Anesthesia in a child operated for cleft lip associated with Patau&apos;s syndrome <span class="so-article-trans-title" dir="auto"> Translated title: Anestesia em criança operada para lábio leporino associado à síndrome de Patau </span>

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Abstract

Patients with Patau's syndrome (Trisomy 13) have multiple craniofacial, cardiac, neurological and renal anomalies with very less life expectancy. Among craniofacial anomalies cleft lip and palate are common. These craniofacial and cardiac anomalies present difficulties with anesthesia. We therefore describe the anesthetic management in the case of a Trisomy 13 child for operated for cleft lip at 10 months of age.

Resumo

Os pacientes com síndrome de Patau (trissomia 13) apresentam várias anomalias craniofaciais, cardíacas, neurológicas e renais, com expectativa de vida bem menor. Entre as anomalias craniofaciais, o lábio leporino e a fenda palatina são comuns. Essas anomalias craniofaciais e cardíacas apresentam dificuldades na anestesia. Portanto, descrevemos o manejo anestésico em uma criança de 10 meses de idade com Trissomia 13 submetida à cirurgia de lábio leporino.

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Most cited references 16

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Population-based analyses of mortality in trisomy 13 and trisomy 18.

Bee K. May, Quanhe Yang, Gerard C L Wong … (2003)

Although trisomy 13 and trisomy 18 are generally considered to be lethal, long-term survival of patients has been reported. We sought to evaluate mortality in people with trisomy 13 or 18 using 2 population-based strategies. In the first analysis, infants who had trisomy 13 or 18 and were born during 1968-1999 were identified using the Metropolitan Atlanta Congenital Defects Program, a population-based birth defects surveillance system. Dates of death were documented using hospital records, Georgia vital records, and the National Death Index. In the second analysis, we used the Multiple-Cause Mortality Files compiled from US death certificates from 1979 through 1997. Using these 2 analyses, we examined median survival time or median age at death, survival beyond 1 year of age, and factors associated with longer survival. Using Metropolitan Atlanta Congenital Defects Program, we identified 70 liveborn infants with trisomy 13 and 114 liveborn infants with trisomy 18. Median survival time was 7 days (95% confidence interval [CI]: 3-15) for people with trisomy 13 and 14.5 days (95% CI: 8-28) for people with trisomy 18. For each condition, 91% of infants died within the first year. Neither race nor gender affected survival for trisomy 13, but for trisomy 18, girls and infants of races other than white seemed to survive longer. The presence of a heart defect did not seem to affect survival for either condition. Using MCMF, we identified 5515 people with trisomy 13 and 8750 people with trisomy 18 listed on their death certificates. Median ages at death for people with trisomy 13 and trisomy 18 both were 10 days; 5.6% of people with trisomy 13 and 5.6% of people with trisomy 18 died at age 1 year or greater. Race and gender seemed to affect survival in both conditions, with girls and blacks showing higher median ages at death. Although survival is greatly affected by trisomy 13 and trisomy 18, 5% to 10% of people with these conditions survive beyond the first year of life. These population-based data are useful to clinicians who care for patients with these trisomies or counsel families with infants or fetuses who have a diagnosis of trisomy 13 or 18.

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Natural history of trisomy 18 and trisomy 13: I. Growth, physical assessment, medical histories, survival, and recurrence risk.

Nicholas B Blackburn, B Baty, Chelsea J. Carey (1994)

The natural history of trisomy 18 and trisomy 13 was investigated using data derived from parent questionnaires and medical records from 98 families with an index case of trisomy 18 and 32 families with an index case of trisomy 13. Data are presented on pregnancy, delivery, survival, medical complications, immunizations, growth, cause of death, cytogenetics, and recurrence risk. Half of the trisomy 18 babies were delivered by C-section. Fetal distress was a factor in half, and the only reason in a third of C-section deliveries. One minute Apgar scores were significantly lower in C-section and breech deliveries. There were more small for gestational age babies than in the general population, but most of the low birth weight newborns were small for gestational age, unlike the general population. Survival in this group of children was better than in other studies due to ascertainment bias. There were more girls than boys at all ages for both conditions, and the sex ratio decreased with time. Growth curves for length, weight, head circumference, and weight vs height are provided. Long-term survival did not appear to be due to mosaicism. We found no adverse reactions attributable to immunizations. At age 1 year there was an average of approximately 2 operations per living child. We report the second case of successful major cardiac surgery in a trisomy 18 child. Almost 70% of deaths were attributed to cardiopulmonary arrest. The sibling recurrence risk for trisomy 18 or trisomy 13 was 0.55%.

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Inpatient hospital care of children with trisomy 13 and trisomy 18 in the United States.

Katherine Nelson, Kari Hexem, Chris Feudtner (2012)

Trisomy 13 and trisomy 18 are generally considered fatal anomalies, with a majority of infants dying in the first year after birth. The inpatient hospital care that these patients receive has not been adequately described. This study characterized inpatient hospitalizations of children with trisomy 13 and trisomy 18 in the United States, including number and types of procedures performed. Retrospective repeated cross-sectional assessment of hospitalization data from the nationally representative US Kids' Inpatient Database, for the years 1997, 2000, 2003, 2006, and 2009. Included hospitalizations were of patients aged 0 to 20 years with a diagnosis of trisomy 13 or trisomy 18. The number of hospitalizations for each trisomy type ranged from 846 to 907 per year for trisomy 13 (P = .77 for temporal trend) and 1036 to 1616 per year for trisomy 18 (P < .001 for temporal trend). Over one-third (36%) of the hospitalizations were of patients older than 1 year of age. Patients underwent a total of 2765 major therapeutic procedures, including creation of esophageal sphincter (6% of hospitalizations; mean age 23 months), repair of atrial and ventricular septal defects (4%; mean age 9 months), and procedures on tendons (4%; mean age 8 years). Children with trisomy 13 and trisomy 18 receive significant inpatient hospital care. Despite the conventional understanding of these syndromes as lethal, a substantial number of children are living longer than 1 year and undergoing medical and surgical procedures as part of their treatment.

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Author and article information

Contributors

Don Varghese

Journal

Journal ID (nlm-ta): Braz J Anesthesiol

Journal ID (iso-abbrev): Braz J Anesthesiol

Title: Brazilian Journal of Anesthesiology

Publisher: Elsevier

ISSN (Print): 0104-0014

ISSN (Electronic): 2352-2291

Publication date PMC-release: 20 April 2016

Publication date Collection: Mar-Apr 2018

Publication date (Electronic): 20 April 2016

Volume: 68

Issue: 2

Pages: 197-199

Affiliations

[a ]Dr. S.N. Medical College, Department of Anesthesia, Jodhpur, Rajasthan, India

[b ]Jodhpur Dental College, Department of Oral and Maxillofacial Surgery, Jodhpur, Rajasthan, India

[c ]Raj Hospital, Jodhpur, Rajasthan, India

Author notes

[* ]Corresponding author. drdonvarghese@ 123456gmail.com

Article

Publisher Item ID: S0104-0014(16)30049-5

DOI: 10.1016/j.bjane.2016.01.002

PMC ID: 9391730

PubMed ID: 28526461

SO-VID: ecb804e8-240c-4190-9d28-8dec2dca0728

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Anesthesia in a child operated for cleft lip associated with Patau's syndrome Translated title: Anestesia em criança operada para lábio leporino associado à síndrome de Patau

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Most cited references 16

Population-based analyses of mortality in trisomy 13 and trisomy 18.

Natural history of trisomy 18 and trisomy 13: I. Growth, physical assessment, medical histories, survival, and recurrence risk.

Inpatient hospital care of children with trisomy 13 and trisomy 18 in the United States.

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