VACTERL associations in children undergoing surgery for esophageal atresia and anorectal malformations: Implications for pediatric surgeons

Lautz, Timothy B.; Mandelia, Ankur; Radhakrishnan, Jayant

doi:10.1016/j.jpedsurg.2015.02.049

Cited by 42 publications

(17 citation statements)

References 26 publications

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“…In 36% of cases, these may occur as part of the VACTERL spectrum (vertebral anomalies, anorectal atresia, congenital heart malformations, tracheoesophageal fistula, renal abnormalities, and limb defects) or CHARGE syndrome (coloboma, heart defects, atresia choanae, retardation of growth and/or development, genital hypoplasia, and ear deformities). Trisomies of chromosomes 18 and 21, Di George syndrome and Pierre Robin syndrome are other significant risk factors for CEA ± TEF [ 7 , 8 , 9 ]. These underlying diseases may be detected before birth.…”

Section: Introductionmentioning

confidence: 99%

Respiratory Morbidity in Children with Repaired Congenital Esophageal Atresia with or without Tracheoesophageal Fistula

Patria

Ghislanzoni

Macchini

et al. 2017

IJERPH

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Congenital esophageal atresia with or without tracheoesophageal fistula (CEA ± TEF) is a relatively common malformation that occurs in 1 of 2500–4500 live births. Despite the refinement of surgical techniques, a considerable proportion of children experience short- and long-term respiratory complications, which can significantly affect their health through adulthood. This review focuses on the underlying mechanisms and clinical presentation of respiratory morbidity in children with repaired CEA ± TEF. The reasons for the short-term pulmonary impairments are multifactorial and related to the surgical complications, such as anastomotic leaks, stenosis, and recurrence of fistula. Long-term respiratory morbidity is grouped into four categories according to the body section or function mainly involved: upper respiratory tract, lower respiratory tract, gastrointestinal tract, and aspiration and dysphagia. The reasons for the persistence of respiratory morbidity to adulthood are not univocal. The malformation itself, the acquired damage after the surgical repair, various co-morbidities, and the recurrence of lower respiratory tract infections at an early age can contribute to pulmonary impairment. Nevertheless, other conditions, including smoking habits and, in particular, atopy can play a role in the recurrence of infections. In conclusion, our manuscript shows that most children born with CEA ± TEF survive into adulthood, but many comorbidities, mainly esophageal and respiratory issues, may persist. The pulmonary impairment involves many underlying mechanisms, which begin in the first years of life. Therefore, early detection and management of pulmonary morbidity may be important to prevent impairment in pulmonary function and serious long-term complications. To obtain a successful outcome, it is fundamental to ensure a standardized follow-up that must continue until adulthood.

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Section: Introductionmentioning

confidence: 99%

Respiratory Morbidity in Children with Repaired Congenital Esophageal Atresia with or without Tracheoesophageal Fistula

Patria

Ghislanzoni

Macchini

et al. 2017

IJERPH

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“…Intravenous antibiotics such as ampicillin are recommended for prevention of urinary tract infections (UTIs) and broadened as required by clinical status. The team should prepare for a nasogastric sump tube insertion to evaluate for an esophageal atresia/tracheoesophageal fistula, present in 7–11% of patients with ARM [ 6 , 26 ]. Heightened vigilance to identify other VACTERL-associated anomalies during the physical exam is needed.…”

Section: Management In the Neonatal Periodmentioning

confidence: 99%

“…In 30–70% of non-syndromic patients with anorectal malformations (ARMs), a combination of congenital anomalies of multiple systems known as the VACTERL (V-Vertebral, A-Anorectal, C-Cardiac, TE-tracheoesophageal-, R-Renal, L-Limb) association occurs. The diagnosis of the VACTERL association requires that three or more of these conditions are present [ 4 , 5 , 6 ]. Patients found to have a cloacal malformation require an expeditious workup of all additional congenital anomalies because they may be associated with life-threatening or urgent conditions.…”

Section: Introductionmentioning

confidence: 99%

Fetal and Newborn Management of Cloacal Malformations

et al. 2022

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Cloaca is a rare, complex malformation encompassing the genitourinary and anorectal tract of the female in which these tracts fail to separate in utero, resulting in a single perineal orifice. Prenatal sonography detects a few cases with findings such as renal and urinary tract malformations, intraluminal calcifications, dilated bowel, ambiguous genitalia, a cystic pelvic mass, or identification of other associated anomalies prompting further imaging. Multi-disciplinary collaboration between neonatology, pediatric surgery, urology, and gynecology is paramount to achieving safe outcomes. Perinatal evaluation and management may include treatment of cardiopulmonary and renal anomalies, administration of prophylactic antibiotics, ensuring egress of urine and evaluation of hydronephrosis, drainage of a hydrocolpos, and creation of a colostomy for stool diversion. Additional imaging of the spinal cord and sacrum are obtained to plan possible neurosurgical intervention as well as prognostication of future bladder and bowel control. Endoscopic evaluation and cloacagram, followed by primary reconstruction, are performed by a multidisciplinary team outside of the neonatal period. Long-term multidisciplinary follow-up is essential given the increased rates of renal disease, neuropathic bladder, tethered cord syndrome, and stooling issues. Patients and families will also require support through the functional and psychosocial changes in puberty, adolescence, and young adulthood.

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“…Серцеві та аномалії сечовивідних шляхів є найпоширенішими, а шлунково-кишкові, інші, ніж атрезія стравоходу, зазвичай зустрічаються дуже рідко. Високі ПАВР демонструють вищу частоту асоційованих аномалій [13].…”

Section: вступunclassified

Staged surgical management of anorectal atresia associated with type II colon atresia and urinary system anomalies in a newborn boy (case report and literature review)

Джам¹,

Sliepov²,

Soroka³

et al. 2021

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Anorectal malformation is a congenital anomaly and can be either isolated or associated. This malformation is often combined with other congenital defects, in particular VACTERL-association (anomalies of the spine, anal region, heart, trachea, esophagus, kidneys and extremities), which requires a comprehensive diagnosis in these patients. Congenital malformations of the gastrointestinal tract except for esophageal atresia are rare. The paper presents a clinical case of associated congenital anomalies of the gastrointestinal tract and urinary system in a newborn boy: congenital anorectal malformation without fistula, type II sigmoid atresia with bowel malrotation, vesicoureteral reflux, right-sided pyelectasia and coronal hypospadias. Comprehensive diagnostics before each stage of surgical treatment made it possible to determine the method and technique of surgical correction. This made it possible to preserve a part of the intestine which was presented in the form of an enterocyst to create a neorectum as well as to diagnose the functional incompetence of the preatritic segment of the colon and carry out its removal. Atresia of the sigmoid colon is not a common concomitant malformation in congenital anorectal malformation, but it should be taken into account in the differential diagnosis in newborns with progressive abdominal distension and anorectal atresia without fistula. The proposed staging of the surgical management of these associated malformations should be regarded as organ-preserving, allowing to restore the normal function of the gastrointestinal tract and urinary system, with a good functional result. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of these Institutes. The informed consent of the patient was obtained for conducting the studies. The authors declare no conflicts of interests. Key words: anorectal atresia without fistula, sigmoid atresia, vesicoureteral reflux, coronary hypospadias, surgical management, newborn boy.

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VACTERL associations in children undergoing surgery for esophageal atresia and anorectal malformations: Implications for pediatric surgeons

Cited by 42 publications

References 26 publications

Respiratory Morbidity in Children with Repaired Congenital Esophageal Atresia with or without Tracheoesophageal Fistula

Respiratory Morbidity in Children with Repaired Congenital Esophageal Atresia with or without Tracheoesophageal Fistula

Fetal and Newborn Management of Cloacal Malformations

Staged surgical management of anorectal atresia associated with type II colon atresia and urinary system anomalies in a newborn boy (case report and literature review)

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