Cases reported "Pierre Robin Syndrome"

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1/62. Mandibular distraction osteogenesis in a neonate.

    Children with craniofacial anomalies are predisposed to airway obstruction and frequently require airway intervention. tracheotomy is performed when the airway obstruction is severe and refractory to other less invasive interventions. tracheotomy is associated with significant morbidity, and there is a trend noted in the literature toward achieving earlier decannulation by the institution of definitive structural changes to the mandible. Mandibular distraction osteogenesis has been shown to alleviate airway obstruction in the pediatric population. We report a case in which mandibular distraction osteogenesis was successfully carried out in a neonate with acute airway obstruction at birth as a result of combined Pierre Robin sequence and klippel-feil syndrome. After 1 year, the patient still had an adequate airway with tolerable scarring and no neurologic sequelae.
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2/62. Familial association of camptodactyly, mental retardation, whistling face and Pierre Robin sequence.

    Two sibs are reported with severe developmental retardation combined with the clinical triad of camptodactyly, whistling face and Pierre Robin sequence as clinical signs of fetal hypokinesia. In spite of tracheotomy, the first child of the family died 10 hours after birth. A sister of this child was born 1 year later. During pregnancy prenatal diagnosis of hydrocephaly was made by ultrasonographic examination. MRI scan showed holoprosencephaly. At 15 months of age psychomotor development is severely impaired, birth and length are delayed.
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3/62. Epignathus teratoma: report of three cases with a review of the literature.

    Three cases of epignathus teratoma associated with other midline anomalies are reported. The first case involved Pierre Robin sequence and a bifid tongue. The second case was characterized by two teratomas, a meningoencephalocele, and a cleft lip and nose. The third case had Pierre Robin sequence associated with duplication of the pituitary gland and hypoplasia of the corpus callosum.
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4/62. Beyond Pierre Robin sequence.

    The label Pierre Robin sequence is given to infants presenting with a triad of specific congenital anomalies: micrognathia, glossoptosis, and cleft palate. However, this label should be considered the first, not the final, step in the diagnostic process. In approximately 80 percent of newborns with Pierre Robin sequence, the triad of anomalies is part of an underlying genetic condition. This article reviews the variable etiologies of and general clinical considerations for Pierre Robin sequence. To illustrate how clinical management might vary based on the identification of an underlying condition, three case examples of neonates with Pierre Robin sequence and different underlying genetic conditions are presented.
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ranking = 8
keywords = sequence
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5/62. Orthodontic treatment for a patient with Pierre-Robin sequence complicated by juvenile periodontitis.

    OBJECTIVE: To arrest the advancement of periodontitis. PATIENT: A 17-year-old boy diagnosed with Pierre-Robin sequence at birth exhibited localized juvenile periodontitis. Severe bone loss and mobile teeth were localized in the incisors and molars, which were irregularly positioned, possibly associated with a residual scar from palatoplasty for a soft palate cleft at an early age. CONCLUSION: Combined orthodontic-periodontic-prosthodontic treatment arrested the advancement of the periodontitis and established a stable occlusion.
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ranking = 5
keywords = sequence
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6/62. Late presentation of upper airway obstruction in Pierre Robin sequence.

    A retrospective review was carried out of 11 consecutive patients with the Pierre Robin sequence referred to a tertiary paediatric referral centre over a five year period from 1993 to 1998. Ten patients were diagnosed with significant upper airway obstruction; seven of these presented late at between 24 and 51 days of age. failure to thrive occurred in six of these seven infants at the time of presentation, and was a strong indicator of the severity of upper airway obstruction. growth normalised on treatment of the upper airway obstruction with nasopharyngeal tube placement. All children had been reviewed by either an experienced general paediatrician or a neonatologist in the first week of life, suggesting that clinical signs alone are insufficient to alert the physician to the degree of upper airway obstruction or that obstruction developed gradually after discharge home. The use of polysomnography greatly improved the diagnostic accuracy in assessing the severity of upper airway obstruction and monitoring the response to treatment. This report highlights the prevalence of late presentation of upper airway obstruction in the Pierre Robin sequence and emphasises the need for close prospective respiratory monitoring in this condition. Objective measures such as polysomnography should be used, as clinical signs alone may be an inadequate guide to the degree of upper airway obstruction.
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keywords = sequence
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7/62. Congenital microgastria with Pierre Robin sequence and partial trismus.

    A female with congenital microgastria, Pierre Robin sequence and partial trismus is described. This is a previously undescribed association and the etiology of the association is discussed.
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8/62. Subperiosteal release of the floor of the mouth musculature in two cases of Pierre Robin sequence.

    Many management approaches have been considered to relieve upper respiratory obstruction in patients with Pierre Robin sequence, but the choice of treatment is determined by the severity of the obstruction. These options include prone positioning, the use of a nasal trumpet, and surgery. One surgical technique is the subperiosteal release of the floor of the mouth musculature. The theory behind this procedure is that this musculature is under tension, and therefore it pushes the tongue upward and backward, resulting in respiratory obstruction. In theory, the release of this musculature from the mandible should alleviate the tension and hence clear the obstruction. In an attempt to objectively evaluate this theory, we performed subperiosteal release surgery on two infants. Our first patient required an emergent tracheostomy on postoperative day 2 because of the onset of surgically induced airway edema. To avoid this complication in the second patient, we performed a tracheostomy at the same time as surgery. Pre- and postoperative magnetic resonance imaging in the second patient revealed only a minimal change in the anatomy of the floor of the mouth musculature. We believe the subperiosteal release of the floor of the mouth musculature requires further evaluation before it can be considered to be effective in the surgical treatment of respiratory obstruction in Pierre Robin sequence.
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ranking = 6
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9/62. Oesophageal perforation presenting as oesophageal atresia in a premature neonate following difficult intubation.

    Iatrogenic oesophageal perforation in neonates is well recognized in the medical and surgical literature with intubation injury listed as a possible contributing mechanism besides nasogastric tube placement and suctioning. Diagnosis can be difficult and sometimes confused with other entities. With early diagnosis, nonsurgical management often leads to complete resolution in neonates. We report the case of a 1-day-old premature neonate who was brought to the operating room with the preliminary diagnosis of proximal oesophageal atresia with stump perforation and distal tracheo-esophageal fistula. His intubation for respiratory distress at birth had been difficult due to Pierre-Robin sequence with micrognathia. Oesophagoscopy in the operating room revealed a patent oesophagus but perforations in the pharynx and in the proximal oesophagus with the nasogastric tube entering the pharyngeal perforation. Oesophageal perforation and the limitations of the difficult airway algorithm in small neonates are discussed.
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10/62. cleft palate in a patient with Williams' syndrome.

    cleft lip or palate has not been reported in the medical literature as a part of Williams' syndrome. We present a patient who had cleft palate among other congenital manifestations. This patient's immediate postnatal period clinically seemed to have a Pierre Robin sequence. With the development of the craniofacial complex, microgenia and micrognathia with glossoptosis gradually became apparent. On further assessment, the patient showed other clinical findings that suggested a syndromic association. This required a complete evaluation to discard other conditions that present with low psychomotor development and distinctive facies, such as Kabuki syndrome or fetal alcohol syndrome. The diagnosis for Williams' syndrome was established based on the clinical features and supported by the fluorescent in situ hybridization test. Williams' syndrome has been described as a rare, congenital disorder characterized by physical and developmental problems. Common features include characteristic "elfin-like" facies, supravalvular aortic stenosis, hypercalcemia, low birth weight, slow weight gain, feeding problems, impulsive and outgoing personality, limited spatial skills and motor control, and intellectual disability. Although individuals with Williams' syndrome may show competence in areas such as language, music, and interpersonal relations, their IQs are usually low and they are considered moderately to mildly retarded.
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