Year : 2008 | Volume
: 20 | Issue : 4 | Page : 166--169
Wolff-Parkinson-White syndrome with cleft lip and palate: A rare, previously unreported association
K Kannan, Rajendra Patil, Suresh Kumar
Department of Oral Medicine and Radiology, Narayana Dental College, Chintareddy Palem, Nellore, Andhra Pradesh, India
Department of Oral Medicine and Radiology, Narayana Dental College, Chintareddy Palem, Nellore, Andhra Pradesh
Wolff-Parkinson-White syndrome, also called Pre Excitation Syndrome, is characterized by an extra pathway that conducts the electrical impulses from the atria to the ventricles without the normal delay. We are reporting a case of WPW syndrome with a cleft lip and palate, which is a rare association and previously unreported in literature.
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Kannan K, Patil R, Kumar S. Wolff-Parkinson-White syndrome with cleft lip and palate: A rare, previously unreported association.J Indian Acad Oral Med Radiol 2008;20:166-169
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Kannan K, Patil R, Kumar S. Wolff-Parkinson-White syndrome with cleft lip and palate: A rare, previously unreported association. J Indian Acad Oral Med Radiol [serial online] 2008 [cited 2022 Aug 17 ];20:166-169
Available from: https://www.jiaomr.in/text.asp?2008/20/4/166/52823
Wolff-Parkinson-White syndrome (WPW syndrome) is also called Pre Excitation Syndrome. This syndrome was first described by Wolff, Parkinson, and White in 1930. They described it as having a distinct ECG pattern in apparently healthy young people with short bursts of tachycardia. This syndrome is characterized by an extra pathway that conducts the electrical impulses from the atria to the ventricles without the normal delay.  The symptoms of WPW syndrome include palpitations, tightness in the chest, dyspnea, and a drop in blood pressure leading to light headedness, dizziness, and fainting spells.  The incidence of WPW syndrome is between 0.9 and 3% of the general population.  While the vast majority of individuals with an extra pathway remain asymptomatic throughout their lives, there is a risk of sudden death associated with the syndrome. Sudden death due to WPW syndrome is rare (incidence of less than 0.6%), and is due to the effect of the accessory pathway of tachyarrhythmias in these individuals. We are reporting a case of WPW syndrome with a cleft lip and palate, which is a rare association and previously unreported in literature
A 9-year-old boy reported to the department of Oral Medicine and Radiology with a chief complaint of irregular teeth in the lower front region of the jaw since childhood [Figure 1]. His medical history revealed a repair of the cleft lip and cleft palate at the age of 9 months and 4 years, respectively and a history of epileptic seizures since the age of 3 years old, for which he was given treatment with Gardenal 30 mg/ day (Phenobarbital). He also had a history of dysarthria for the past 5 years, for which he is undergoing speech therapy since the past 3 years. In the past 1 month, the patient experienced a burning sensation during micturition. The family medical history revealed that his parents had a consanguineous marriage. On general examination, the patient had an unsteady gait and appeared emaciated. An examination of systems revealed shortness of breath on mild exertion and palpitations.
An extra oral examination revealed facial asymmetry, Dolico Cephaly, underdeveloped mandible, ptosis of the left eye, increased lacrimation from the left eye, increased intercanthal distance, a depressed nasal bridge, incompetent lips, an everted lower lip, and a surgical scar over the upper lip.
An intra oral hard tissue examination revealed over retained deciduous anterior teeth, generalized attrition, cleft of palate in pre maxillary region, and a narrow and high arched palate. A soft tissue examination revealed pallor of oral mucosa, depapillation of the tongue, bifid uvula, and a surgical scar over the soft palate [Figure 2] and [Figure 3].
Based on the medical history and clinical examination, this case was provisionally diagnosed as Velo-Cardio-Facial syndrome. However, Crouzon syndrome and Hemifacial microsomia were considered in a differential diagnosis.
Radiological investigations were performed. An ortho pantomography showed hypoplasia of the mandible on the left side, hyper pneumatization of the maxillary sinus on the right side and a cleft palate [Figure 4]. Steiner's analysis of lateral cephalography revealed deficient maxilla and Class III skeletal growth [Figure 5]. The patient was referred to a cardiologist for further evaluation of cardiac status and to rule out any congenital cardiac malformations. An echocardiogram and electrocardiogram (ECG) were performed. The echocardiogram revealed a normal echo study, however, the ECG showed short PR interval, a wide QRS complex, and a slurred onset producing a delta wave in the early part of the QRS complex [Figure 6]. Based on the clinical findings and investigations, the patient was diagnosed with partial expression of Velo-Cardio-Facial syndrome overlapped with Wolff-Parkinson-White syndrome, which was diagnosed by a cardiologist at Narayana Medical College and Super Specialty hospital in Nellore.
Although cleft lip and palate can occur as a distinct malformation in the general population, they are also known to be associated with syndromes like Pierre Robin syndrome, Apert syndrome, Crouzon syndrome, Treacher Collins syndrome, Hemifacial microsomia, Velo-Cardio-Facial syndrome (Shprintzen syndrome), and Nager syndrome (Preaxial acro facial dysostosis). Among these syndromes, cardiac anomalies are present in hemifacial microsomia and Velo-Cardio-Facial syndrome. 
Hemifacial microsomia is primarily a syndrome of the first branchial arch involving underdevelopment of the temporomandibular joint, mandibular ramus, mastication muscles, and the ear. It usually manifests as facial asymmetry, cleft lip and palate, and cardiac malformations. 
Velo-Cardio-Facial Syndrome is also known as 22q11.2 deletion syndrome, DiGeorge Syndrome, Conotruncal anomaly face syndrome, Congenital Thymic Aplasia, and Strong Syndrome.  This syndrome is associated with genetic deletions found on the long arm of the 22 nd chromosome. Some patients with similar clinical features may have deletions on the short arm of chromosome 10. DiGeorge syndrome causes migration defects of neural crest-derived tissues, particularly affecting the development of the third and fourth Branchial pouches. Also affected is the thymus gland (largely responsible for differentiation and induction of T-cells). Impaired immune function may result from dysfunction of the thymus. The associated congenital anomalies are Tetralogy of Fallot, interrupted aortic arch, Ventricular Septal defects, palatal abnormalities (69%), seizures, learning difficulties (70 to 90%), renal anomalies (37%), hypocalcaemia (50%), hearing loss, and skeletal abnormalities. ,
Normally heart rhythm begins at the Sino Atrial (SA) node, which acts as a pace maker. When the SA node is activated, the electrical impulses will be transmitted to the AV node through the anterior, middle, and posterior internodal tract and will finally be conducted to the bundle of Kent. This is divided into the right and left bundle branches. These bundle branches further divide into Purkinje fibers. These fibers end at the myocardium. These electrical impulse cause ventricular contraction that sustains normal cardiac function. 
WPW syndrome is characterized by the transmission of electrical impulses from the SA node to the ventricle through extra nodal pathways without the normal nodal delay that allows for ventricular filling and causes excitation of ventricles prior to the normal pathway (hence, the term Preexcitation syndrome) and causes supra ventricular tachycardia. This extra nodal pathway of conduction of impulses increases the heart rate to greater than 200 beats per minute. In some patients, the ECG abnormality may be present without any symptoms, such as tachycardia. However, the ECG may show clear evidence of underlying pathology but the patients themselves may not have any symptoms like tachycardia. 
The syndrome presents with episodes of tachycardia (usually faster than 200 beats per minute), palpitations, dyspnea, and a decrease in blood pressure, anxiety, and symptoms of tightness in the chest sometimes given as a racing feeling in the chest, light headedness, dizziness, and fainting spells. The complications associated with WPW syndrome are fatal owing to the increased risk of ventricular arrhythmias due to the extremely fast conduction across the bypass tract and the development of atrial flutter or fibrillation, heart failure, and sudden death. 
Therefore, this syndrome needs to be diagnosed early with detailed meticulous medical history, clinical observation, echocardiogram, electrocardiogram, and stress testing. The management part includes careful observation and if tachycardia occurs, vagal maneuvers may be the first choice of treatment. If the patient does not respond to vagal maneuvers, anti-arrhythmic medication such as Adenosine 6 mg but not exceeding 12 mg should be given, Propranolol 1-3 mg not exceeding 1 gm should be given intravenously, or Verapamil 80-160 mg should be given three times a day.
If the patient does not respond to anti-arrhythmic medication, an electric cardio version or cardiac ablation should be considered and intensive cardiac care should be life saving for many such patients with WPW syndrome. 
There is need to be aware that congenital heart diseases like Septal defect, Valvular defects, and even conduction defects like those seen in WPW syndrome may occur and should be suspected in patients with congenital anomalies in the maxillofacial region. Prompt recognition and taking adequate and appropriate precautions can go a long way towards preventions of any morbidity in these patients.
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