Background/Objectives: Crouzon’s syndrome is characterized by premature closure of the cranial sutures, midface hypoplasia, orbital deformities &amp; other associated abnormalities.Children with Crouzon syndrome frequently have obstructive sleep apnea due to the underdevelopment of the midface.Case report: A 12 year old boy of Crouzon’s syndrome with chronic adeno-tonsillitis was managed by adeno-tonsillectomy under general anaesthesia by scalpel cautery method. The boyresponded well to surgery &amp; the mild sleep disorder disappeared within a week uveventfully.Conclusion: Sleep disorders in this condition can be treated by improving the airway by selective procedures like midface advancement, mandibular expansion , adeno-tonsillectomy,uvulo-palatopharyngoplasty, anterior tongue reduction &amp; endoscopic tracheal granuloma excision.

Appaji, Mohan

Devi, Pinky

MK, Goutham

Naik, Sarika s

Naik, Sudhir M

S, Ravishankar

SM, Annapurna

English

ABSTRACTBackground/ObjectivesTonsillectomy using local anesthesia (local tonsillectomy) is a safe and effective alternative to general anesthesia in the healthy cooperative teenage or adult patients. Blood loss, morbidity, complications and patient satisfaction were better in some studies with tonsillectomies done under general anesthesia.DesignComparative case series analysis study of two groups of patients who underwent tonsillectomies under local and general anesthesia during the study period of 57 months from March 2007 to December 2011.Materials and methodsOverall 1,349 cases of tonsillectomies done for chronic tonsillitis were included under the study. Three hundred and sixty-seven cases operated under general anesthesia and 982 cases operated under local anesthesia were compared. The parameters compared were duration of surgery, blood loss and the mean pain visual analog score.ResultsSignificant difference in duration of surgery, blood loss during surgery and visual analog scale (VAS) pain scores were seen in the two groups with local anesthesia scoring over general anesthesia.ConclusionTonsillectomy under local anesthesia is a good alternate for the procedure under general anesthesia with limited resources and in cooperative adults.How to cite this articleNaik SM, Naik SS, Ravishankara S, Appaji MK, Goutham MK, Devi NP, Mushannavar AS. Advantages of Tonsillectomy done under Local Anesthesia compared to General Anesthesia in Adults. Int J Head Neck Surg 2013;4(1):13-18.</jats:sec

Sudhir Naik

Sarika S Naik

S Ravishankara

Mohan K Appaji

MK Goutham

Nonthombam Pinky Devi

Annapurna S Mushannavar

Crossref

International Journal of Head and Neck Surgery

Advantages of Tonsillectomy done under Local Anesthesia compared to General Anesthesia in Adults

Otolaryngology online journal

ISSN:2250-0359                                            Volume 3 Issue 3 2013
Crouzon’s syndrome with adenotonsillitis: conventional surgery in
altered anatomy.
Sudhir M Naik
1, Mohan Appaji
2 , Ravishankar S
3, Goutham MK
4, Annapurna SM
5, N Pinky
Devi
6, Sarika S Naik
7
1 Fellow, Department of Head and Neck Oncosurgery, KMIO, Bangalore.
2 Professor & Head of Department of ENT, Head and Neck surgery, KVG Medical College, 
Sullia, Karnataka.
3 Associate Professor , Department of ENT, Head and Neck surgery, KVG Medical College, 
Sullia, Karnataka.
4,5,6Junior resident, Department of ENT, Head and Neck surgery, KVG Medical College, Sullia, 
Karnataka.
7 Senior resident, Department of Anaesthesia and Critical care, Narayana Hrudayalaya, 
Bangalore.
Abstract:
Background/Objectives: Crouzon’s syndrome is characterized by premature closure of the 
cranial sutures, midface hypoplasia, orbital deformities & other associated abnormalities. 
Children with Crouzon syndrome frequently have obstructive sleep apnea due to the 
underdevelopment of the midface. 
Case report:  A 12 year old boy of Crouzon’s syndrome with chronic adeno-tonsillitis was 
managed by adeno-tonsillectomy under general anaesthesia by scalpel cautery method. The boy 
responded well to surgery & the mild sleep disorder disappeared within a week uveventfully.
Conclusion:  Sleep disorders in this condition can be treated by improving the airway by 
selective procedures like midface advancement, mandibular expansion , adeno-tonsillectomy, 
uvulo-palatopharyngoplasty, anterior tongue reduction & endoscopic tracheal granuloma 
excision.      
Introduction:
 
Crouzon’s syndrome is a rare autosomal dominant skeletal disorder caused by multiple mutations
in the fibroblast growth factor receptor 2 (FGFR2) gene.
1 Paternal age older than 35 years is 
associated with the 60% of all  new mutations leading to this condition.
1 It occurs in one of every
25,000 live births & accounts for 5% of cases of craniosynostosis.
1 
      Crouzon syndrome has 2 variants, the classic variant is caused by a mutation of the fibroblast
growth factor receptor gene FGFR-2, located on chromosome 10 & the second variant, notable 
for the presence of abnormal skin pigmentation called acanthosis nigricans, is the result of a 
mutation in FGFR-3, located on chromosome 4.
2 Nucleotide alterations causing amino-acid 
substitutions at the FGFR2 gene on chromosome 10 lead to the Crouzon phenotype.
1
    It is commonly inherited as an autosomal dominant trait, with complete penetrance & variable
expressivity,
3 but 30 to 60% of cases are sporadic & represent fresh mutations.
5 It accounts for 
approximately 4.8% of all cases of craniosynostosis, with the prevalence of approximately 
1/25,000 live births worldwide.
3,4  Clinical findings include brachycephalic craniosynostosis, 
significant hypertelorism, proptosis, maxillary hypoplasia, beaked nose & possibly, cleft palate. 
Intracranial anomalies include hydrocephalus, Chiari 1 malformation & hindbrain herniation.
1
        It is characterized by premature closure of the cranial sutures, midface hypoplasia, orbital 
deformities & other abnormalities.
3 The diagnosis is based on clinical findings and radiological 
examination.
3 Early recognition and specific therapeutic intervention are essential to guide the 
growth & development of the craniofacial region.
3 Pathology of the ear & cervical spine is 
common.
2 Infants with Crouzon’s syndrome do not have anomalies of the hands & feet as do 
infants with Apert’s syndrome.
2
           The abnormal skull shape is usually noted in the newborn period although, occasionally, it
may be detected either prenatally or not until later in infancy. The appearance of an infant with 
Crouzon’s syndrome can vary in severity depending on the order and rate of the fusion of the 
sutures.
3 Despite tremendous advances in the establishment of inheritance mode of this condition
and its prevention & treatment, it remains a significant cause of morbidity worldwide.
 3     It is important to be aware of the syndrome so as to impart better care.
3 Genetic testing & 
individual study of each patient with suspected syndrome are essential for early diagnosis and 
management & to differentiate the condition from other syndromic and non-syndromic 
craniosynostosis.
3
     We report a rare case of Crouzon’s syndrome in a 12-year-old boy with characteristic features
of maxillary hypoplasia, proptosis  with minimal cranial cranial deformity  & chronic 
adenotonsillitis.
Case report:
    A 12-year-old boy presented with complains of sore throat painful swallowing since a week 
with similar episodes since 3 year. He incidentally had features suggestive of midfacial 
hypoplasia with no complains of obstructive sleep disorders.
    No difficulty in speech but occasional difficulty in swallowing due to enlarged tonsils was 
reported. All other family members were normal. At the time of birth father was 37 years and his
mother 29 year old & they had a non-consanguineous marriage. History from his parents 
revealed that his facial features were different from other children of his age. They had noticed 
the gradual bulging of his eyes after the age of 1 year. Parents complained of chronic mouth 
breathing and snoring at sleep with chronic denasal speech.
     His developmental milestones were normal, but he appeared smaller than other children of his
age. At clinical examination cranial vault was regular with no undue prominence. No 
brachycephaly, flatness of forehead or occiput was seen with normal frontal hairline. No ridging 
of the skull was seen in regions of coronal, lambdoid sutures & anterior to the vertex. Midface 
flattening, with maxillary hypoplasia and a relatively large mandible was seen. The upper lip was
small compared to the everted lower lip. (fig1,2,3)
   Ophthalmologic evaluation revealed shallow orbits, hypertelorism, bilateral proptosis, mild 
exotropia, no papillary edema or optic atrophy. His nasal bridge was normal with right septal deviation. His nose was slightly pointed with dorsal hump with normally set ears with normal 
hearing. No abnormalities seen in hands and feet and dentition.
Oral cavity showed U-shaped dental arches with a high-arched palate with hard and soft palate 
being normal. There was no evidence of temporomandibular dysfunction. Ortho-pantomographic
view confirmed the clinical findings. 
    A retruded maxilla with a relatively large mandible was seen on x-ray lateral view of the skull.
The widely spaced orbits, hypoplastic maxilla and zygoma are due to early fusion of coronal and 
lambdoid sutures. Cardiovascular, respiratory, and abdominal examinations were normal. 
Routine hematological and biochemical tests were normal.(fig4,5)
     
Based on the above clinical and radiological findings and in the absence of hand and feet 
anomalies, a diagnosis of Crouzon’s Syndrome was made. Clinically normal airway within 
normal cardiac and respiratory functions was seen on pre-anaesthethic examination. He was 
accepted for adenotonsillectomy in ASA II under general anaesthesia. Initially difficulty was 
seen in fixing the mouth gag with large tongue and retruded maxilla.The surgery was done in 45 
minutes under scalpel cautery method. 
Discussion:
  
      Crouzon’s syndrome was first described by Octave Crouzon in a mother & son with the 
characteristic triad of calvarial deformities, facial anomalies & proptosis.
6 Mutation of the FGFR 
gene is also responsible for other cranio-synostosis such as Apert’s, Pfeiffer’s, Jackson-Weiss’, 
and Saether-Chotzen’s syndromes.
7  Rarely, acanthosis nigricans may coexist with Crouzon 
syndrome & is caused by mutation in the transmembrane region of the FGFR3 gene.
8 The 
sporadic cases are postulated to be associated with advanced paternal age & some investigators 
have found that this mutation is more common in the sperm of older men.
9       Due to variability of phenotypic expression, some patients exhibit complete phenotypic 
penetrance, whereas other family members may appear normal & still carry a Crouzon mutation 
as seen in our patient.
10  Crouzon’s syndrome has no racial or sex predilections.
10 The 
craniosynostosis  of sagittal or metopic types are more predominance in boys, while coronal 
craniostenosis is more common in girls.
11 The condition is usually detected in the first year of life
& sometimes congenital forms are seen at birth due to synostosis in the uterus itself.
11
       Here the coronal sutures usually close first and eventually all cranial sutures close early.
11 
The coronal and sagittal sutures are most commonly involved.
11 As the skull grows in planes 
perpendicular to the cranial sutures, premature suture closure causes skull growth to cease in the 
plane perpendicular to the closed suture & to proceed parallel to the suture.
11 The skull shape 
becomes asymmetric, with the shape depending on the sutures involved.
12  The characteristic 
cranial shapes are brachycephaly (disproportionate shortness of the head), scaphocephaly 
(boat-shaped head), trigonocephaly (triangle-shaped head) or, in severe disease, cloverleaf skull 
deformity.
12
      The facial malformations consist of hypoplasia of the maxilla with mandibular prognathism,
high-arched palate, bilateral palatal swelling, rarely cleft palate, exaggerated facial angle, nose 
appears prominent & pointed (beak-like nose), recalling a ‘parrot beak’ due to short & narrow 
maxilla.
13 Conductive hearing loss and ear infections are common due to middle ear 
deformities.
11 Upper airway obstruction may occur due to midfacial hypoplasia & a narrow 
epipharynx.
11 Ocular abnormalities such as bilateral ocular proptosis due to extremely shallow 
orbits and hypertelorism due to decrease in the growth of sphenozygomatic & sphenotemporal 
sutures.
14
      Optic atrophy is frequently seen in 30 to 80% of patients.
15 Other ocular abnormalities 
include divergent strabismus, conjunctivitis or exposure keratoconjunctivitis, as well as 
unexplained loss of visual accuracy.
15 Mental ability and psychomotor development is generally 
within the normal limits. However, increased intracranial pressure can lead to mental 
retardation.
11 Sometimes headache & convulsions are also seen.
11 Thorough clinical, radiological,
and genetic analysis is required for early recognition & diagnose of this syndrome.
11       X-rays show obliterated sutures, hypoplastic maxilla with shallow orbits, shortened cranial 
fossa, enlarged hypophyseal cavity & small paranasal sinuses.
13  Prominent cranial markings of 
the inner surface of the cranial vault may be seen as multiple radiolucencies appearing as 
depressions (so called digital impressions) resulting in the hammered silver (beaten metal/copper
beaten) appearance.
 13 
       A copper beaten skull indicating internal remodelling of the calvaria due to an increase in 
intracranial pressure as a result of premature cranial suture fusion may be seen.
11 On spine 
radiography, abnormal craniocervical junction, butterfly-shaped vertebrae, and fusion of the 
cervical vertebrae (C2-C3 and C5-C6) may be visible.
11  X-ray examination of the metacarpal 
bones & fingers may reveal slight achondroplasia.
13  Ventriculomegaly is frequently noticed in 
central nervous system imaging, but is usually asymptomatic & does not require treatment.
16 
Corpus callosum agenesis &  optic atrophy are rarely seen on MRI.
16
      3-dimensional ultrasonography and MRI aid early detection & diagnosis of fetal 
malformation.
17 Prenatal diagnostic testing for a known FGFR gene mutation is a reasonable 
option for couples at risk for having a child with Crouzon syndrome due to germline 
mosaicism.
17 The syndrome must be differentiated from simple craniosynostosis & other 
syndromic cranio-synostosis.
18  Once craniosynostosis is seen radiographically, it is important to 
determine whether it occurred because of abnormal biology of the cranial suture, possibly caused
by an FGFR mutation (primary cranio-synostosis).
18 
      The features of Apert’s syndrome are very similar but are characterized by craniosynostosis, 
midface hypoplasia, and symmetric syndactyly of the hands
and feet.
19 Differential diagnosis is also made with the other syndromes associated with features 
of craniosynostosis such as Pfeiffer’s syndrome, Saethre-Chotzen’s syndrome, and 
Jackson-Weiss’ syndrome.
19  All these syndromes involve craniofacial abnormalities as well as 
other abnormalities, including the hands & feet.
19       The management requires a multidisciplinary approach to prevent early fusion of 
craniofacial suture, thus minimizing ICP & secondary craniofacial deformities.
19 Early release of 
the synostotic sutures of the skull allows adequate cranial volume for brain growth.
19 Skull 
reshaping may need to be repeated as the child grows.
19 Depending on the severity of the 
deformity, midfacial advancement and jaw surgery can be done to provide adequate orbital 
volume & correcting the occlusion for appropriate function.
19 The prognosis depends on the 
severity of the malformation and the timing of intervention. Patients usually have a normal 
lifespan.
19
        In a study of 244 children  20% of these children with  syndromes with respiratory distress 
required tracheostomy.
20 Craniofacial synostosis patients (Crouzon, Pfeiffer, or Apert syndrome) 
had the highest rate of tracheotomy (48%), mandibulofacial dysostoses patients (Treacher 
Collins or Nager syndrome) being the next highest rate (41%) &  patients with 
oculo-auriculo-vertebral sequence were less likely to undergo tracheotomy (22%).
20  Children 
with craniosynostosis rarely required a surgical airway, unless there was marked associated 
facial dysmorphism (1%).
20 
     The presence of a cleft palate correlated with reduced risk for tracheotomy. We performed 
adeno-tonsillectomy to improve the snoring & sleep disorder problem. Other procedures done to 
improve airway are midface advancement, mandibular expansion, uvulopalato-pharyngoplasty, 
anterior tongue reduction, and endoscopic tracheal granuloma excision.
20
  Conclusions:
      Crouzon syndrome can present with a wide range of clinical features. The affected boy here 
presents with varying degees of craniosynostosis, ocular proptosis, hypertelorism, hypoplastic 
maxilla & chronic tonsillitis. The diagnosis is usually based on clinical & radiographic findings 
of the craniofacial region. 
    Molecular genetic testing for mutations in the FGFR2 gene may be useful adjuncts, 
particularly when prenatal detection in subsequent family members is desired. Sleep disorders in 
this condition can be treated by improving the airway by selective procedures like midface advancement , mandibular expansion , adeno-tonsillectomy, uvulo-palatopharyngoplasty, 
anterior tongue reducition & endoscopic tracheal granuloma excision.
Figure showing Protrusion of the mandible seen with no maxillary prominenceFigure showing No deformity of the palate but a large tongue compared to the mouth cavity.
Normal x-ray of the hand & cranial vault with maxillary hypoplasia seenNormal chest x-ray with maxillary hypoplasia seen.
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Crouzon’s syndrome with adenotonsillitis: conventional surgery in altered anatomy.

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Crouzon’s syndrome with adenotonsillitis: conventional surgery in altered anatomy.

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