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\begin{document}

\title{Collaboration of perioperative management in an adult patient with 22 q
11.2 deletion syndrome: A case report}



\author[1]{Mikiko Ito}%
\author[2]{Tatsuya Tokura}%
\author[3]{Tomoya Miyauchi}%
\author[4]{Aiji Sato (Boku)}%
\author[2]{Hiroyuki Kimura}%
\author[5]{Hayami Tsuchihashi}%
\author[6]{Yoshiko Katayama}%
\affil[1]{School of Dentistry, Aichi-Gakuin University}%
\affil[2]{Nagoya University Graduate School of Medicine}%
\affil[3]{Kachi Memorial Hospital}%
\affil[4]{Aichi Gakuin University School of Dentistry}%
\affil[5]{School of Dentistry, Aichi-Gakuin  University}%
\affil[6]{ School of Dentistry, Aichi-Gakuin  University}%


\vspace{-1em}



  
  \date{May 27, 2024}


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\selectlanguage{english}
\begin{abstract}
22 q 11.2 deletion syndrome is a genetic disease associated with a high
rate of various psychiatric disorders in addition to multiple physical
diseases. This case report suggested that a multidisciplinary team
approach is important for perioperative management of patient with 22 q
11.2 deletion syndrome.%
\end{abstract}%



\sloppy


\textbf{Title:}

Collaboration of perioperative management in an adult patient with 22 q
11.2 deletion syndrome: A case report

\textbf{Authors:}

Mikiko Ito D.D.S., Ph.D.\textsuperscript{1} , Tatsuya Tokura M.D.,
Ph.D.\textsuperscript{2}, Tomoya Miyauchi M.D.,
Ph.D.\textsuperscript{3}, Aiji Sato (Boku) D.D.S., Ph.D.
\textsuperscript{4}, Hiroyuki Kimura M.D., Ph.D.\textsuperscript{2} ,
Hayami Tsuchihashi D.D.S.\textsuperscript{1}, Yoshiko Katayama D.D.S.
\textsuperscript{1}

\textbf{Institutional affiliations and address for each author:}

1 Department of Oral and Maxillofacial Surgery, School of Dentistry,
Aichi-Gakuin

University, 2-11 Suemori-dori, Chikusa, Nagoya, Aichi 464-8651, Japan

2 Department of Psychiatry, Nagoya University Graduate School of
Medicine, 65 Tsurumai, Showa, Nagoya, Aichi 466-8550, Japan

3 Department of Psychiatry, Kachi Memorial Hospital, 456 Fujimi,
Minamiooshimizu, Toyohashi, Aichi 441-8132, Japan

4 Department of Anesthesiology, School of Dentistry, Aichi-Gakuin
University, 2-11 Suemori-dori, Chikusa, Nagoya, Aichi 464-8651, Japan

\textbf{Corresponding author:}

Tatsuya Tokura M.D., Ph.D.

Department of Psychiatry, Nagoya University Graduate School of
Medicine\textbf{,} 65 Tsurumai, Showa, Nagoya, Aichi 466-8550, Japan

Tel: +81 52 7442282; Fax: +81 52 7442293; E-mail:
tatsuyatokura@gmail.com

\textbf{Key clinical message:}

A multidisciplinary team approach is important for perioperative
management of adult patient with 22 q 11.2 deletion syndrome.

\textbf{Abstract:}

22 q 11.2 deletion syndrome is a genetic disease associated with a high
rate of various psychiatric disorders in addition to multiple physical
diseases. This case report suggested that a multidisciplinary team
approach is important for perioperative management of patient with 22 q
11.2 deletion syndrome.

\textbf{Keywords:}

22 q 11.2 deletion syndrome, multidisciplinary team approach,
perioperative management.

\textbf{Introduction}

22 q 11.2 deletion syndrome (22 q 11.2 DS) is a genetic disease that is
often complicated by psychiatric disorders such as intellectual
disability, autism spectrum disorder, attention-deficit/hyperactivity
disorder, and schizophrenia in addition to physical diseases such as
congenital heart disease, palate hypoplasia, and immunodeficiency. This
syndrome is said to be the most common of the chromosomal microdeletion
syndromes, and a recent Danish cohort study reported that 1 in 3672
people developed it\textsuperscript{1}. Although the prognosis has been
said to be poor, the adult population with 22 q 11.2 DS is increasing
due to advances in pediatric care\textsuperscript{2}.

Acceptable standard care for individuals with 22 q 11.2 DS is a careful
coordination and multidisciplinary team approach that provides access to
medical services throughout their life cycle\textsuperscript{3}. In this
report, we successfully collaborated in the perioperative management of
oral surgery in an adult 22 q 11.2 DS patient with multiple physical
diseases and schizophrenia.

\textbf{Case Report}

The patient was a 24-year-old woman. Anal atresia and cleft palate were
found at birth, and the patient took a genetic test and was diagnosed
with 22 q 11.2 DS. At one month after birth, the patient was diagnosed
as having aortic stenosis due to bicuspid aortic valve in the pediatric
cardiology department. The patient was placed under observation, and
general exercise restrictions were not necessary, but sports club
activities were prohibited.  The patient underwent palatoplasty at the
age of 1 year and 6 months, and orthodontic treatment from the age of 9
to 20 years with a diagnosis of dental stenosis. At the age of 20, the
patient got married and gave birth.

At the age of 22, the patient was diagnosed with schizophrenia due to
the patient's hallucination and delusion and was admitted to a
psychiatric hospital for 10 months. The symptoms have improved by
taking antipsychotic drug and modified electroconvulsive therapy.
After being discharged from the hospital, the patient noticed
toothache and was referred to our hospital by another dental clinic.

The patient was 148 cm tall, weighed 41 kg, and had a blood pressure of
110/70 mmHg at the time of medical examination. There were no other
problems with blood tests or electrocardiograms.

Extraoral findings of the patient showed microtia, micromandibula,
micronostrils, micrognathia and trismus. The intraoral findings showed
that 15, 17, 28, 34, 42, 43, 45, 46, 48 (F\selectlanguage{ngerman}édération Dentaire
Internationale: FDI) were in a residual root state. The patient's oral
hygiene status was extremely poor. Panoramic radiographs showed the
apices of 11, 45, 46 (FDI) with round radiolucent images suggesting
apical lesions (Figure). \selectlanguage{english}We planned to extract 10 teeth, specifically
(11, 15, 17, 28, 34, 42, 43, 45, 46, 48 (FDI) ). \selectlanguage{english}The risk of the
procedure itself was low, but the risks arising from complications of 22
q 11.2 DS were a concern.

The severity of aortic stenosis was unknown because the patient
interrupted the periodic medical examination by self-judgement at the
age of 20. \selectlanguage{english}We contacted the pediatric cardiologist and obtained the
general anesthesia records of this patient's maternal delivery by
cesarean section three years ago.

\selectlanguage{english}Echocardiography at the department of cardiology of our hospital showed
a pressure gradient between the left ventricle and the aorta of 20 mmHg.
The severity of aortic stenosis was judged to be mild. \selectlanguage{english}Although there
were no problems with cardiac function, the patient was at risk for
infective endocarditis, and 2 g of ampicillin was prepared.

The dental anesthesiologist diagnosed the patient as having difficult
intubation due to micrognathia and severe trismus and judged that
conscious fiberscope-guided intubation under mild sedation was also
impossible because the patient's psychiatric symptoms were unstable.
After induction of general anesthesia, the dental anesthesiologist
administered 30 mg of rocuronium, a muscle relaxant, and performed
nasotracheal intubation under fiber scope guidance. We explained the
risks of anesthesia to the patient and the patient's family in advance
and obtained their consent in writing.

The following risks information were provided by the attending
psychiatrist: 1) discontinuation of psychotropic drugs may aggravate
schizophrenia; 2) examination by an oral surgeon may increase anxiety
and irritation; and 3) interactions between antipsychotics and
epinephrine contained in the local anesthetics may occur. Therefore, the
administration of psychotropic drugs was continued even on the night
before the operation, the preoperative examination by the oral surgeon
was shortened, and \selectlanguage{english}the local anesthetic for surgery was prepared as
ferripressin-containing propitocaine hydrochloride without epinephrine.

The above risks were shared by a multidisciplinary team including oral
surgeons, dental anesthesiologists, psychiatrists, cardiologists, nurses
and social workers. We extracted 10 teeth as scheduled. The day after
the operation, we transferred the patient to the psychiatric hospital
where the patient had been hospitalized. One week later, the patient had
good progress and we completed the collaboration of perioperative
management.

\textbf{Discussion}

The course of this patient provides the following important clinical
suggestions. Since the symptoms and severity of 22 q 11.2 DS vary widely
among individuals, multidisciplinary medical collaboration is necessary
for perioperative management.

Frequent comorbidities of 22 q 11.2 DS are multidisciplinary, including
cardiac anomalies (49-83\%), palatal anomalies (69-100\%), dental
skeletal anomalies (40-50\%), hypocalcemia (17-60\%), schizophrenia
(6-30\%) and attention-deficit/hyperactivity disorder
(25\%)\textsuperscript{4}.

Life-threatening complications include congenital heart disease and the
most common one is tetralogy of Fallot (20-45\%)\textsuperscript{5}.
Congenital heart disease adds to the complexity associated with acquired
cardiovascular problems and multiorgan comorbidities, and requires
perioperative management that considers immune dysfunction,
thrombocytopenia and hypocalcemia\textsuperscript{5}. This patient had
no tetralogy of Fallot but aortic stenosis. Aortic stenosis is a rare
complication of 22 q 11.2 DS\textsuperscript{5}, but as it is a risk
group for infective endocarditis. Therefore, antibiotic ampicillin was
administered prophylactically. Because the severity of aortic stenosis
was unknown, the risk for general anesthesia was also unknown. Through
the medical cooperation system, general anesthesia records of the recent
cesarean section and modified electroconvulsive therapy were received
from the pediatric cardiologist and the psychiatrist.

\selectlanguage{english} In generally, physicians have trouble dealing with patients with
psychiatric disorders, while psychiatrists have trouble dealing with
patients with physical illness. Perioperative management of oral surgery
for patients with schizophrenia requires a history of psychiatric
treatment, assessment of psychotic symptoms and consideration of the
duration of psychotropic drug withdrawal to control schizophrenic
symptoms\textsuperscript{6,7}. This case was also coordinated to be
transferred to the psychiatric hospital where the psychiatrist worked
the day after the operation in accordance with the risk information
obtained from the psychiatrist.

In 22 q 11.2 DS, a high rate of palatal hypoplasia is observed, and it
is necessary to devise measures for difficult intubation. We decided
that it would be difficult to intubate this patient due to micrognathia
and trismus. The patient had experienced general anesthesia in the past.
At that time oral intubation by laryngeal mask was performed, because
the operative field was not the oral cavity. We finally chose
nasofiberscope-guided intubation after induction of general anesthesia.
In patients with 22 q 11.2 DS, general anesthesia or intravenous
sedation has been used for dental treatment\textsuperscript{8}. However,
there have been no previous reports of fiberscope-guided nasal
intubation after induction of general anesthesia. Our team responded
flexibly to risks with our own ideas, using precedents as a guide.

\selectlanguage{english}We experienced an adult patient with 22 q 11.2 DS in whom a
multidisciplinary team approach was effective in collaboration of
perioperative management. In the future, it is expected that the number
of adult patients with 22 q 11.2 DS who require oral surgery under
general anesthesia with multiple risks will increase as in this case.
Since the symptoms and severity of 22 q 11.2 DS vary widely among
individuals, it is suggested that accumulation of individual
perioperative management reports may be clinically useful in the future.

\textbf{Conclusion}

We experienced an adult patient with 22 q 11.2 DS who needed to extract
teeth. Through our experience with this case, we conclued that a
multidisciplinary team approach is important for perioperative
management of adult patient with 22 q 11.2 DS.

\textbf{Acknowlegements:}

This case report was supported by a governmental grant in aid for young
scientists (No. 19K17108) from the Japan Society for the Promotion of
Science.

\textbf{Conflict of interest:}

There were no conflicts of interest related to this publication.

\textbf{Author contributions} :

All authors contributed to the case. Preoperative management was
performed by YK, MI and HT. Anesthetic planning and management were
performed by AS. The first draft of the manuscript was written by MI, TM
and HK. And all authors commented on previous versions of the
manuscript. TT supervised the manuscript. All authors read and approved
the final manscript.

\textbf{Consent for publication:}

Written informed consent was obtained from the patient and the patient's
family for this case report.

\textbf{ORCID:}

Mikiko Ito: 0000-0002-4618-3688

Tatsuya Tokura (corresponding author): 0000-0002-2293-2349

\textbf{References:}

\begin{enumerate}
\tightlist
\item
  Olsen L, Sparso T, Weinsheimer SM, et al. Prevalence of rearrangements
  in the 22q11.2 region and population-based risk of neuropsychiatric
  and developmental disorders in a Danish population: a case-cohort
  study. Lancet Psychiatry 2018;\textbf{5} :573-80.
\item
  Fung WL, Butcher NJ, Costain G, et al. Practical guidelines for
  managing adults with 22q11.2 deletion syndrome. Genet Med
  2015;\textbf{17} :599-609.
\item
  Habel A, Herriot R, Kumararatne D, et al. Towards a safety net for
  management of 22q11.2 deletion syndrome: guidelines for our times. Eur
  J Pediatr. 2014;\textbf{173} :757-65.
\item
  Kobrynski LJ, Sullivan KE. Velocardiofacial syndrome, DiGeorge
  syndrome: the chromosome 22q11.2 deletion syndromes. Lancet
  2007;\textbf{370} :1443-52.
\item
  Unolt M, Versacci P, Anaclerio S, et al. Congenital heart diseases and
  cardiovascular abnormalities in 22q11.2 deletion syndrome: From
  well-established knowledge to new frontiers. Am J Med Genet A
  2018;\textbf{176} :2087-98.
\item
  Karayiorgou M, Simon TJ, Gogos JA. 22q11.2 microdeletions: linking DNA
  structural variation to brain dysfunction and schizophrenia. Nat Rev
  Neurosci 2010;\textbf{11} :402-16.
\item
  Constance LSL, Lansing MG, Khor FK, et al. Schizophrenia and
  anaesthesia. BMJ Case Rep 2017;\textbf{23} :bcr2017221659.
\item
  AlQarni MA, Alharbi A, Merdad L. Dental management of a patient with
  22q11.2 deletion syndrome (22q11.2DS). BMJ Case Rep 2018;\textbf{18}
  :bcr2018225765.
\end{enumerate}

\textbf{Figure Legends:}

\textbf{Figure.} Panoramic X-ray photograph at the first visit

Panoramic radiographs showed the apices of 11, 45, 46 (Federation
Dentaire Internationale) with round radiolucent images suggesting apical
lesions (Arrows to the lesions).\selectlanguage{english}
\begin{figure}[H]
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\includegraphics[width=0.70\columnwidth]{figures/Ito-figure-300dpi/Ito-figure-300dpi}

\end{center}
\end{figure}

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