of approach and management for H-type rectovestibular fistula at a single institution. METHODS: From February 2002 to August 2015, we cared for 11 patients who had congenital H-type rectovestibular fistula and reviewed their clinical presentation, accompanied anomalies, diagnostic modalities, operative technique, and postoperative progress. RESULTS: Most patients with H-type rectovestibular fistula presented with symptoms including vestibular defecation and major labial abscess. We could find the fistula tract in most of patients by fistulography using contrast dye. All of the patients had been operated. There were 2 recurrences after surgical treatment who had inflammation and infection associated with the fistula. All other patients recovered without complications. CONCLUSION: We think the operation including fistulectomy and repair of perineal body through a transanal approach can be a feasible option to the congenital H-type rectovestibular fistula. Also, combined inflammation and infection should be treated prior to surgery to reduce postoperative complications.
Abscess ; Anal Canal ; Congenital Abnormalities ; Defecation ; Diagnosis* ; Fistula* ; Humans ; Inflammation ; Postoperative Complications ; Recurrence

Pulmonary arteriovenous fistula (PAVF) is abnormally dilated vessels that provide a right-to-left shunt between pulmonary artery and pulmonary vein and is clinically divided into simple and complex type. Here, we report four cases of surgically resected sporadic PAVFs presenting various clinical and histologic spectrums. Cases 1 (a 57-old-female) and 2 (a 54-old-female) presented as incidentally identified single aneurysmal fistulas and the lesions were surgically removed without complication. On the other hand, case 3 (an 11-old-male) showed diffuse dilated vascular sacs involving both lungs and caused severe hemodynamic and pulmonary dysfunction. Embolization and surgical resection of the main lesion failed to relieve the symptoms. Case 4 (a 36-old-male) had a localized multiloculated cyst clinically mimicking congenital cystic adenomatoid malformation. Microscopically, the lesion consisted of dilated thick vessels, consistent with the diagnosis of fistulous arteriovenous malformation/hemangioma.
Aneurysm ; Arteriovenous Fistula* ; Arteriovenous Malformations ; Cystic Adenomatoid Malformation of Lung, Congenital ; Diagnosis ; Fistula ; Hand ; Hemodynamics ; Lung ; Pulmonary Artery ; Pulmonary Veins

Rectovestibular fistula is the most common type of anomaly found in a female newborn with anorectal malformation. However, when the baby is found to have two orifices in the introitus, rectovaginal fistula is much less common and suspected. The rare differential diagnosis of Müllerian agenesis, a condition in which the rectum shifts anteriorly and the vagina is absent, is seldom considered. In many cases, the diagnosis of Müllerian agenesis is made only during definitive anorectoplasty. In view of its impact on management, a proper examination under anaesthesia, imaging studies and a diagnostic laparoscopy may be required to confirm the presence or absence of Müllerian structures in such patients. We herein describe a patient with the rare coexistence of VACTERL association and Müllerian agenesis, and discuss the management of anorectal malformations in female patients with Müllerian agenesis.
Abnormalities, Multiple ; diagnosis ; Anal Canal ; abnormalities ; surgery ; Anorectal Malformations ; Anus, Imperforate ; complications ; diagnosis ; surgery ; Child ; Diagnosis, Differential ; Esophagus ; abnormalities ; Female ; Heart Defects, Congenital ; complications ; Humans ; Infant, Newborn ; Kidney ; abnormalities ; Laparoscopy ; Limb Deformities, Congenital ; complications ; Mullerian Ducts ; abnormalities ; Rectal Fistula ; diagnosis ; Rectum ; abnormalities ; surgery ; Spine ; abnormalities ; Trachea ; abnormalities ; Vagina ; abnormalities

We report a case of congenital lacrimal sac fistula located on the temporal side of the lateral canthus. A systemically healthy 1-year-old girl came to the outpatient clinic with a complaint of tearing on the temporal side of the right lateral canthus since birth. On examination, a small orifice was found in the skin on the temporal side of the lateral canthus. There was no evidence of inflammation or swelling within the opening. Surgeons carried out an operation under general anesthesia. They passed a probe through the lacrimal orifice and advanced it toward the lacrimal sac. Next, they introduced saline to the inferior punctum and found that it drained to the lateral fistula. The lower lid stretched as the dissected fistula was pulled. After the operation, the patient was free of the symptom. This paper is to report a case of congenital lacrimal sac fistula located on the temporal side of the lateral canthus.
Diagnosis, Differential ; *Eye Abnormalities ; Female ; Fistula/*congenital/diagnosis ; Humans ; Infant ; Lacrimal Apparatus/*abnormalities ; Lacrimal Apparatus Diseases/*congenital/diagnosis

OBJECTIVECongenital esophageal stenosis owing to tracheobronchial remnants (TBR) is a rare condition. This study was conducted to understand the clinical features of TBR.

METHODThe data of the four cases with TBR admitted to our hospital and 76 patients identified from the literature were reviewed. The clinical manifestation, X-ray, endoscopy, biopsy and treatment were studied retrospectively.

RESULTOf the total of 80 cases, 45 were male, 33 were female, and for 2 cases the gender was unknown. Symptoms of dysphagia and regurgitation developed at the age of 1-day to 12-month. Definitive treatment was carried out at the age of 1-month to 16-year. Twenty-seven patients had associated anomalies with esophageal atresia being the most prevalent. X-ray esophagography showed segmental stenosis at the distal third of the esophagus in all patients except three. An abrupt narrow segment at the lower esophagus with marked proximal dilatation was found in 32 cases. Esophagography of 12 cases showed distal esophageal stenosis with tapered narrowing. Esophagography of 20 cases showed flask-shaped shadow of distal esophageal stenosis and one patient showed linear projection of barium at the level of stenosis. Endoscopy found almost complete obstruction of the lower esophageal lumen without signs of the esophagitis or reflux. Esophagoscopic dilatation of the stenosis was attempted in 24 cases, but was ineffective, and 3 patients suffered esophageal perforation. Seventy-nine patients underwent resection of the stenotic segment. Histologic examination of the resected specimen showed cartilage, mucus glands, resembling bronchal tissue. Post-operative complication included anastomotic stenosis, anastomotic leakage, hiatal hernia, and gastroesophageal reflux.

CONCLUSIONTBR should be suspected in patients who present with a typical history of dysphagia after ingestion of solid food. Esophagography and esophagoscopy are the essential means for diagnosis. TBR should be different from achalasia and should be diagnosed by biopsy. Operation is the only choice of treatment.

Anastomosis, Surgical ; Barium Sulfate ; Biopsy ; Child, Preschool ; Choristoma ; complications ; Dilatation ; Esophageal Atresia ; complications ; Esophageal Perforation ; etiology ; Esophageal Stenosis ; congenital ; diagnosis ; pathology ; surgery ; Esophagoscopy ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Postoperative Complications ; epidemiology ; Retrospective Studies ; Tracheoesophageal Fistula ; etiology

OBJECTIVETo explore the clinical characteristics, diagnosis and surgical treatment of adult congenital bronchoesophageal fistula.

METHODSEleven cases of adult congenital bronchoesophageal fistula that were diagnosed and surgically treated between May 1990 and August 2010 had been reviewed. There were 7 male and 4 female patients, ranging in age from 28 to 66 years (mean 48.7 years). The chief clinical presentation included coughing and sputum in 10 cases, recurrent bouts of coughing after drinking liquid food in 6 cases, hemoptysis in 6 cases, low fever in 4 cases, chest pain in 3 cases. The duration of symptoms before diagnosis ranged from 5 to 36 years (mean 16.8 years). The diagnosis of bronchoesophageal fistula was confirmed most by esophagography. Associated diseased lung was resected in all patients (lobectomy in 10 cases and pneumonectomy in 1 case). The operation included right thoracotomy in 7 cases and left thoracotomy in 4 cases. The fistula was completely resected in 10 cases. The tract was simply divided and the end was sutured in 1 case.

RESULTSThe postoperative course was uneventful in 10 patients who were discharged from hospital 10 to 18 d after operation. One patient suffered from esophageal fistula and received second operation. Regular follow-up was conducted on all 11 patients, proving that 3-year survival rate was 11/11 and 5-year survival rate was 9/11.

CONCLUSIONPersistence of congenital bronchoesophageal fistula into adulthood is rare. The main symptom is nonspecific coughing and bouts of coughing after drinking liquid food. The most useful diagnostic method is the esophagography. Even though it is benign disease, life-threatening complications might occur and it must be treated surgically as soon as the diagnosis is established.

Adult ; Aged ; Bronchial Fistula ; congenital ; diagnosis ; surgery ; Esophagoscopy ; Female ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Survival Rate ; Thoracotomy

To discuss the diagnosis and therapy of congenital vesicovaginal fistula (VVF). We reported 1 case of pregnancy and congenital VVF and summarized the pathogenesis and characters. Congenital VVF is extremely rare and characterised by continuous urinary leakage,cyclical hematuria with severe suprapubic pain. It is always associated with other urinary tract abnormalities, urinary tract infection and stone. The pathogenesis is related with genetic, environment, drugs, radiation and incomplete fusion of mullerian. The diagnosis relies on clinical manifestation and radiological examinations.Surgical resection is the key to treatment. Pregnancy and congenital VVF is extremely rare and always misdiagnosised associated with other urinary tract abnormalities. Early diagnosis and making sure the location, size and relationship with other tissues are important. Reasonable preoperative preparation, surgical and postoperative surgical care are the key for treatment.
Adult ; Female ; Humans ; Pregnancy ; Pregnancy Complications ; diagnosis ; Vesicovaginal Fistula ; congenital ; diagnosis

PURPOSE: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. METHODS: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. RESULTS: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were 37(+2) weeks and 2.5+/-0.7 kg, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. CONCLUSION: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.
Anal Canal ; Birth Weight ; Cause of Death ; Dilatation ; Duodenostomy ; Esophageal Atresia ; Esophageal Stenosis ; Esophagus ; Extremities ; Gastroesophageal Reflux ; Gastrostomy ; Gestational Age ; Heart Defects, Congenital ; Humans ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Kidney ; Limb Deformities, Congenital ; Prenatal Diagnosis ; Retrospective Studies ; Sepsis ; Spine ; Trachea ; Tracheoesophageal Fistula

A coronary arteriovenous (AV) fistula consists of a communication between a coronary artery and a cardiac chamber, a great artery or the vena cava. It is the most common anomaly that can affect coronary perfusion. Yet bilateral involvement of a coronary fistula, constitutes an uncommon subgroup of coronary AV fistulas. We herein report on a case of bilateral coronary AV fistula that was coexistent with variant angina originating from the distal right ventricular branch of the right coronary artery and the distal septal branch of the left anterior descending artery, and the latter drained into the right ventricle.
Angina Pectoris, Variant/*etiology/radiography ; Coronary Vessel Anomalies/*complications/*diagnosis ; Female ; Heart Ventricles/*abnormalities ; Humans ; Middle Aged ; Vascular Fistula/complications/*congenital/*diagnosis