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 Table of Contents  
Year : 2017  |  Volume : 9  |  Issue : 2  |  Page : 77-81

Dental management of bilirubin encephalopathy patient: A case report and review

Department of Pedodontics, College of Dental Sciences, Davangere, Karnataka, India

Date of Web Publication26-Jul-2017

Correspondence Address:
Neena Eregowda
Department of Pedodontics, College of Dental Sciences, Davangere, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jorr.jorr_2_17

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Patients with special health care needs often present with compromised oral health for which they need dental treatment. Such patients and often their guardians find it difficult to maintain oral health due to various reasons such as decreased manual dexterity and preference for soft diet. Bilirubin encephalopathy is a condition occurring as a result of neonatal jaundice/hyperbilirubinemia. Elevated levels of unconjugated bilirubin at birth often leads to brain damage manifesting as auditory and motor defects such as athetoid cerebral palsy. This paper reports one such case of dental management of a known case of bilirubin encephalopathy.

Keywords: Athetoid, bilirubin encephalopathy, kernicterus, passive stabilization

How to cite this article:
Parameswarappa P, Sharma R, Korishettar R, Eregowda N. Dental management of bilirubin encephalopathy patient: A case report and review. J Oral Res Rev 2017;9:77-81

How to cite this URL:
Parameswarappa P, Sharma R, Korishettar R, Eregowda N. Dental management of bilirubin encephalopathy patient: A case report and review. J Oral Res Rev [serial online] 2017 [cited 2023 May 30];9:77-81. Available from: https://www.jorr.org/text.asp?2017/9/2/77/211633

  Introduction Top

Special and medically compromised patients constitute a unique group of the population that challenge the dentist's skill and knowledge. People with disabilities often need extra efforts from the dental team to establish good oral health.[1] One such condition rarely encountered and reported in dentistry is chronic bilirubin encephalopathy.

The classic form of chronic bilirubin encephalopathy is also called kernicterus, a pathological term indicating the yellow staining of the deep nuclei of the brain (kern-related to basal ganglia; icterus-yellow staining). The terms acute and chronic bilirubin encephalopathy are used to describe the clinical symptoms associated with the neuropathology.[2] Kernicterus or chronic bilirubin encephalopathy is a very rare complication of neonatal unconjugated hyperbilirubinaemia. In this, high levels of bilirubin cause brain damage leading to neurological symptoms such as hearing loss and cerebral palsy and can prove to be fatal in some cases.

It is also associated with green discoloration of teeth. Bilirubin is extensively deposited in the body, and during hyperbilirubinemia, although it disappears from soft tissues after remission, it permanently gets trapped in hard tissues.[3]

Chronic bilirubin encephalopathy is a clinical tetrad[4] comprising (1) a movement disorder consisting of athetosis and dystonia, may also include spasticity and hypotonia, (2) auditory dysfunction involving deafness or hearing loss and auditory neuropathy or auditory dys-synchrony, (3) oculomotor impairments especially impairment of upgaze, lateral gaze impairments including strabismus may also be present, and (4) dental enamel hypoplasia of the deciduous teeth. The neurological findings are attributed to the neuropathological lesions in (1) basal ganglia, specifically the globus pallidus, subthalamic nucleus, cerebellum and brainstem nuclei involved with truncal tone and posture, (2) auditory brainstem nuclei and the auditory nerve, and (3) brainstem oculomotor nuclei.

The present case report is written to state dental treatment of one such diagnosed case of bilirubin encephalopathy since the data on the dental management of such patients are sparsely reported.

  Case Report Top

A 22-year-old male patient reported to the dental hospital with a complaint of pain in lower left back tooth region for 2 days. He was a known case of bilirubin encephalopathy with a history of neonatal jaundice. Although 22-year-old, the patient lacked the cooperation ability and thus was difficult to manage; he was finally referred to the Department of Pediatric Dentistry.

The patient had thin hypotonic lower extremities and was carried to the clinic on his brother's shoulder. He was very happy and smiling but was unable to talk. He also had a difficulty looking upwards. Involuntary jerky movements were observed with his jaw, hands, and legs and he was unable to maintain a constant head position with continuous side to side movements. He could listen and understand all simple commands and tried to follow the same but was unable to maintain an open mouth position due to involuntary movements. He had underdeveloped speech and could pronounce bilabial sounds only with difficulty.

Mother gave a history of epileptic attack while he was a child, accompanied with high fever but the same ceased to occur, and the antiepileptic drugs were terminated 2 years back, and no recent attack history was reported. The patient had developed few simple motor skills such as closing his shirt button, eating his own food, and waving out to people.

The patient's chief complaint as communicated by the mother was pain in lower left back tooth region of the patient since 2 days. Pain aggravated on chewing food and on drinking cold water. Past dental history indicated multiple extractions at the age of 8 years due to multiple extensive carious milk teeth and lower left posterior tooth. On further history, it was concluded that milk teeth had an altered morphology and discolored. The permanent dentition, however, did not show any signs of discoloration.

On further evaluation, deep caries with tenderness on percussion was found in relation to lower left second permanent molar. Occlusal enamel caries were present with upper right and left maxillary first permanent molars. Furthermore, calculus and stains with spontaneous gingival bleeding were present in relation to lower anterior teeth. After clinical and radiographic examination, a diagnosis of acute irreversible pulpitis was formulated in relation to lower left second molar.

Thus, oral prophylaxis, amalgam restorations for maxillary molars and root canal treatment in relation to lower left second molar was planned in multiple appointments since the patient was unable to maintain position in the dental chair.

Owing to the jerky jaw movements, macroglossia and inability to maintain a stable position on the dental chair, dental treatment under general anesthesia (GA) was planned. On medical evaluation, however, he was found to have increased degree of scoliosis along with decreased chest volume. On knowing the complications, parents were not willing for taking the patient under GA, and thus outpatient treatment plan in multiple appointments was finalized for him. There was no contraindication for local anesthesia.

Parental consent was obtained, and the triangular sheet was used to stabilize him on a dental chair [Figure 1]. Along with that a rolled towel was used under his neck as head support. Both upper extremities were stabilized with the chair armrest. Head was also stabilized using active stabilization by the assistant and tongue movement was restricted with mouth mirror. Upper limbs were juxtaposed and maintained in the same position. A flossed mouth prop was used for all procedures to maintain open mouth. The patient showed hyperactive gag reflex thus high volume suction was used with intermittent rest breaks for the patient during procedures.
Figure 1: Twenty-two-year-old bilirubin encephalopathy patient being stabilized using triangular sheet in the dental chair

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Behavior management was done using communication, modeling and tell show do techniques. After gaining his confidence, oral prophylaxis was done. Restorations were successfully placed in relation to upper right first and left first permanent molars using composite and amalgam. Following appointment, single visit root canal treatment was done in relation to lower left second molar [Figure 2]. The patient is currently under follow-up.
Figure 2: An intraoral periapical radiograph of 46 showing root canal treatment done

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The patient was happy on all his dental appointments and made his maximum efforts for cooperation except the involuntary jerky movements of his jaws and body. Physical restraints and active stabilizations were used to manage the same and provide a noncompromised dental service.

  Discussion Top

Bilirubin encephalopathy is a complication of neonatal unconjugated hyperbilirubinaemia. In this condition, elevated level of bilirubin leads to brain damage and often manifests with neurological symptoms such as hearing loss and cerebral palsy. The risk of neuronal injury by bilirubin is determined by the concentration of unbound or “free” unconjugated bilirubin and hydrogen ion in blood. Unconjugated bilirubin tends to enter brain tissue when the blood's bilirubin-binding capacity is exceeded, or when other displacing substances, such as sulfonamides, compete for bilirubin-binding sites on albumin. Few more important risk factors for kernicterus include neuronal susceptibility, gestational age, infection or sepsis, and hemolysis, especially Rh isoimmunization.

Various mechanisms have been hypothesized regarding the effect of bilirubin Necrosis is one of the mechanisms of brain cell injury. There is good evidence from various studies that bilirubin causes apoptosis. Another possible mechanism is that bilirubin interferes with intracellular calcium homeostasis through different pathways, such as altering function and expression of calcium/calmodulin kinase II.[5],[6] Another possibility is that it sensitizes the cell to other injuries, triggering apoptosis. Bilirubin may also kill cells by causing neuronal hyperexcitability perhaps via excitatory amino acid neurotoxicity, or it may have other membrane or neurotransmitter effects. Finally, it may act by interfering with mitochondrial respiration and energy production.

Shapiro in 2010 proposed a classification for Kernicterus based upon location [Table 1].[2]
Table 1: Classification of kernicterus

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The incidence of kernicterus in the USA is estimated to be 1 in 40,000 births, with about 1 in 650–1000 neonates born >35 weeks' postmenstrual age experiencing transient hyperbilirubinemia (>25 mg/dL).[7]

The symptom of bilirubin encephalopathy which poses maximum difficulty in providing quality dental treatment is the presence of various motor impairment. Classic motor symptoms caused by bilirubin encephalopathy are identified as the athetotic or dyskinetic form of cerebral palsy and are related to lesions in the globus pallidus and subthalamic nuclei most prominently and sometimes in the cerebellum and brainstem.[8]

According to Mutch et al.,[9] cerebral palsy can be called an umbrella term covering a group of nonprogressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of development. There are different types of cerebral palsy depending on signs, but bilirubin encephalopathy is most commonly associated with dyskinetic or athetoid type of palsy.

According to the European classification of motor impairment in cerebral palsy, dyskinetic type is dominated by an abnormal pattern of posture or movement and involuntary, uncontrolled, recurring, and occasionally stereotyped movements.[10]

When treating this patient following difficulties were encountered

  1. Difficulty of communication especially with auditory or visual or speech deficit
  2. Low intelligence
  3. Poor concentration with slightest of things distracting attention
  4. Uncontrolled involuntary jerky movements
  5. Posture: Since athetoid type, he needed more manual support and control in the chair
  6. Ability to cooperate: Compromised due to physical condition.

To minimize the effects of this medical condition on quality of treatment provided, both the operatory as well as dental personnel should be well equipped and prepared.

The choice of patient or guardian should be given prime importance in all aspects of treatment. Consideration should be given whether the patient wishes to be treated in dental chair or wheelchair itself. In the current case, however, the patient was always carried to the operatory by the brother on his shoulders and hence was treated in the dental chair.

The assistive stabilization and postural maintenance can be achieved through the following techniques:[11]

  1. Head position maintained in the midline by one of the dental staff over a head support (position device) located at the occipital level
  2. Maintenance of bent and juxtaposed upper members in the midline, with the help of Velcro straps
  3. Maintenance of bent lower members decreasing the hip angle to 120° in relation to the trunk using soft foam[12],[13],[14] rolls as positioning devices, such as for support under the knees
  4. Maintenance of an open mouth with the use of mouth props
  5. GA and conscious sedation can be considered in these patients to reduce the number of visits and provide a quality care but obtaining a thorough consent from patient's physician is of utmost priority.

A recent rise in the incidence of kernicterus, after decades of improvement, may beattributable to relaxed standards for initiation of phototherapy, or due to earlier infantdischarge from the hospital. Thus, there is a greater chance now for the dentists to encounter patients suffering from the same kind of condition, especially in developing countries. The current case provides basic information and treatment techniques for managing such patients.

  Conclusion Top

Compassion and patience on the dentist's side can help satisfy guardian and the patient with special heath care needs. Bilirubin encephalopathy is one such condition which is certainly on the rise in developing countries. Pediatric dentists are best suited to treat such patients with basic and advanced behavior management techniques. Basic knowledge about this disorder is thus necessary for all clinicians.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

National Institute of Dental and Craniofacial Research. Practical Oral Care for People with Cerebral Palsy. NIH Publication No. 09-5192. Bethesda, MD: National Institute of Dental and Craniofacial Research; 2009.  Back to cited text no. 1
Shapiro SM. Chronic bilirubin encephalopathy: Diagnosis and outcome. Semin Fetal Neonatal Med 2010;15:157-63.  Back to cited text no. 2
Watanabe K, Shibata T, Kurosawa T, Morisaki I, Kinehara M, Igarashi S, et al. Bilirubin pigmentation of human teeth caused by hyperbilirubinemia. J Oral Pathol Med 1999;28:128-30.  Back to cited text no. 3
Shapiro SM. Definition of the clinical spectrum of kernicterus and bilirubin-induced neurologic dysfunction (BIND). J Perinatol 2005;25:54-9.  Back to cited text no. 4
Churn SB, DeLorenzo RJ, Shapiro SM. Bilirubin induces a calcium-dependent inhibition of multifunctional Ca2+/calmodulin-dependent kinase II activity in vitro. Pediatr Res 1995;38:949-54.  Back to cited text no. 5
Conlee JW, Shapiro SM, Churn SB. Expression of the alpha and beta subunits of Ca2+/calmodulin kinase II in the cerebellum of jaundiced Gunn rats during development: A quantitative light microscopic analysis. Acta Neuropathol 2000;99:393-401.  Back to cited text no. 6
Bhutani VK, Johnson L. Kernicterus in the 21st century: Frequently asked questions. J Perinatol 2009;29 Suppl 1:S20-4.  Back to cited text no. 7
Rose J, Vassar R. Movement disorders due to bilirubin toxicity. Semin Fetal Neonatal Med 2015;20:20-5.  Back to cited text no. 8
Mutch L, Alberman E, Hagberg B, Kodama K, Perat MV. Cerebral palsy epidemiology: Where are we now and where are we going? Dev Med Child Neurol 1992;34:547-51.  Back to cited text no. 9
Cans C. Surveillance of cerebral palsy in Europe: A collaboration of cerebral palsy surveys and registers. Dev Med Child Neurol 2000;42:816-24.  Back to cited text no. 10
Santos MT, Manzano FS. Assistive stabilization based on the neurodevelopmental treatment approach for dental care in individuals with cerebral palsy. Quintessence Int 2007;38:681-7.  Back to cited text no. 11
Stevenson DK, Maisels MJ, Watchko JF. Care of the Jaundiced Neonate. New York: McGraw-Hill Medical; 2012.  Back to cited text no. 12
Johnson LH, Bhutani VK, Brown AK. System-based approach to management of neonatal jaundice and prevention of kernicterus. J Pediatr 2002;140:396-403.  Back to cited text no. 13
Przekop A, Sanger TD. Birth-related syndromes of athetosis and kernicterus. Handb Clin Neurol 2011;100:387-95.  Back to cited text no. 14


  [Figure 1], [Figure 2]

  [Table 1]


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