Beckwith-Wiedemann syndrome is a rare congenital malformation,1 which is mainly characterized by giant tongue, macrosomia, and abdominal wall defect. Clinically, the patient has ear creases or notches, abdominal visceral hypertrophy, hypoglycemia, facial nevus flammeus, and other manifestations can also be seen. Embryonic tumors in childhood are relatively high,2 and the mortality rate is extremely high. At present, there are no effective treatment measures for reported cases in China, which can only be supported by symptomatic treatment. Careful and correct nursing is beneficial to the long-term prognosis and recovery of the cases. Recently, a case of newborn Beckwith-Wiedemann syndrome was treated in our department. After careful nursing, the child was discharged smoothly from hospital 34 d after the operation. Now, the nursing measures are introduced as follows.
The sex of the baby is male. During a pregnancy screening on the baby’s mother forming part of the regular prenatal check-up, an umbilical tumor was discovered, and a case of omphalocele was confirmed. After prenatal consultation in our hospital, it was recommended to refer to our department for treatment after birth. Cesarean section was performed at 36 weeks of pregnancy on August 6, and the birth weight of the child was 3500 g. After endotracheal intubation, the baby was transported to our department through the green channel. After admission, the child had respiratory failure and neonatal shock, and was administered invasive ventilator-assisted ventilation, symptomatic acid-base correction and volume expansion treatment, symptomatic anti-infection, albumin supplementation, and correction of coagulation function. The physical examination in the hospital found that the upper body of the child was hypertrophic, with nevus erythematosus on the face and forehead, slightly wider eye distance, neck webbed on both sides, and wide and hypertrophic tongue. After endotracheal intubation, the tongue was found full and protruded out of the mouth, and the navel protruded out into a cystic mass, pink, about 3 cm × 2 cm × 2 cm in size, soft in quality. The package is free of damage and ascites fluid. After hospitalization, the child was diagnosed with Beckwith-Wiedemann syndrome through improving various auxiliary examinations. On August 13, under general anesthesia, laparotomy, intestinal torsion reduction, duodenal rhombic anastomosis, left anterior descending diagonal (LADD), omphalocele repair, appendectomy, and intestinal adhesion lysis were performed. After the operation, the patient was transferred back to the surgical intensive nursing unit with tracheal intubation, assisted ventilation with invasive ventilator, intravenous anti-infection, fluid volume restriction, and diuretic treatment with pump. As a result of this routine, the edema gradually relieved. Anemia was corrected by infusion of suspended red blood cells, and yellow light irradiation was used. On the third day after the operation, tracheal tube was removed and assisted ventilation was given using a non-invasive ventilator. To prevent the child’s tongue from falling back and compressing the airway, prone and lateral positions were used, and the nasal catheter oxygen inhalation was gradually shifted to restore enteral nutrition. The blood routine and inflammatory indexes were generally normal after reexamination. The child was discharged from the hospital after his condition was stable on September 10.
Immediately after delivery, the child was placed on a radiation rescue table to ensure warmth, considering that it is easy for the capsule to lose a significant portion of its water content when exposed to a constant temperature environment, and its brittleness gradually increases. It may easily rupture in the process of being absorbed back, causing abdominal infection and organ prolapse in the capsule, which would directly affect the operation and treatment outcome. In severe cases, it will lead to the death of the child. Doctors immediately treated the bulging capsule of umbilicus, wrapping it with oil gauze and scald gauze, and then covering it with plastic wrap to prevent dryness of capsule, swelling of intestinal tube, and ischemic necrosis caused by water loss.3 During the operation, attention should be paid to ensuring gentle movements directed at preventing capsule rupture.
Hypoglycemia is the most characteristic manifestation of Beckwith-Wiedemann syndrome.4 Immediately after admission, the blood glucose of the child was measured as 3 mmol/L; the child was in a state of continuous fasting, and the blood glucose was measured every 2 h according to the doctor’s advice. Hypoglycemia was blood glucose lower than 2.2 mmol/L, while 2.6 mmol/L is the limit value that needs to be treated clinically, and rehydration treatment needs to be carried out according to the doctor’s advice when there is a deviation from the limit. When the blood glucose value is low, the doctor should notified in time for treatment. Further, during the infusion period, it is necessary to ensure that the child can metabolize drugs at an average speed.
After being admitted to the hospital, the child was rescued and assisted with tracheal intubation ventilator. During transit, the child repeatedly underwent bruising under the condition of pressurized oxygen supply from endotracheal intubation sac. In Pressure Control Valve (PCV) mode, the oxygen supply concentration gradually decreased from 100% to 65%, the peak pressure decreased from 35 cm H2O to 30 cm H2O, the blood oxygen saturation of the child was maintained at 90%–100%, and the blood pressure of the child was low. Peripherally Inserted Central Catheter (PICC) catheterization was performed on the child according to the doctor’s advice, dopamine was pumped to maintain blood pressure, and arterial puncture catheterization was used to monitor arterial blood pressure. After doctors introduced catheterization, the child was supervised with hourly monitoring of inflow and outflow.
We closely observed the alarm prompts of various parameters of the ventilator during ventilator-assisted ventilation, checked the depth of endotracheal intubation in each shift, removed secretions in endotracheal intubation and nasal cavity as needed, used closed sputum suction tube, strictly performed the aseptic operation, avoided cross infection, and monitored blood gas analysis according to doctor’s advice, so that doctors were able to adjust the oxygen concentration of ventilator and the frequency of mechanical ventilation according to the results. In this case, the tongue was hypertrophic and exposed, which increased the risk of bacterial infection. Therefore, we found it very important to do oral nursing as well. Oral nursing was performed with normal saline cotton swabs every 4 h to prevent ventilator associated pneumonia (VAP). On the day following the operation, the child was given sedative and analgesic drugs, and his spontaneous breathing was weak; so, he still needed invasive ventilator-assisted ventilation through tracheal intubation, which was administered using the parameters of PCV mode, an oxygen concentration of 30%, PEEP3 cm H2O, and PC9 cm H2O, and his oxygen saturation remained at 92%–100%. On the third day after the operation, the child had spontaneous breathing, and we switched to Continuous Positive Airway Pressure (CPAP) mode of invasive ventilator-assisted ventilation, characterized by the parameters FiO240% and PS15 cm H2O, and we did not observe any dyspnea or hypoxia. The vital signs of the child were stable, and the blood gas results were satisfactory. After full sputum suction, the child was removed from the tracheal intubation, and non-invasive ventilator-assisted ventilation was administered, together with monitoring of the parameters biphasic f40 times/min, FiO240%, and PEEP4–5 cm H2O. The transition was made 18 d after the operation. During the transition period, 2 L/min of oxygen inhalation was facilitated by nasal catheter; and 18 d after operation, and the child had giant tongue, which easily caused the tongue to fall back and lead to respiratory obstruction.5 The gravity in the supine position will aggravate tongue fallback and respiratory obstruction. Therefore, giving the child’s head a high lateral position or prone position is beneficial in keeping the respiratory tract unobstructed, thus reducing or avoiding the occurrence of tongue fallback.6 Alternating the left and right lateral positions with prone position can also avoid skin pressure and ventilator associated pneumonia (VAP), which are typically caused by maintaining a single position over a prolonged period of time. Nurses are always around when the child is in prone position to prevent suffocation.
The reported critical value blood glucose of the child was 2.19 mmol/L on the day after operation, and the retest blood glucose was 3.8 mmol/L after 50% glucose + sterilized water was injected using static push pump according to the doctor’s advice. Therefore, it was still necessary to continue monitoring the blood glucose level dynamically after the operation. Accordingly, after conclusion of the operation, we measured the blood glucose q4h, and used PICC catheter to infuse intravenous nutrient solution at a constant speed for 24 h during fasting; resultantly, we could maintain the blood glucose level at 3.3–5.2 mmol/L. Once breast feeding was permitted for the infant, we could gradually reduce the administration of parenteral nutrition solution; however, during this reduction, the imminent need for frequent/continuous monitoring for hypoglycemia always remained. Since the clinical manifestations of hypoglycemia are nonspecific, neonatal hypoglycemia is less likely to be found.7,8 If newborns exhibit frequent blinking, sucking, apnea, sweating, and other manifestations, we should attach great importance to these phenomena, check the limb activities of such newborns, and take definite preventive measures to avoid brain damage caused by hypoglycemia.
A solid mass was found palpable in the left costal abdomen of the child. B-ultrasound indicates that the child has a giant kidney and the left renal pelvis is separated. There were 4 reports of blood biochemical critical value after blood biochemical examination, and the urea was 13.2–19.8 mmol/L. Urea is one of the end products of amino acid metabolism, which can reflect the status of renal function. Therefore, it is necessary to pay attention to the monitoring of renal function and access in children diagnosed with or suspected to have Beckwith-Wiedemann syndrome. The most common clinical manifestation is decreased urine output. Therefore, it is necessary to record the inflow and outflow in children at every hour, and inform the doctor in time when the inflow and outflow are unbalanced. The child in the present study had decreased urine output and edema of limbs after the operation. According to the doctor’s advice, under such conditions, children need to be administered pump albumin plus furosemide to maintain a certain urine output, reduce the heart load, and prevent tissue edema and renal tubular blockage.9
Most children with this syndrome have difficulty in feeding. During fasting, the nutritional needs of children are met through intravenous high nutrition. After tracheal intubation is removed, the child is given a disposable nipple for non-nutritional sucking. After swallowing function is gradually coordinated, the child may slowly transit to oral feeding. The child began to drink water on the 9th day after operation and milk on the 11th day. Since the child has a huge tongue, which often extends out of the mouth, it is obviously more difficult to feed than normal children.10 When the child has been placed in the prone and lateral positions, we put a towel of appropriate height underneath the head and shoulders of the child to raise the head and shoulders by 20–30 cm, and try to make the airway straight and unobstructed to reduce the degree of dyspnea and prevent or reduce vomiting. Additionally, it is ensured that when feeding, the child’s head is turned to one side; and the child’s swallowing situation and complexion need to be continuously monitored, together with similarly scrutinizing whether there is asphyxia in the child, as evidenced by bluishness of skin. After feeding, the child should be picked up and patted on the back to help eliminate the gas swallowed into the stomach when sucking milk.
Since children with this syndrome have a huge tongue, which often extends out of their mouths, sucking and normal occlusion will be more significantly affected with age. During the time of the children’s food-intake, we must observe their complexion and breath, and with the increase of age, there may be slurred enunciation and speech disorder.11 For the treatment of giant tongue, parents are told to come to the hospital regularly for reexamination. If it is too large or grows too fast, it needs to be trimmed.12 Attention is given to the body position that the child assumes, since, to prevent the tongue from falling back and compressing the airway, it is necessary to ensure that the child takes a lateral or prone position. After discharge, it is ideal to have an oxygen inhalation device at home for urgent use.
The sick children can survive for a long time, and their mental retardation is often a consequence of the damage caused by severe hypoglycemia to the nervous system during the neonatal period. Therefore, the prevention and treatment of hypoglycemia during the neonatal period is a key step in treatment. Before discharge, parents should be advised to buy a household blood glucose meter to monitor blood glucose changes at least once a day, and to pay attention to whether the children have hypoglycemia: If the child exhibits poor spirit, slow response, lethargy, sweating, hypothermia, weak sucking, milk rejection, weak crying, apnea, irritability, convulsions, limb convulsions, pale or cyanotic complexion, cold limbs, and hypotonia, it is necessary to measure blood glucose immediately and feed the child with glucose water or milk powder.13
The child’s blood alpha-fetoprotein (AFP) ≥2000 ng/mL during hospitalization indicates that it falls into a high-risk group. Parents should be informed of the necessity for outpatient follow-up after discharge, together with the tentative schedule outlined for the follow-up: Beckwith-Wiedemann syndrome may be accompanied by multiple malformations and the tendency of abdominal malignant tumors,14 and thus long-term follow-up is required after the operation. Generally, outpatient follow-up is conducted once every 2 weeks after discharge, and once a month after 3 months. Follow-up can be done every 3 months for 1 year after the operation. Since the disease is a monogenic genetic disease, which conforms to the autosomal recessive inheritance law, if genetic testing is carried out, the examination results confirm that the couple should not have a second child after diagnosis.15
Beckwith-Wiedemann syndrome is a rare congenital malformation. Therefore, targeted nursing measures should be implemented in perioperative nursing. Before conducting the operation, we need to handle and observe the omphalocele, monitor the blood glucose, and observe the condition closely; after the operation, airway care, blood glucose monitoring, renal function observation, and feeding observation should be performed well. At the time of discharging the child, we need to conduct a program of health education for the parents, and this should ideally include teaching them how to observe giant tongue, communicating the importance of monitoring blood glucose and participating in regular follow-up of tumor markers, and providing information about preliminary measures that can be taken at home to relieve the common illnesses observed in children with this syndrome; such a program would be immensely helpful in promoting early recovery and achieving long-term high-quality survival in children with Beckwith-Wiedemann syndrome.
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