Anesthetic management of two cases of Beckwith-Wiedemann syndrome

Two cases of children with Beckwith-Wiedemann syndrome are presented. This syndrome consists of various abnormalities, including macroglossia, visceromegaly, omphalocele, and gigantism. These abnormalities frequently require operative correction during the neonatal period. We anesthesiologists should make plans for difficult airway managements in patients with this syndrome. Our two patients also showed larger sized tracheas than those estimated by their age and height. A cuffed tube, though still controversial, has recently been used in children. We recommend using a cuffed tube in patients with this syndrome, because the appropriate tracheal size may not be predictable, tracheal intubation might be difficult, and risks incurred during changing of a tracheal tube should be avoided.     

Anaesthesia and the Beckwith-Wiedemann syndrome

Infants with Beckwith-Wiedeman syndrome usually present different abnormalities which may require surgical correction. Anaesthetic management may be complicated by abnormal airway anatomy, congenital heart disease and severe hypoglycaemia. Careful preoperative evaluation, perioperative monitoring and suitable choice of anaesthetic technique are required for a successful outcome. We report the perioperative management of a patient with Beckwith-Wiedemann syndrome presenting for omphalocoele surgery on his first day of life and for bilateral inguinal hernia repair four months later.     

Intraneural perineurioma in a child with Beckwith-Wiedemann syndrome

To the authors' knowledge, this is the first report of an intraneural perineurioma in a child with Beckwith-Wiedemann syndrome. Intraneural perineurioma, previously known as localized hypertrophic neuropathy, is a rare benign peripheral nerve sheath tumor arising from perineurium. This report adds a new entity in the spectrum of tumor formation in Beckwith-Wiedemann syndrome.     

Anesthetic considerations of two sisters with Beckwith-Wiedemann syndrome.

Anesthetic considerations of 21-mo-old and 4-yr-old sisters with Beckwith-Wiedemann syndrome during surgical repair of cleft palate and reduction of macroglossia are presented and discussed. This syndrome is characterized by exomphalos, macroglossia, gigantism, hypoglycemia in infancy, and many other clinical features. This syndrome is also known as exomphalos, macroglossia, and gigantism (EMG) syndrome. Principal problems associated with anesthetic management in this syndrome are hypoglycemia and macroglossia. Careful intraoperative plasma glucose monitoring is particularly important to prevent the neurologic sequelae of unrecognized hypoglycemia. It is expected that airway management would be complicated by the macroglossia, which might cause difficult bag/mask ventilation and endotracheal intubation following the induction of anesthesia and muscle paralysis, so preparations for airway difficulty (e.g., awake vocal cord inspection) should be considered before induction. A nasopharyngeal airway is useful in relieving postoperative airway obstruction.     

Beckwith-Wiedemann syndrome and its association with type III polycystic kidney disease

Two cases from our institution and another from the literature in which Beckwith-Wiedemann syndrome (BWS) and type III polycystic kidney disease (PKD) occuring simultaneously are discussed. The importance of recognizing the subtle signs of BWS is stressed, because of the increased risk of malignancies, as well as the need for continued evaluation of patients with BWS for the development and complications of PKD.     

Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature

A case of pancreatoblastoma (PB) in a 2 month-old male infant with incomplete Beckwith-Wiedemann syndrome is presented. Clinical examination disclosed left hemihypertrophy, macroglossia, bilateral exophthalmos, and enlargement of the left testis. Imaging with ultrasound and computed tomography scan showed a well-defined, heterogeneous, and grossly cystic mass arising from the head of the pancreas. Serum alpha-fetoprotein (AFP) level was elevated. The tumor was completely resected, and the histological analysis showed PB. The patient's recovery was uneventful, and AFP returned to normal values after surgery. The child has been disease-free for 5 years, and his serum AFP remained within normal values. Six other examples of this association, PB, and Beckwith-Wiedemann syndrome are recorded in the literature. The risk of developing tumor in this syndrome (complete and incomplete form) increases when hemihypertrophy is present, and the need for routine screening examination is warranted. Beckwith-Wiedemann syndrome was suggested to be a favorable biological marker for survival in children who have intraabdominal tumors.     

Assessment of obstructive apnea by using polysomnography and surgical treatment in patients with Beckwith-Wiedemann syndrome

Purpose

Obstructive apnea is sometimes seen in patients with Beckwith-Wiedemann syndrome. The cause of apnea is not limited to macroglossia, and the surgical indication for obstructive apnea has not yet been established. The authors performed polysomnography for the assessment of apnea.

Method

Overnight polysomnograms were obtained in 2 patients who developed obstructive apnea after 1-stage repair for omphalocele.

Case 1

Apnea index (AI), defined as apneic events per hour, indicated 17.3, and SpO₂ below 95% occupied 80% of the total sleep time. Computed tomography and magnetic resonance imaging indicated obstruction of the airway between macroglossia and the hypopharynx. Central tongue resection and the division of the frenulum linguae for associated ankyloglossia were performed 97 days after birth. One month after surgery, apneic events disappeared and SpO₂ below 95% occupied only 1% of the total sleep time.

Case 2

Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polysomnogram indicated a marked reduction of AI.

Conclusions

These results indicated that polysomnography was useful for evaluating obstructive apnea and that advancement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with Beckwith-Wiedemann syndrome.     

The anesthetic management of a patient with chromosome 10qter deletion syndrome

Terminal deletions of chromosome 10q are uncommon. The resulting syndrome includes cardiac and facial anomalies, urogenital abnormalities, limb defects, and mental retardation. Most affected infants require surgical correction of these anomalies. Presented are,features inherent in the syndrome that will aid the anesthesiologist in the perioperative management of such patients.     

Hypoplastic left heart syndrome: Anesthetic care prior to transplantation or surgical palliation

Hypoplastic left heart syndrome is the most common lethal cardiac defect in neonates. Options for treatment include cardiac transplantation and surgical palliation. When cardiac transplantation is chosen as the preferred option, a considerable delay may occur until a suitable donor is available. During this time, anesthetic care may be required for various surgical procedures. Associated anomalies seen in these infants and the anesthetic implications imposed by the abnormal cardiac anatomy are discussed.     

Anesthesia in Beckwith-Wiedemann syndrome

Anesthetic management of a 3-month-old boy with Beckwith-Wiedemann syndrome for bronchoscopy is reported. Management may be complicated by a difficult airway, congenital heart disease, and hypoglycemia. We did not have difficulty in airway management either with tracheal intubation or rigid bronchoscopy, but we could not extubate the baby because of tracheomalacia.     

Anesthetic management of an infant with Freeman-Sheldon syndrome

We report the anesthetic management of Freeman-Sheldon (whistling face) syndrome in a two-month-old boy scheduled for lateral canthoplasty. He had features of the syndrome including blepharophimosis, hypertelorism, a flat nose, microstomia with a limited opening, micrognathia, a very short webbed neck, scoliosis and multiple arthrogryposis. He was fed with a naso-gastric tube and suffered from several episodes of aspiration and oxygen desaturation. Difficult airway and intubation were anticipated. Anesthesia was induced via a mask with sevoflurane, although mask ventilation was difficult. Direct laryngoscopy and the insertion of a laryngeal mask airway were impossible due to microstomia with the limited opening as anticipated. A naso-tracheal intubation was achieved using a fiberoptic bronchoscope via a fiberoptic mask while ventilating the lungs. The operation and anesthesia afterwards were uneventful. In the ward, he was given supplemental oxygen but with occasional desaturation episodes. Thirteen days after the operation he was found cyanotic and resuscitation was attempted but failed. Autopsy demonstrated the hypoplasia of the lungs and thorax, atelectasis and bronchitis.     

Anterior mediastinal mass in a pregnant patient: Anesthetic management and considerations

Patients with anterior mediastinal masses are recognized to be at risk for cardiorespiratory compromise when general anesthesia is induced.^1,2 Likewise, pregnancy has a widely known constellation of potential complications that confront the anesthesiologist. The combination of both problems in a single patient presents an unusual anesthetic challenge. The following is a case report of a pregnant patient with a large, symptomatic anterior mediastinal mass who required general anesthesia for a diagnostic procedure before definitive therapy could be initiated.     

Anesthetic management of an infant girl with cloverleaf syndrome

We experienced anesthetic management of an infant girl with Cloverleaf syndrome complicated with prenatal diagnosis of craniosynostosis. She received posterior-cranioplasty and foramen magnum decompression at the age of 44 days, ventricuro-peritoneal shunting at 80 days and cranioplasty at 149 days all under general anesthesia without serious complications. In all three occasions, we induced general anesthesia with oxygen, sevoflurane, nitrous oxide and thiopental with a facemask. After we made sure it was not impossible to maintain the airway and ventilation, we performed orotracheal intubation with vecuronium. We maintained anesthesia paying particular attention to intracranial pressure and possible massive hemorrhage particularly during cranioplasty. Postoperative course was uneventful. Anesthesiologists should keep in mind that this syndrome is characterized by severe skull deformity, facial bone abnormalities, hydrocephalus and increased intracranial pressure.     

Anesthetic management of an infant with Cornelia de Lange syndrome

We report anesthetic management of an infant with Cornelia de Lange syndrome. A 12-month-old girl with Cornelia de Lange syndrome was scheduled for ureterocystoneostomy because of vesicoureteral reflux. Preoperative physical examination suggested difficult tracheal intubation. After induction of anesthesia with sevoflurane (5%) in nitrous oxide (70%) and oxygen, a laryngeal mask airway (# 1.5) was inserted. A guide wire was inserted in the trachea through a laryngeal mask airway under direct vision of a fiberoptic bronchoscope. A tube-exchanger stylet was inserted around the guide wire after the laryngeal mask airway and fiberoptic bronchoscope had been removed. An endotracheal tube (ID 4.0 mm) was easily intubated around the tube-exchanger stylet. During the surgery, anesthesia was maintained with sevoflurane (2-3%) in nitrous oxide (50%) and oxygen. There was no perioperative pulmonary complication.     

Successful airway control with the laryngeal mask in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma for central line catheterization

We present a case of an infant with severe macroglossia, hypoglycaemia and inguinal hernia associated with hepatoblastoma (Beckwith-Wiedemann syndrome) in which a laryngeal mask airway (LMA) was useful to secure the airway during central line insertion. Carbon dioxide monitoring through LMA proved effective to assess airway patency during positioning for central vein puncture. In this syndrome, where a potentially difficult airway may be encountered, LMA allowed adequate ventilation, avoiding the risk and inconvenience of tracheal intubation.