Medical Management of VACTERL Association

Abstract

VACTERL association is a collection of congenital malformation caused by various factors. Normally, there must be at least three of its constituents for a condition to be defined as a VACTERL association. The constituent includes V: Vertebral anomalies; A: Anal malformation; C: Cardiovascular defect; TE: Tracheal and esophageal malformation; R: Renal agenesis; and L: Limb anomalies. The fact that its incidence is rare translates to limited information regarding its medical management. For this reason, the medical management of the VACTERL association is complex.

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In an effort to expand knowledge and understanding regarding the condition, this paper explores its medical management. In that regard, its treatment, surgical options, and both care plan are discussed. The research advocated that patients with potentially critical medical conditions that constitute the VACTERL association should receive urgent and appropriate multidisciplinary medical care.

Keywords: VACTERL association, medical management, preoperative and postoperative care

VACTERL Association Meaning

VACTERL association, also known as VACTERLS or VATER association is a cluster of congenital malformations caused by multiple factors (Shaw-Smith, 2006). Typically, the VACTERL association is defined by the diagnosis of at least three constituents of the baseline congenital malformations. These include

V: Vertebral anomalies;

A: Anal malformation;

C: Cardiovascular defect;

TE: Tracheal and esophageal malformation;

R: Renal agenesis;

L: Limb anomalies (NIH, 2016).

Since the cause is unidentified, there are no standardized laboratory tests that can rule out or diagnose the association (Castori, 2016). Therefore, its diagnosis is based on visible features. According to the NIH (2016), and Thulasidoss, Duraisamy, and Babu (2015), the incidence of VACTERL association is approximately one patient in the range of 10,000 to 40,000 infants. Additionally, most of the reported cases are sporadic, implying that they occur in individuals with no medical history of the condition in their lineage. In other words, it is rare to register multiple incidences in a single-family.

Due to the sporadic incidence of VACTERL association, few cases are reported and studied. Logically, its medical management calls for a comprehensive approach to the diagnosed conditions. For these reasons, it is relatively complex to manage it. This paper provides a discussion of the medical management of the VACTERL association. In that respect, it explores its treatment, surgical options, preoperative and postoperative plans of care.

Medical Management of Patients with VACTERL Association

As highlighted in the introduction, the management of patients with VACTERL association is relatively complex compared to other conditions. The complexity is tied to the fact that all the issues related to each constituent condition must be addressed without compromising other conditions (Solomon, 2011). Normally, the management of VACTERL association patients will be divided into two major phases. In the first stage, health providers address conditions that would complicate the life of the patients (Solomon, 2011).

In other words, the healthcare specialists must manage all conditions that would make the life of a VATER association patient unbearable. Some of the conditions that must be addressed within this stage of medical management include imperforate anus, TEF, and severe cardiovascular malformations (NIH, 2016). Such conditions as managed through respective surgeries during the neonatal period or immediately medical circumstances allow surgical options (Solomon, 2011).

For example, the imperforate anus is often treated through an immediate colostomy, which can be followed by “pull-through” operation or re-anastomosis (Levitt & Peña, 2007). In addition, comorbid genitourinary malformations are managed through this staged approach. Normally, TEF is repaired through a single surgery; however, developing surgical complications or the re-emergence of the fistula may demand more surgical procedures (Spitz, 2007).

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The second stage of managing the VACTERL association entails the medical management constituent conditions that are less critical. Since most of the malformation can develop into enduring sequelae, it is important to identify them and address them based on their severity. As noted by Solomon (2011), it is important to identify all subtle congenital malformations because they are often medically important, as their management improves medical care. One of the subtle yet vital congenital malformations includes vertebral anomalies, which tend to develop into back pain in the later stage of life (Raam, Pineda-Alvarez, Hadley, & Solomon, 2011).

Other congenital malformations that require urgent medical attention fall under the renal anomalies. Renal anomalies tend to decline renal function, nephrolithiasis and infections. For this reason, managing the VACTERL association demands that health providers should factor in all long-term issues linked to each congenital malformation (Ahn, Mendoza, Kaplan, & Reznik, 2009). In addition, irrespective of the considerable morbidity linked to constituent congenital malformations, it is important to note that VACTERL association patients rarely display neurocognitive impairments (Levitt & Peña, 2007).

Therefore, the diagnosis of any neurocognitive impairment must be addressed through an appropriate diagnosis (Wheeler & Weaver, 2005). To improve the efficacy of management approaches, patients and families must be advised to seek advanced treatment as soon as possible. By seeking specialized care among multidisciplinary specialists, patients receive an opportunity to have better outcomes after the treatment.

Treatment and Surgical Options

The treatment for each patient with VACTERL must be individualized due to significant variations in component congenital malformation. It is worth noting that the treatment of component congenital malformations is center on the symptoms that are visible in each patient, but varies greatly. A diagnosis is made by conducting a physical examination of the neonate. Additionally, doctors may conduct various specialized tests meant to identify both minor and major features of the component VACTERL association defects (Castori, 2016).

Normally, prognostic classifications should serve as the basis of treatment (NIH, 2016). A prognosis classification should be used to provide effective guidance; especially among patients will multiple congenital malformations. However, early and timely identification of the most critical anomaly serves as the foundation of effective treatment (UCSF, 2015). Most of the structural deformities including heart defects, anal atresia, and radial defect are corrected surgically (Raam, Pineda-Alvarez, Hadley, & Solomon, 2011).

Surgical options vary with the type of repair needed for each congenital malformation. In the same line, surgical techniques vary with pathologic anatomy and the surgeon’s preferences. Typically, VACTERL association malformations are treated through multiple surgeries depending on the severity of the defect.

V: Vertebral Anomalies

Spinal surgery is common for vertebral anomalies and underlying costovertebral anomalies. As noted by Castori (2016), thoracic insufficiency syndrome (TIS) is treated through a vertical expandable prosthetic titanium rib (VEPTR). The congenital condition is marked by severe deformities of the ribs, chest, and spine. As of consequence, it prevents normal lung development and breathing (Castori, 2016). Patients with scoliosis or back pain undergo laminectomy. Other surgical options for vertebral anomalies include cervical laminectomy, occipital stabilization, and spinal cord stimulation.

A: Anal Malformation

Surgery for anal atresia or imperforate anus is often performed a few days after birth. In severe cases, surgeries for full reconstruction of the anal canal and intestine are performed. Atresia entails the repair without colostomy (UCSF, 2015). On the other hand, imperforate anus covers either colostomy followed by reanastomosis or complete correction immediately after the birth.

C: Cardiovascular Defect

The most common defects include atrial septal defect (ASD), ventricular septal defect (VSD), and tetralogy of fallot (TOF). Other defects include patent ductus arteriosus (PDA), transposition of the great arteries, coarctation of the aorta, and truncus arteriosus. Cardiovascular defects, such as atrial septal defect and ventricular septal defect, require specialized heart surgeries including truncus arteriosus correction and correction of the transposed arteries.

TE: Tracheal and Esophageal Malformations

Tracheal and esophageal malformations are often repaired within the initial day of neonatal care. Typically esophageal atresia and tracheoesophageal fistula repair is achieved through the primary anastomosis, which entails thoracotomy or extrapleural approach (Richards, Dillow, & Percesepe, 2014). For cases where the primary anastomosis is complicated to perform, a staged procedure is conducted. Some of the factors that can limit primary anastomosis include low general condition, long gap, and other major malformations.

The staged procedure entails feeding jejunostomy, abdominal esophagostomy, and cervical esophagostomy. If both congenital heart disease and TEF are present, TEF repair precedes all the surgeries. Tracheal stenosis entails slide tracheoplasty, whereas esophageal stenosis involves dilation of the esophagus via balloon. Laryngotracheoesophageal clefts (LTEC) is treated through external surgery or endoscopy (Solomon, 2011). Other surgical options include diaphragmatic hernia repair, thoracoscopic aortopexy (Tracheomalacia), and GOR.

 

R: Renal Agenesis

Renal anomalies are often critical especially when both kidneys are defective. Additionally, they can be severe where there are multiple urological problems including obstruction of urine outflow from the kidney (Richards, Dillow, & Percesepe, 2014). Other renal anomalies include ectopic or dysplastic kidney and nephrolithiasis. For these imperfections, surgery is largely performed in order to prevent damage from urological and kidney complications, such as persistent cloaca abnormalities. In the same context, genitourinary abnormalities are treated in a staged manner through reconstructive surgery. Primary repair covers hypospadias and rectovaginal fistula.

L: Limb Anomalies

Limn anomalies included absent, displaced or hypoplastic thumb/s, syndactyly, polydactyly and radial hypoplasia/dysplasia; clubfoot; tibia deformities; and hypoplasia of great toe (Richards, Dillow, & Percesepe, 2014). Normally, bilateral limb defects have urological or kidney problems on both sides of the body. Unilateral limb defects have the same issues on the same side. The most common surgical option for limb anomalies is plastic surgery. In this context, severe degrees of thumb hypoplasia are treated through pollicization. Plastic surgery is the most preferred option for wrist radialization polydactyly and syndactyly.

Preoperative and Postoperative Plans of Care

The complexity of the surgery performed for most VACTERL association defects requires general anesthesia. Care must always be taken during caudal in VACTERL association patients, particularly for those having genitourinary abnormalities, imperforate anus as they normally have occult spinal dysraphisms (Richards, Dillow, & Percesepe, 2014). In the process of planning for neuraxial technique, it is recommended that a spine ultrasound must be performed to minimize complications during the surgery (Ahn, Mendoza, Kaplan, & Reznik, 2009). Ultrasound helps in accurate needle placement under neuraxial surgery techniques, especially when spinal pathology is present. Ventilation should be adjusted to ensure that there is sufficient oxygenation.

Preoperative Care

The preparation of a neonate for congenital malfunction surgery entails various measures including administration of Prophylactic broad-spectrum IV antibiotics; administration of Intravenous (IV) fluid with adequate glucose concentration; and keeping the neonate warm using overhead warmer or incubator (Saxena, 2016). Perioperative warming includes activities performed in the preoperative, intraoperative, and postoperative environments of surgical operations designed to stabilize the patient’s body temperature (Kumar, Wong, Melling, & Leaper, 2005).

Since the temperature difference in the operating room environment causes the patient’s body heat to flow outwards, patients are often at risk of critical hypothermia. Morbidity linked to severe hypothermia entalis infection, cardiac events, delayed postanesthetic recovery, and coagulopathy (Sydnor & Perl, 2011). Delayed wound healing due to the mentioned issues can lead to prolonged hospitalization (Kumar, Wong, Melling, & Leaper, 2005). For this reason, it is important to monitor the temperature of VACTERL association patients during and after the surgery.

Since multiple body systems are affected by VACTERL association, there is a need to carry baseline investigations to help the clinicians eliminate or establish the severity of the VACTERL association to traditional observations including weight and height.

Skin infection poses a significant health risk to patients. For this reason, it is important to minimize skin infection during and after the surgeries (CDC, 2014; Melny & Fineout-Overholt, 2005). Skin infections can elongate the average length of hospital stay among VACTERL association patients, thus resulting in the induced complications (Sydnor & Perl, 2011). Further hospital induced skin infections can cause pain and discomfort of the infants after surgery; thereby reducing their quality of life.

Postoperative Care

Postoperative care entails monitoring patients after the surgery. Monitoring depends largely on the type of surgery performed, as well as the preoperative medical condition (Levitt & Peña, 2007). Typically, incubated patients are moved from the operative environment to a neonatal ICU. Antibiotics must be administered until the wound begins to heal (Saxena, 2016). Suctioning may be necessary to remove the oral secretions that might be built up after the surgery. Normally, a secretion taper is applied as swelling reduces. Necessary radiographs should also be obtained to establish the number of complications. For example, a chest radiograph helps the doctors to study the esophagus after surgery; hence, aiding in assessing the efficacy of repair and the progress of the corrected malformation (Saxena, 2016).

As a result, the chances of survival are increased. Additionally, the radiograms are used as the foundation for preparing progressive surgeries. Some of the key operations, including cardiac surgery and laryngeal cleft demand intensive care admission after surgery for continued ventilation, fluid management, and hemodynamic monitoring (Raam, Pineda-Alvarez, Hadley, & Solomon, 2011). Close observation is necessary for the prompt response to any potential complications that may develop after surgery. Other important activities that are conducted during postoperative care include monitoring complications induced by antibiotics, analgesia, and parental nutrition (Shaw-Smith, 2006; Thulasidoss, Duraisamy, & Babu, 2015).

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Tetrology of Fallot patients are susceptible to respiratory infections, such as pneumonia and atelectasis, because their tracheal muscles are weak. For these reasons, such patients must be given active respiratory healthcare with antibiotics and physiotherapy both preoperatively and postoperatively. Other issues to be monitored in the postoperative environment include tracheomalacia and gastro-oesophageal reflux, which may threaten the life of patients (Richards, Dillow, & Percesepe, 2014).

If all is well after surgery and the neonate and its parents have been briefed regarding the things they should help the patient heal, a meaningful follow-up is needed to manage the patient. Some of the measures that should be taken under the long-term monitoring effort include returning the patient to the hospital for assessment; the surgeon should assess the patient’s condition, healing, and growth; the surgeon should also interview the parents and initiate contact with the physician responsible for the medical care of the patient (Saxena, 2016).

These efforts are meant to ensure that any complications are detected in time and addressed effectively. Normally, the community physician is responsible for the care of the patient after the surgery; and the release from the hospital is briefed about the baby’s health condition, healing, and growth.

Conclusion

From the discussion above, it is evident that the management of the VACTERL association is complex; hence it demands multidisciplinary care. As highlighted in the review and discussion above, multidisciplinary special care is vital for high-risk patients with complex surgical and medical morbidities. With improved surgical techniques in specialized neonatal, as well as postoperative settings, the diagnosis of VACTERL association will result in a better prognosis.

However, even via optimized surgical corrections of congenital malformations, including TEFF, limb abnormalities, and cardiac anomalies, patients are likely to develop complications late in their life. It substantiates the initial hypothesis that managing patients with VACTERL association is complex. Usually, the neonate may experience pain. Additionally, there may be soreness and swelling around the operated area. In order to avoid panic, the surgeon must inform the patient’s parents about the expected side effects.

In that regard, parents may be first to note any unexpected behaviors or developments after the surgery due to their direct contact with the baby. Some of the complications or unplanned events that may be observed and communicated to the responsible physician or surgeon include reaction to anesthesia and excessive bleeding. Despite the complexity, special consideration should be made for surgical options to reduce the complication induced due to surgical errors. Additionally, patients and their families should be encouraged by the clinicians to seek further care where necessary in order to achieve the best possible health outcome.

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