Infant, Newborn ; Humans ; Pyriform Sinus/surgery* ; Neck ; Fistula/congenital* ; Retrospective Studies ; Pharyngeal Diseases/congenital*

Adolescent ; Adult ; Arteriovenous Fistula ; surgery ; therapy ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; surgery ; therapy ; Heparin ; therapeutic use ; Humans ; Infant ; Male ; Middle Aged ; Pulmonary Artery ; abnormalities ; surgery ; Pulmonary Veins ; abnormalities ; surgery ; Retrospective Studies ; Telangiectasia, Hereditary Hemorrhagic ; surgery ; therapy ; Young Adult

Animals ; Brain ; growth & development ; Child ; Eyelids ; abnormalities ; Female ; Humans ; Intellectual Disability ; genetics ; Limb Deformities, Congenital ; genetics ; Mice ; Microcephaly ; genetics ; Mutation, Missense ; N-Myc Proto-Oncogene Protein ; genetics ; Tracheoesophageal Fistula ; genetics

PURPOSE: To present a rare case of tear drainage since birth from a lacrimal ductule fistula, which is the first report in the Republic of Korea. CASE SUMMARY: 3-month-old female who presented with discharge of clear fluid from a small skin opening lateral to the right upper eyelid since birth visited the outpatient clinic. The patient was healthy and was receiving no medication. She was born on gestation age 35 weeks and 3 days. No other specific history or other ophthalmic abnormality was found. On examination, a small skin orifice approximately 2 mm diameter and no sign of infection or discoloration was observed. To relieve this symptom, the patient underwent a fistulectomy under general anesthesia. Histopathological examination of the surgical specimen was performed. The patient's symptom subsided postoperatively without recurrence. CONCLUSIONS: This is the first report in the Republic of Korea of tears draining from a lacrimal ductule fistula since birth and the subsequent clinical treatment procedures.
Ambulatory Care Facilities ; Anesthesia, General ; Congenital Abnormalities ; Drainage ; Eyelids ; Female ; Fistula ; Humans ; Infant ; Lacrimal Apparatus ; Parturition ; Pregnancy ; Recurrence ; Republic of Korea ; Skin ; Tears

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

Vertebral anomalies-anal atresia-cardiac abnormalities-tracheoesophageal fistula-renal agenesis-limb (VACTERL) defects association is a rare congenital disease. While most scientific literature focus on the clinical presentation and management of pediatric patients with this condition, this paper focuses on the challenges faced by a 22-year-old primigravid, who was able to carry a pregnancy to term, despite the many anomalies associated with being afflicted with VACTERL.
Humans ; Tracheoesophageal Fistula ; VACTERL association ; Limb Deformities, Congenital

Many congenital and acquired defects occur in the maxillofacial area. The buccal fat pad flap (BFP) is a simple and reliable flap for the treatment of many of these defects because of its rich blood supply and location, which is close to the location of various intraoral defects. In this article, we have reviewed BFP and the associated anatomical background, surgical techniques, and clinical applications. The surgical procedure is simple and has shown a high success rate in various clinical applications (approximately 90%), including the closure of oroantral fistula, correction of congenital defect, treatment of jaw bone necrosis, and reconstruction of tumor defects. The control of etiologic factors, size of defect, anatomical location of defect, and general condition of patient could influence the prognosis after grafting. In conclusion, BFP is a reliable flap that can be applied to various clinical situations.
Adipose Tissue* ; Congenital Abnormalities ; Humans ; Jaw ; Necrosis ; Oroantral Fistula ; Prognosis ; Re-Epithelialization ; Transplants

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

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

After cleft lip repair, many patients suffer from nasolabial fistulas, asymmetrical nasal floor, or an indistinct nostril sill, as well as intraoral wound dehiscence and subsequent scar contracture of surgical wounds leading to vestibular stenosis. For successful primary nasolabial repair of complete cleft deformity of the primary palate, cleft surgeons need special care in reconstructing the sound nasal floor. Especially when the cleft gap is wide or when any type of nasoalveolar molding therapy was not performed, three-dimensional reconstruction of the nasal floor is critical for a balanced nasal shape. In this study, the author describes an effective method for reconstructing a double-layered nasal floor using two mucosal flaps from both sides of the fissured upper lip. This is a report of six patients with unilateral or bilateral complete cleft of the primary palate with a detailed description of the surgical technique and a literature review.
Cicatrix ; Cleft Lip ; Cleft Palate ; Congenital Abnormalities* ; Constriction, Pathologic ; Contracture ; Fistula ; Fungi ; Humans ; Lip* ; Palate* ; Wounds and Injuries