SINDROME DE CRI DU CHAT PDF

Cri-du-Chat syndrome: conservative dental treatment in an 8-year old child. The aim of this article was to report the case of an 8-year old patient with Cri-du-Chat syndrome CdCS referred to the pediatric dental clinic for urgent dental treatment. The chief complaints were a traumatic injury to both permanent maxillary central incisors and difficulty performing oral hygiene. The patient was extremely cooperative during clinical evaluation, demonstrating ability to withstand conservative treatment options. Dental examination revealed accentuated overjet, carious lesions, and very poor oral hygiene. Clinical and radiographic evaluations were necessary to diagnose and determine treatment strategies for the traumatic injuries, carious lesions, and gingivitis, which were implemented over a total of five dental visits.

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Cri-du-Chat syndrome: conservative dental treatment in an 8-year old child. The aim of this article was to report the case of an 8-year old patient with Cri-du-Chat syndrome CdCS referred to the pediatric dental clinic for urgent dental treatment.

The chief complaints were a traumatic injury to both permanent maxillary central incisors and difficulty performing oral hygiene. The patient was extremely cooperative during clinical evaluation, demonstrating ability to withstand conservative treatment options. Dental examination revealed accentuated overjet, carious lesions, and very poor oral hygiene. Clinical and radiographic evaluations were necessary to diagnose and determine treatment strategies for the traumatic injuries, carious lesions, and gingivitis, which were implemented over a total of five dental visits.

Outcomes of the treatment strategies adopted are described. This case report illustrates the variety of clinical findings that pediatric dentists may encounter in the oral cavity and face of patients with CdCS and the problems that these alterations may cause. When faced with a CdCS patient, dentists should try to provide the best treatment possible and prioritize the use of conservative techniques.

The condition was first described by Lejeune et al. Cri du Chat in French means "cat-like cry" and refers to the characteristic cry of affected children at birth. Diagnosis can be made immediately after birth, based on the peculiar cry, probably caused by abnormal development of the larynx or a variety of neurological disorders 4.

Studies have reported incidence rates ranging from , to , live births 1,5 , but the prevalence found by Niebuhr4 in 6, individuals with mental retardation was approximately Furthermore, data reported by Higurashi's et al. Mainardi et al. Clinical evaluation of those patients revealed higher degree severity for microcephaly, dysmorphism, and psychomotor retardation related to the size of deletion of chromosome 5, i.

The main characteristics of CdCS are a high-pitched cat-like cry, distinct facial dysmorphism, microcephaly, severe psychomotor and mental retardation, as well as abnormal facial features 1 , e. Cardiac malformations, neurological and renal abnormalities, preauricular tags, syndactyly, hypospadias, and cryptorchidism can also be found 1,2,4. Reported orofacial manifestations include mandibular microretrognathia, high palate, variable malocclusion, enamel hypoplasia, poor oral hygiene, generalized chronic periodontitis, and delayed tooth eruption 2,4,7.

The main characteristics found were skeletal class II malocclusion caused by mandibular retrognathism, dental biprotrusion, and a small upper airway.

Cornish and Pigram 9 assessed 27 children with CdCS and demonstrated that, with early special education and an atmosphere of family support, some affected children reached psychomotor and social levels compatible with those of normal children. That study demonstrated the potential of children with this syndrome to develop and maintain important skills, including the ability to communicate needs, to socially interact with others, and to develop some degree of mobility.

These findings underscore the need for these patients to receive a multidisciplinary treatment. Whenever general anesthesia is needed, it should be carefully accomplished using all necessary measures, as this procedure increases the risk of complications. In addition, intubation may be hindered by retrognathia and deep palate.

When possible, dental treatment should be carried out under local anesthesia, and sedatives should be used if necessary to improve cooperation and avoid injuries as much as possible In view of the scarce literature describing dental conditions and dental treatments in CdCS patients, the aim of this article was to report the case of a CdCS female patient who received conservative dental treatment under local anesthesia, as well as describe the peculiarities related to this syndrome.

The chief complaints were a traumatic injury to both permanent upper central incisors and difficulty performing oral hygiene. The patient's mother was 32 years old when she became pregnant. She received medical follow-up throughout pregnancy, and syphilis was diagnosed at 39 weeks' gestation, after which delivery was immediately undertaken. The mother did not report the presence of congenital anomalies in other family members. During the clinical interview, the mother informed that the girl had CdCS and that she had been under medical supervision since birth.

It was not possible to obtain the father's medical history. At the moment of the interview, the child was not under any systemic drug therapy and did not present any involvement of vital organs.

Notwithstanding, she showed some degree of mental retardation, chronic anemia, worms, and flu. The mother also informed that the patient consumed mainly pasty foods due to mastication problems.

The patient did not present congenital heart alterations. She was extremely cooperative during the interview and clinical evaluation, demonstrating ability to withstand conservative treatment options under local anesthesia if necessary. During the first clinical evaluation, some characteristics were recorded: facial asymmetry, low-set ears, preauricular tags, eyes slightly separated, epicanthal folds, down-turned corners of mouth, convex facial profile with mandibular microretrognathia, difficulty opening the mouth, especially on the left side, hypofunction of perioral muscles, cheeks and tongue, and absence of lip seal Figure 1a-b.

Dental examination revealed vestibular projection of upper central incisors, which suffered lateral and extrusive luxation, carious lesions affecting all posterior teeth on the left side, due to the difficulty opening the mouth, and very poor oral hygiene. Other structures showed normal characteristics for an 8-year old child.

Clinical and radiographic evaluations were necessary to diagnose and determine treatment strategies for the traumatic injuries, carious lesions, and gingivitis Figure 1c-d. At the first periapical radiograph, no sign of resorption or apical lesion was diagnosed, indicating the monitoring of trauma.

Incomplete root formation was also observed in both teeth. Still during the first visit, the child and her mother received instructions on how to perform effective oral hygiene as a strategy to reduce gingivitis. After 4 days, the patient showed better gingival condition, and a flexible splint was made using nylon floss and composite resin and anchored to the primary molar to allow periodontal healing of both traumatized incisors Figure 2a-b.

Three weeks later, in the first periapical control radiograph, an initial process of external root resorption in both traumatized teeth was observed. In this case, as the patient seemed able to undergo local anesthesia, pulpectomy was the treatment proposed. The mother and patient readily agreed with the suggestion, and pulpectomy was initiated using the tell-show-do technique, under local anesthesia. At first, a calcium hydroxide-based intracanal medicament Calen, S.

White, Rio de Janeiro, RJ, Brazil was introduced into the root canals to stimulate apexification of the immature teeth The patient was extremely collaborative throughout the procedure, demonstrating ability to undergo more conservative treatments without the need for general anesthesia. None of the endodontic procedures was performed under rubber dam isolation, due to patient's difficulty in opening her mouth but extreme collaboration.

All carious lesions affected posterior teeth located on the left side, probably due to difficulties cleaning that side as a result of limited mouth opening on that side. They all involved up to the inner half of dentin, with no pulp involvement. The patient and her mother did not report spontaneous pain. After 3 months, the patient presented good oral hygiene and absence of periodontal disease.

The flexible splint was removed, and the first primary molars were extracted as part of a serial extraction plan to allow the correct eruption of permanent teeth. The girl is now 10 years old and has lost all her primary teeth. She returns for regular maintenance visits to monitor the traumatized teeth and oral hygiene conditions Figure 3. Even being considered a rare genetic autosomal disorder, not common in dental practice, CdCS presents orofacial manifestations that require specific and specialized dental treatment.

Consequently, it is important that health professionals have some understanding of the developmental and behavioral characteristics of children with this condition. The present case report describes orofacial characteristics that are in accordance with previous descriptions found in the literature The increased risk for caries and periodontal disease in these patients can be associated with the orofacial manifestations of the syndrome and poor oral hygiene resulting from difficulties opening the mouth 2.

Anterior open-bite and accentuated overjet, common craniofacial characteristics of the syndrome, may have contributed to the traumatic injuries observed in our patient 8, Carious lesions were treated conservatively by partial caries removal 12,13 and restored using a modified glass-ionomer cement after pulp capping with calcium hydroxide.

This technique involves minimal intervention, reducing the risk of pulp exposure and technical complications 13 , advantages that are extremely important for this special patient. Flexible splinting and apexification were used to treat the traumatic injuries of permanent incisors. This procedure enabled periodontal healing and apexification of the roots, enhancing endodontic treatment conditions.

The calcium hydroxide apexification technique, which was part of the conservative treatment proposed for this child, is well established in the literature, with proven biocompatibility and high success rates stimulating apexification of immature permanent teeth Due to the patient's difficulty opening the mouth, the whole treatment was adapted to the patient's oral condition, and thus performed without rubber dam isolation.

During partial caries removal 12,13 , no local anesthesia was used, and the patient did not report any discomfort. Another treatment option in this situation syndromic patients would be the extraction of teeth with difficult access to treatment pulpectomy in one session for traumatized teeth, under general anesthesia. According to Ohtawa et al. Considering that other dental clinics had previously refused to provide dental treatment to the patient because of the diagnosis of CdCS, the fact that the patient presented the syndrome was not an impediment for clinical dental treatment.

In this case, after full explanation of the treatment plan to the mother and child using the tell-show-do technique, the patient fully collaborated with all procedures, allowing the conservative treatments proposed. This paper illustrates the variety of clinical findings that pediatric dentists may encounter in the oral cavity and face of patients with CdCS and the problems that these alterations may cause.

It also shows that, even though patients with CdCS may present some difficulties because of their condition, a well-prepared team of dentists should be able to treat them. In sum, when faced with a CdCS patient, dentists should try to provide the best treatment possible according to patient cooperation and prioritize the use of conservative techniques with local anesthesia when necessary.

Mainardi PC. Cri du Chat syndrome. Orphanet J Rare Dis. Assessment of orofacial characteristics and oral pathology associated with Cri-du-Chat syndrome.

Oral Dis. Niebuhr E. The Cri du Chat syndrome: epidemiology, cytogenetics, and clinical features. Hum Genet. Live birth prevalence and follow-up of malformation syndromes in 27, newborns. Brain Dev. Clinical and molecular characterisation of 80 patients with 5p deletion: genotype-phenotype correlation. J Med Genet. Scully C, Davison MF.

Orofacial manifestations in the Cri du Chat syndrome 5p-. J Dent. Craniofacial characteristics in cri-du-chat syndrome. Cornish KM, Pigram J. Developmental and behavioural characteristics of Cri du Chat syndrome. Arch Dis Child. Cri du Chat syndrome: a case report.

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Cri-du-chat syndrome

The Cri du Chat syndrome CdCS is a genetic disease resulting from a deletion of variable size occurring on the short arm of chromosome 5 5p-. The incidence ranges from , to , live-born infants. The main clinical features are a high-pitched monochromatic cry, microcephaly, broad nasal bridge, epicanthal folds, micrognathia, abnormal dermatoglyphics, and severe psychomotor and mental retardation. Malformations, although not very frequent, may be present: cardiac, neurological and renal abnormalities, preauricular tags, syndactyly, hypospadias, and cryptorchidism. Molecular cytogenetic analysis has allowed a cytogenetic and phenotypic map of 5p to be defined, even if results from the studies reported up to now are not completely in agreement. Genotype-phenotype correlation studies showed a clinical and cytogenetic variability. The identification of phenotypic subsets associated with a specific size and type of deletion is of diagnostic and prognostic relevance.

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Cri du chat syndrome

Cri-du-chat cat's cry syndrome, also known as 5p- 5p minus syndrome, is a chromosomal condition that results when a piece of chromosome 5 is missing. Infants with this condition often have a high-pitched cry that sounds like that of a cat. The disorder is characterized by intellectual disability and delayed development, small head size microcephaly , low birth weight, and weak muscle tone hypotonia in infancy. Affected individuals also have distinctive facial features, including widely set eyes hypertelorism , low-set ears, a small jaw, and a rounded face.

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