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A complex of anomalies involving the vertebral column and spinal canal (V), anal atresia(A), congenital lesions of the heart (C), defects involving the trachea esophageal complex (TE), renal system, and urinary tract (R), and limb lesions (L) is known as VACTERL complex. VACTERL is an umbrella term for patients with abnormalities involving three or more of the systems mentioned above. It can be potentially life-threatening and should be promptly recognized and managed. Thorough investigations are required to prevent long-term sequelae and to improve morbidity. We present a case of a neonate born to a mother with twin gestation at 38 weeks of gestation with antenatally diagnosed severe polyhydramnios and a single umbilical artery and vein. This manuscript discusses the imaging findings of the congenital abnormalities involving the cardiac and skeletal system with tracheoesophageal fistula in our patient., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Sood et al.)

The clinical presentation of a wandering spleen is characterized mainly by unspecific acute symptoms, ranging from diffuse abdominal pain to left upper/lower quadrant and referred shoulder pain to asymptomatic. This has challenged accelerated medical care and impeded the acquisition of confirmatory diagnosis; therefore, increasing morbidity and mortality risks. Splenectomy is an established operative procedure for a wandering spleen. However, there has not been enough literature emphasizing the clinical history of congenital malformations and surgical corrections as inferential tools for facilitating a decisive and informed procedure. The case presented is of a 22-year-old female who reported to the emergency department with a five-day persistent left upper quadrant and left lower quadrant (LLQ) abdominal pain, associated with nausea. According to the medical history, the patient had a significant history of vertebral defects, anal atresia, cardiac anomalies, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL) associated with congenital anomalies. By the age of eight years, the patient had undergone multiple surgical interventions, including tetralogy of Fallot repair, an imperforate anal repair with rectal pull-through, Malone antegrade continence enema (MACE), and bowel vaginoplasty. Computed tomography imaging of the abdomen revealed evidence of a wandering spleen in the LLQ with associated torsion of the splenic vasculature (whirl sign). Intra-operatively, appendicostomy was identified extending from the cecum in a near mid-line position, to the umbilicus, and carefully incised distally, preventing injury to the appendicostomy. The spleen was identified in the pelvis, and the individual vessels were clamped, divided, and ligated. Blood loss was minimal with no post-operative complications. This rare case report adds valuable teaching points about the treatment of wandering spleen in individuals with VACTERL anomalies., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, French et al.)

Butterfly spine is a rare benign congenital abnormality. The onset of a minimum of three of the congenital malformations of vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities often characterises the VATER/VACTERL relationship. Recognising this anomaly is crucial for diagnosis, although this rare aberration is thought to be asymptomatic most of the time. Here we are describing a case of a one-year-old female child who has tetralogy of Fallot, congenital anal atresia, vesicovaginal fistula, and butterfly vertebrae which were found as an incidental finding. Furthermore we suggest screening all the children with any one abnormality of VACTERL, usually vertebral anomalies are screened., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Rao et al.)

Anorectal malformations (ARMs) can occur in isolation or in association with other anomalies, most commonly those of the genitourinary systems. Morbidity and mortality are highest among patients who develop end-stage renal disease (ESRD) either from severe congenital anomalies (dysplastic kidneys) or from repeated infections in those who have vesicoureteral reflux or persistent recto-urinary fistulas. We describe our management strategy for a patient born with an ARM and bilateral dysplastic kidneys to highlight the nuances and complex decision-making considerations required in taking care of this complex patient population. Our patient is a male twin born at 32 weeks' gestational age who was found to have bilateral dysplastic kidneys on prenatal ultrasound. On initial examination, an imperforate anus was identified along with a severe urethral stricture. Full workup also revealed sacral dysgenesis and confirmation of the dysplastic kidneys. On day of life 3, a laparoscopic diverting sigmoid colostomy was performed; urologic evaluation confirmed the severe urethral stricture, which required dilation to place an 8F council tip catheter. Due to his small size, peritoneal dialysis could not be initiated until five weeks of age. As full volumes could not be reached with peritoneal dialysis, he was soon transitioned to continuous renal replacement therapy. At five months of age, a laparoscopic-assisted posterior sagittal anorectoplasty (PSARP) was performed. As his urethral stricture had worsened, a suprapubic catheter had been placed for bladder decompression. Reversal of his colostomy was performed 15 days after PSARP. Unfortunately, the patient required three further surgical interventions due to abdominal wall and inguinal hernias contributing to filling and emptying dysfunction when utilizing peritoneal dialysis. He is currently 16 months of age and remains inpatient due to intermittent hemodialysis requirements along with autocycling of his peritoneal dialysis. He is working on developmental milestones, can pull to a stand, and is currently being evaluated for kidney transplantation. The development of ESRD in a neonate or infant with an ARM is rare and can be due to congenital dysplasia or agenesis of bilateral kidneys. While peritoneal dialysis is the preferred approach, catheter dysfunction can result from intra-abdominal adhesions or inadequate fluid removal from inguinal or abdominal wall hernias that form in the setting of increased intra-abdominal pressure required for peritoneal dialysis. Close collaboration is required between pediatric surgeons, nephrologists, and urologists to facilitate colonic and urologic reconstruction and manage catheter-related complications., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Svetanoff et al.)

VACTERL is a condition that includes various anatomic anomalies including vertebral, cardiac, tracheoesophageal fistula (TEF), renal, and limb defects. TEF can be found in up to 80% of patients with the condition. Although TEFs are usually identified early in life, the H-type TEF can be more difficult to detect. We report the case of a 15-year-old male with a previous diagnosis of VACTERL who presented with a history of recurrent pneumonia, chest pain, and asthma and was found to have a previously undetected H-type TEF that was surgically repaired. When evaluating a patient with features of VACTERL, it is important to choose studies that can explore the presence of all associated features. Clinical history and type of imaging utilized can be essential in making a timely diagnosis, especially for H-type TEF., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2020, Stack et al.)