ABSTRACT
VATER syndrome is defined as the combinations of three or more of the followings; Vertebral defect, Anal atresia, Esophageal atresia and/or Tracheo-Esophageal fistula, Renal dysplasia, and Radial-ray limb anomalies. A 21-month old female patient was admitted. She had the history of anastomosis of trcheo-esophageal fistula, V-P shunt operation due to hydrocephalus, and spina bifida. Upon admission, physical examinations showed increased deep tendon reflexes of all four extremities with grade 1 spasticity and popliteal angle being 45 degrees, bilaterally. The overall development of the patient was delayed. The imaging study showed hydrocephalus, periventricular leukomalatic change, underdevelopment of the cerebral cortex, atrophy of corpus callosum, Arnold-Chiary malformation, a vascular malformation along the intradural space from lower cervical to nearly entire thoracic vertebral level, multiple vertebral anomalies, and the fusion of multiple ribs. Furthermore hydronephrosis of the left kidney was detected. Follow-up of natural history and management of the patient is needed.
Subject(s)
Female , Humans , Anus, Imperforate , Atrophy , Cerebral Cortex , Corpus Callosum , Esophageal Atresia , Esophagus , Extremities , Fistula , Heart Defects, Congenital , Hydrocephalus , Hydronephrosis , Kidney , Muscle Spasticity , Natural History , Physical Examination , Radius , Reflex, Stretch , Ribs , Spinal Dysraphism , Spine , Trachea , Vascular MalformationsABSTRACT
Ten cases of VATER syndrome were reviewed to determine the type of anomalies, genitourinary anomalies, complications of treatment and prognosis. None of the four cases that were born in our hospital and six cases that were referred to us were diagnosed prenatally. Eight patients were born at full term with normal birth weight, two were premature and one showed pre-eclampsia at IUP 34 weeks.The male to female ratio was 6 to 3 with one patient having ambiguous genitalia. Tracheo-esophageal fistula was found in five patients. Three of these five patients died after the parents refused treatment, and one died the next day after birth even with treatment. Imperforate anus was observed in seven patients. Unilateral renal agenesis, crossed renal ectopia and dysplastic kidney were found in four, three (one pelvic kidney, one crossed renal ectopia with fusion, one crossed renal ectopia) and one patients, respectively. There was one hypospadias. Six of the eight patients with vertebrae anomaly survived; four of the survivors had vesicoureteral reflux (VUR) and neurogenic bladder. Two of the four patients with VUR received ureteroneocystostomy then clean intermittent catheterization (CIC), sympatholytic and antibiotics. The other two patients with neurogenic bladder received clean intermittent catheterization (CIC) and vesicostomy. Complications of recurrent urinary tract infection (UTI) and acute epididymitis occurred when descending loop colostomy was performed due to imperforate anus and urethro-rectal fistula. Separated colostomy instead of descending loop colostomy is recommended for recto-urinary fistula since infections of the upper urinary tract in such cases required ureteroneocystostomy before the correction of imperforate anus and continued antibiotic administration was needed because of the possibility of delayed wound healing and recurrent UTI. A strong will by the patient`s parents, protection of renal function through early detection of genit-ourinary anomalies and other appropriate treatments are required for the high success rate in the surgical correction of VATER syndrome.
Subject(s)
Child , Female , Humans , Male , Anti-Bacterial Agents , Anus, Imperforate , Birth Weight , Colostomy , Cystostomy , Disorders of Sex Development , Epididymitis , Fistula , Hypospadias , Intermittent Urethral Catheterization , Kidney , Parents , Parturition , Pre-Eclampsia , Prognosis , Spine , Survivors , Urinary Bladder, Neurogenic , Urinary Tract , Urinary Tract Infections , Vesico-Ureteral Reflux , Wound HealingABSTRACT
The VATER syndrome is a group of congenital anomalies with a nonrandom tendency for concurrence. Defects include vertebral, anorectal malformation, tracheoesophageal fisutla with esophageal atresia, radial-limb, vascular, and renal abnormalities. The critical period of organogenesis is at or before the sixth or seventh week of gestation. We experienced one case of VATER syndrome in a 1 day old male neonate having vertebral anomalies, esophageal atresia with tracheoesophageal fistula to the distal esophageal segment, imperforated anus, left renal dysplasia with hydronephrosis of the right kidney and both hydroureter, patent ductus arteriosus. We report a case of VATER syndrome with brief review of related literature.
Subject(s)
Humans , Infant, Newborn , Male , Pregnancy , Anal Canal , Critical Period, Psychological , Ductus Arteriosus, Patent , Esophageal Atresia , Hydronephrosis , Kidney , Organogenesis , Tracheoesophageal FistulaABSTRACT
The VATER syndrome is defined as a non-random association of congenital anomalies including three or more of vertebra(V), anal(A), tracheoesophageal(TE), and radial or renal(R) deficiencies. We experienced two cases of VATER syndrome; one is a 2-year-old male having imperforate anus. V-U reflux. polydactyly and secondary ASD and another one is a 17-year-old Female having lumbar scoliosis, imperforate anus, renal agenesis and didelphy uterus with ovarian cystadenoma.
Subject(s)
Adolescent , Child, Preschool , Female , Humans , Male , Anus, Imperforate , Cystadenoma , Polydactyly , Scoliosis , UterusABSTRACT
The VATER syndrome is defined as a nonrandom association of congenital anomalies including three or more of vertebral(V), anal(A), tracheoesophageal(TE) and radial or renal(R) deficiencies. We experienced one case of VATER syndrome in a 20 month-old male having lumbarization of sacrum, congenital imperforate anus (Ladd and Gross`s type 3) with urethrorectal fistula and left megaureter with right renal agenesis and urethrocutaneous fistula