Otolaryngologic surgery in children with trisomy 18 and 13

Karimnejad, Kaveh; Costa, Dary J.

doi:10.1016/j.ijporl.2015.08.017

Cited by 15 publications

(14 citation statements)

References 14 publications

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“…Subsequently, an Otolaryngologist will often be part of the treatment team for T13 and T18 patients. 16 For this reason, it is important to understand the clinical presentations and most common procedures performed on these patients. Here, we report the largest single institution study of otolaryngologic clinical presentations and procedures in T13 and T18 patients.…”

Section: Discussionmentioning

confidence: 99%

“…The cardiac‐related survival gains mean that these patients often need support for the management of their other medical conditions, including feeding, hearing, and respiratory difficulties. Subsequently, an Otolaryngologist will often be part of the treatment team for T13 and T18 patients 16 . For this reason, it is important to understand the clinical presentations and most common procedures performed on these patients.…”

Section: Discussionmentioning

confidence: 99%

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Otolaryngologic Manifestations of Trisomy 13 and Trisomy 18 in Pediatric Patients

et al. 2022

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Objective The survival rate of patients with trisomy 13 and trisomy 18 has increased dramatically over the past two decades. We sought to comprehensively describe the otolaryngologic clinical characteristics and procedures required for these patients at our institution. Methods We performed algorithmic identification of patients with a diagnosis of trisomy 13 and trisomy 18 for whom the otolaryngology service provided inpatient or outpatient care at our institution between the dates of February 1997 and March 2021. Results Of the 47 patients studied, 18 patients had a diagnosis of trisomy 13, and 29 had a diagnosis of trisomy 18. Complete trisomy was present in 44% (8/18) of trisomy 13 patients and 55% (16/29) of trisomy 18 patients. 81% of patients were living at the time of the study. About 94% (44/47) of patients required consultation with another specialty in addition to Otolaryngology. Overall, the most common diagnoses among this cohort were gastroesophageal reflux disease (47%), dysphagia (40%), otitis media (38%), and obstructive sleep apnea (34%). Nearly three‐quarters (74%) of patients studied required an otolaryngologic procedure. The most common surgical procedure was tonsillectomy and/or adenoidectomy. Patients with trisomy 18 were significantly more likely to have external auditory canal stenosis and obstructive sleep apnea whereas patients with trisomy 13 were more likely to have cleft lip and palate. Conclusions Patients with a diagnosis of trisomy 13 or 18 often require multidisciplinary management and the range of required care spans the breadth of otolaryngology. Level of Evidence 4 Laryngoscope, 133:1501–1506, 2023

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Otolaryngologic Manifestations of Trisomy 13 and Trisomy 18 in Pediatric Patients

et al. 2022

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“…One of the more common types of procedures performed in infants with trisomy 18 are otolaryngologic surgeries. Of all the surgeries in trisomy 18 patients, 17% are otolaryngologic, including tympanostomy placement (25%), cleft lip repair (17%), tracheostomy (16.5%), tonsillectomy/adenoidectomy (16%), and cleft palate repair (13%) (Karimnejad & Costa, 2015). While most surgeries are performed early in life, consistent evaluation of otolaryngologic complications should be assessed over the life course.…”

Section: Otolaryngologicmentioning

confidence: 99%

Surveillance guidelines for children with trisomy 18

Kepple

Fishler

Peeples

2021

American J of Med Genetics Pt A

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Trisomy 18 is the second most common aneuploidy syndromes in live born infants. It is associated with high mortality rates, estimated to be 75%–95% in the first year of life, as well as significant morbidity in survivors. The low survival is largely due to the high prevalence of severe congenital anomalies in infants with this diagnosis. However, interventions to repair or palliate those life‐threatening anomalies are being performed at a higher rate for these infants, resulting in increased rates of survival beyond the first year of life. While it is well documented that trisomy 18 is associated with several cardiac malformations, these patients also have respiratory, neurological, neoplastic, genitourinary, abdominal, otolaryngologic, and orthopedic complications that can impact their quality of life. The goal of this review is to present a comprehensive description of complications in children with trisomy 18 to aid in the development of monitoring and treatment guidelines for the increasing number of providers who will be caring for these patients throughout their lives. Where the evidence is available, this review presents screening recommendations to allow for more rapid detection and documentation of these complications.

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“…Karimnejad and Costa examined a trisomy database for otolaryngologic surgeries from 1988 to 2014. Of the 231 otolaryngologic surgeries performed; 57 (25%) were tympanostomy tube placement, 40 (17%) were cleft lip repair, and 38 (16.5%) were tracheostomy placement (Karimnejad & Costa, 2015). Further studies will be needed to examine the long‐term otolaryngologic needs of these patients.…”

Section: Otolaryngologymentioning

confidence: 99%

Interdisciplinary care of children with trisomy 13 and 18

Weaver

Anderson

Beck

et al. 2020

American J of Med Genetics Pt A

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Children with trisomy 13 and 18 (previously deemed “incompatible with life”) are living longer, warranting a comprehensive overview of their unique comorbidities and complex care needs. This Review Article provides a summation of the recent literature, informed by the study team's Interdisciplinary Trisomy Translational Program consisting of representatives from: cardiology, cardiothoracic surgery, neonatology, otolaryngology, intensive care, neurology, social work, chaplaincy, nursing, and palliative care. Medical interventions are discussed in the context of decisional‐paradigms and whole‐family considerations. The communication format, educational endeavors, and lessons learned from the study team's interdisciplinary care processes are shared with recognition of the potential for replication and implementation in other care settings.

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Otolaryngologic surgery in children with trisomy 18 and 13

Cited by 15 publications

References 14 publications

Otolaryngologic Manifestations of Trisomy 13 and Trisomy 18 in Pediatric Patients

Otolaryngologic Manifestations of Trisomy 13 and Trisomy 18 in Pediatric Patients

Surveillance guidelines for children with trisomy 18

Interdisciplinary care of children with trisomy 13 and 18

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