Year : 2007 | Volume
: 25 | Issue : 3 | Page : 140--143
Dental considerations in the management of children suffering from sickle cell disease: A case report
Department of Pedodontics & Preventive Dentistry, Sharad Pawar Dental College & Hospital, Wardha, Maharashtra, India
Department of Pedodontics and Preventive Dentistry, Sharad Pawar Dental College and Hospital, Sawangi (Meghe), Wardha - 442 004, Maharashatra
Sickle cell disease (SCD) is a genetically derived disorder characterized by the presence of an abnormal hemoglobin molecule, designated as hemoglobin S (HbS). It is one of the most common genetic disorder worldwide, with an estimated 70 million people carrying the sickle gene. This article describes the dental considerations in managing a 6-year-old child suffering from SCD (SS type).
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Ramakrishna Y. Dental considerations in the management of children suffering from sickle cell disease: A case report.J Indian Soc Pedod Prev Dent 2007;25:140-143
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Ramakrishna Y. Dental considerations in the management of children suffering from sickle cell disease: A case report. J Indian Soc Pedod Prev Dent [serial online] 2007 [cited 2020 Feb 26 ];25:140-143
Available from: http://www.jisppd.com/text.asp?2007/25/3/140/36566
Sickle cell disease (SCD) is a genetically derived disorder characterized by the presence of an abnormal hemoglobin molecule, designated as hemoglobin S (HbS). When present in significant amounts and in specific circumstances, HbS causes erythrocytes to distort into the so-called "sickle cells." The term sickle cell disease is most accurately reserved for the homozygous state SS that causes sickle cell anemia. This disease was originally recognized and described by Herrick  in 1910. The disease state is characterized by two abnormal alleles that is manifested by chronic hemolytic anemia, recurrent attacks of pain, frequent bacterial infections, gradual deterioration of tissue and organ function, and shortened life expectancy. ,, It is one of the most common genetic disorder worldwide, with an estimated 70 million people carrying the sickle gene.  The gene is present in 10% of African-Americans and in upto 25% of the population in Western Africa. 
A 6-year-old girl child reported to the Department of Pedodontics with a complaint of pain and swelling in lower-left back region of the jaw and enlarged right and left cervical lymph nodes since 4 days. Medical history revealed that she is a known patient of SCD (SS type) and had undergone blood transfusion 10 days ago because of severe sickle cell anemia. Patient was hospitalized for the treatment of upper respiratory tract infection with bronchospasm 15 days ago. Medical reports of the patient revealed the following:
a) Hb% - 4.2g/dl
b) Peripheral smear - Many sickled RBCs
c) Sickling type - "SS" type
Hb electrophoresis revealed "Hemoglobin S"Soft enlarged liver on palpationCardiovascular system and central nervous system were within normal limitsUltrasonography of abdomen confirmed sickle cell hepatopathyBone marrow cytology showed increased erythropoietic activity
At present, the condition is under control because of blood transfusion and chemotherapy. On examination, the patient was conscious, cooperative with normal gait and was well-oriented. On extra oral examination, there was a diffuse swelling of soft to firm in consistency on the left side of the face, extending from left corner of mouth to the pinna of the ear. Bilateral submandibular, submental and bilateral upper cervical lymph nodes [Figure 1] were palpable. Cervical nodes were tender and firm in consistency. Intraoral examination revealed deep carious lesions in 74, 84 and hypoplastic lesions in 54, 64. There was a swelling in relation to 74. Intraoral periapical radiographs of 74 region [Figure 2] showed periapical and periradicular radiolucency, [Figure 3] 84 region showed horizontal trabeculation creating a "ladder-like effect," which is specific for SCD patients. The horizontal trabeculation is not appreciable in the orthopantomogram [Figure 4] as in periapical radiographs. The lateral skull radiograph [Figure 5] revealed thickened diploe; the trabeculae are coarse and are running perpendicular to the inner and outer table giving a characteristic appearance of "hair-on-end pattern".
Since the condition of the patient was stable, it was decided to go for the endodontic therapy of 74 and 84 under medical supervision. Pulpotomy of 84, multivisit pulpectomy of 74 and restoration of 54, 64 was carried out using all aseptic techniques and under antibiotic coverage. All necessary precautions were taken during treatment procedure in order to avoid the initiation of sickle cell crisis.
Patient was referred to the medical department for the opinion regarding the upper cervical lymph nodes. Fine needle aspiration cytology of the cervical nodes revealed tubercular lymphadenitis and the patient was put on an antitubercular drug regime. During regular follow-ups, the neck swelling gradually reduced in size and presently the condition is stable only to recur again.
The basic abnormality in SCD resides in the globin portion of the hemoglobin molecule. The exact aminoacid sequence of each chain was established by Pauling et al . 1949  In SCD, a single base change in the DNA results in a substitution of valine for glutamic acid at the sixth position in the beta globin chain. The ensuing pathologic manifestations are due to the altered physical properties of the red cell with hemoblogin S. Under deoxygenated conditions, the HbS undergoes polymerization within the red cells, leading to distortion of shape, membrane changes, cellular dehydration and decreased deformability (sickle cells). Microvascular occlusion results from the altered behavior of sickled red cells and produces progressive autoinfarction.  Organs that are commonly affected by recurrent episodes of vasoocclusion are the joints of the extremities, brain, kidneys, bones including the mandible, lungs, retina and skin - particularly the lower extremities. ,,
Certain circumstances such as lowering of pH, increase in the core temperature and tonicity may contribute to or enhance sickling by modifying the oxygen dissociation curve of hemoglobin.  The actual sickling process is usually reversible in 10-15 s on the reoxygenation of the intracellular hemoglobin. Red cell survival is reduced in SCD and to a lesser extent in the sickle cell trait disease. The mean life span of the red cell in SCD is approximately 17 days and approximately 29 days in sickle cell trait.  Sickled RBCs are the cause of end-organ pathology in SCD.
The SCD is characterized by four major problems:
Painful vasoocclusive crisisSevere hemolytic anemiaPredisposition to bacterial infectionChronic hyperbilirubinemia ,,
In general, the clinical manifestations of the disease are first noted between 6 months and 3 years of age. The reason for the delay in the appearance of symptoms is due to the protective effect of the fetal hemoglobin (HbF).  At birth, 50-95% of the hemoglobin is HbF. It gradually declines 2-4% each week post-partum and is replaced by HbS. The protective effect of the HbF is lost and symptoms of the disease then develop. In the sickle cell trait, only 20-45% of the hemoglobin is HbS and the rest is normal hemoglobin. Moreover, only one of the β chains is thought to be abnormal, where both β chains are abnormal in SCD.
Microvascular occlusions are thought to be responsible for most of the clinical manifestations of SCD. They may involve most of the organ systems of the body and are frequently characterized by pain as well as by various signs and symptoms of organ dysfunction. The most characteristic manifestations of SCD is the sickle cell crisis. It consists of an episode of severe pain of 1-2 weeks duration, often accompanied by a low-grade fever and leukocytosis.  A crisis may be precipitated by infection, dehydration or acidosis or may be associated with any identified etiology.
The various sickling disorders and its clinical features are as follows: [Table 1]
The hematocrit and the total number of sickled cells on a peripheral smear usually remain unchanged through out the crisis. The crisis typically subsides spontaneously, only to recur after a few weeks or months. Narcotic analgesics are often required to control pain, making drug addiction common. Cardiopulmonary, renal, bone and extremity, central nervous system and ophthalmic complications are the result of repeated microinfarcts. Chronic hemolysis present in sickle cell anemia also predisposes to cholelithiasis. This occurs most frequently in the second and third decades of life and is usually accompanied by elevated levels of conjugated bilirubin in the range of 1-4 g/dl. ,,,,,
Hematological manifestations are characterized by anemia with hemoglobin values of 5-9 g/dl. Red blood cell survival time is markedly decreased. Normoblastic hyperplasia in the marrow, aids in the maintenance of constant hemoglobin levels. Reticulocyte count usually ranges from 5-25%. The white blood cell count is generally in the range of 10,000-20,000 cells/mm 3 with the platelet count being normal to slightly elevated. Hemoglobin electrophoresis with the separation of HbS, a positive HbS solubility test and a positive sickle preparation will accompany the preceding hematological findings. Even if the course is mild or severe, most of the patients with SCD and the more severe related hemoglobinopathies will eventually die of their disease or its sequelae. Some will die in childhood of overwhelming bacterial infections due to the loss of splenic function.
There is no treatment for SCD other than symptomatic treatment. Antibiotics should be used early in the treatment of infection and analgesic drugs should be used if necessary but with caution to prevent iatrogenic addiction. Transfusions are avoided unless the patient has an aplastic crisis with a extremely low hemoglobin level because the transfusion effects are transitory and the patients tend to develop antibodies, making it difficult to find suitable donors for future transfusions.
Folic acid dietary supplements are being used to treat an aplastic crisis. Cytotoxic drugs such as hydroxyurea have shown to increase hemoglobin F and reduce the frequency of painful episodes. The increase in HbF tends to ameliorate the intracellular polymerization of HbS. , Gene therapy via allogenic stem cell transplantation is being studied as a possible curative option for severely affected young patients. 
The initial evaluation of the patient with a sickle hemoglobinopathy must include a thorough history and physical examination. In most instances, the patients with classic SCD are aware of their diagnosis and have experienced multiple hospitalizations for painful crises, episodes of severe anemia, multiple blood transfusions and recurrent bouts of bacterial pulmonary infections.
The oral mucosa may be pale or jaundiced, and there may be a susceptibility to dental infections. Patients often show delayed eruption and hypoplasia of the dentition secondary to their general underdevelopment. Hard tissue changes are conspicuous: hypercementosis may develop, and bone marrow hyperplasia with apparent osteoporosis of the jaw, increased radiolucency resulting from the decreased number of trabeculae observed on the dental radiographs. This change is noted particulary in the alveolar bone between the roots of the teeth, where trabeculae may appear as horizontal rows, creating a ladder like effect. In the skull films, diploe is thickened and the trabeculae are coarse and run perpendicular giving a radiographic appearance of hair-on-end pattern.
Preventive dental care is important to lessen the need for radical treatment requiring general anesthesia (GA). GA is hazardous in sickle cell patients because of severe anemia and a crisis may be precipitated. Routine conservative treatment should be carried out under local or relative analgesia. Pulpal symptoms are common in the absence of any obvious dental disease. Acute infections should be treated immediately since they may precipitate a sickling crisis. Patients are more prone to developing osteomyelitis because of hypovascularity of the bone marrow secondary to thromboses. Surgical procedures should be carried out under antibiotic coverage. Wherever possible, the dental treatment should be carried out under local anesthesia.
Sickle cell disease is still a major public health problem and a challenge to those who encounter it. Preventive dental care is important to lessen the need for radical treatment. Elective dental procedures should not be performed in patients with poorly controlled disease unless absolutely necessary because of increased risk of chronic anemia and delayed wound healing. The elimination of the oral sources of infection and treatment of acute infections should be instituted immediately since the infection can precipitate a sickle cell crisis.
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