The child was included in the Baby Clinic of Araçatuba Dental School program for preventive dental care at 11 months of age, and this clinical case report is being presented when the child is 4 years old.
The child's mother reported that her child had a PBS during the anamnesis procedure, and he had been undergoing regular medical check-ups since birth because of this finding. The pregnancy was planned by the parents, and the mother received prenatal care. An ultrasound examination performed during the fifth week of gestation showed a gastrointestinal malformation of the fetus.
A subsequent morphologic ultrasound examination revealed the following findings: gastrointestinal abnormality, enlarged kidneys, and a low amount of amniotic liquid. However, although the parents were aware that their child would be born with health problems during the gestation period, they did not receive conclusive information regarding the baby's condition.
The baby boy was born prematurely at 7 months by caesarian, with a weight of 1450 kg and height of 41 cm. According to the mother, postnatal examination revealed that the baby had a distended and wrinkled abdomen, resembling a dried prune. The form of the penis was abnormal, with an enlarged diameter and lax excess skin. Despite these characteristics, an immediate diagnosis was not made.
Numerous medical examinations were performed during this period, and the results combined with the clinical features resulted in a diagnosis of PBS. The child does not have relatives with any type of syndrome.
These features included abdominal musculature deficiency, gastrointestinal and urinary abnormalities, a larger-than-normal bladder, malformed kidneys (only 1 with normal function), and cryptorchidism (bilateral intra-abdominal testes).
Although only 1 kidney was found to function normally on medical examination, the mother stated that the child did not have problems associated with this and denied impairment of renal function. The mother reported episodes of urinary infection requiring antibiotic treatment that were related to excess skin on the penis in the first to third years of life.
Moreover, the mother reported that the child did not have cardiac or pulmonary disease, and no anal alteration was found. The alterations mentioned above may result from the syndrome.
The characteristics of the inferior and superior limbs, hands, feet, fingers, and toes were normal. Structural abnormalities were only noted in both of the child's knees, which is a feature consistent with this syndrome, according to the mother.
The child underwent plastic surgery of the abdomen and penis and a surgery to position the testes correctly at 2 years of age. The present state of the child's abdomen is shown in Figure 1.
The following oral health status findings were observed at the initial clinical examination and in all follow-up appointments: no dental caries, low plaque indices, no supragingival calculus deposits, and no other periodontal problems. The following dental anomalies were clinically and radiographically noted when the primary dentition was complete: a geminated mandibular right central incisor with 1 root and 1 canal; the absence of a mandibular permanent left incisor (Figure 2 and 3); and congenitally missing primary teeth, namely, the mandibular right and left lateral incisors (Figure 2 and 3). In addition, agenesis of the mandibular left lateral permanent incisor germ was noted on radiography. All dental anomalies in the primary and permanent dentition were confirmed by panoramic radiography (Figure 4).
Intraoral view of the mandibular primary double right central incisor and congenitally absence of both right and left lateral primary incisors.
Periapical radiograph displaying a geminated mandibular right central primary incisor with one root and one root canal and absence of a mandibular permanent left incisor.
The child's family received preventive and educational dental guidance and the child is seen by a dentist 4 times a year since his placement in the Baby Clinic of Araçatuba Dental School program. The parents have maintained strict oral health care of the child, which is demonstrated by the aspects cited above regarding the child's oral health.
The monitoring system of the program and the parent's access to appointments whenever necessary, i.e., not only in cases of dental emergency, allows the dental team to register any manifestation of oral diseases. Oral soft tissue manifestations arising from disorders caused by the syndrome[15–19] have not been noted up to this point.
Although metabolic disorders can affect mineralized structures, an alteration of dental enamel was not found in the primary dentition. However, it will be necessary to check for the presence of enamel hypoplasia in permanent teeth. A radiographic exam did not reveal bone problems.
While the etiology of PBS is unknown, the literature related to this disorder describes it as a mesodermal defect consisting essentially of genitourinary tract abnormalities.[2,3,6] Nevertheless, the wide range of severities and the previously reported alterations of others organs,[7,11,13] including the association with some syndromes,[2,8,9] provide evidence that ectoderm tissues are affected by the syndrome.[11,13] This is the only report of a child with PBS associated with dental anomalies in literature. The dental anomalies found in this case report justify the necessity of an extra-abdominal assessment, especially an intra-oral evaluation. The importance of this anomaly tends to be underestimated because of the low prevalence of geminated teeth. However, anomalies in primary teeth can significantly affect the permanent successors teeth.[20,21] Several studies have shown that geminated primary teeth have an influence on permanent successors teeth, resulting in hypodontia (missing teeth), supernumerary teeth, repeated geminated teeth, and peg-shaped teeth.[20–22] In cases of geminated primary teeth involving the mandibular lateral incisors and canines, hypodontia of permanent successors is most common.[20,21,23] Careful monitoring is required, and orthodontic management should be considered part of a treatment plan to ensure functional occlusion and to advance esthetics.
BMC Oral Health. 2012;12(56) © 2012 BioMed Central, Ltd.