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285Chapter 20 Pediatric Sinonasal Disorders: Imaging
A
C D
E F
B
Section 5 Congenital/Pediatric Diseases286
ILLUSTRATIVE CASES
Sinus Masses
Pediatric sinus masses can be benign or malignant.
Benign masses seen in children includes:
■ Polyposis
■ Mucocele
■ Aneurysmal bone cyst
■ Fibrous dysplasia
■ Ossifying broma
■ Osteoma.
Malignant masses includes:
■ RMS
■ JNA
■ Lymphoma
■ Chloroma
■ Metastasis (Neuroblastoma)
■ Aggressive hemangioma
■ Ewings sarcoma
■ Germ cell tumor
■ Carcinoma (rare).
Benign:
Antrochoanal Polyp
A and B. Coronal computed tomography (CT) image shows homogenous
hypodense mass in the left maxillary sinus projecting in the nasal cavity
widening the osteomeatal unit (arrows).
C. Similar mass seen in another patient in right maxillary sinus obliterating
the right nasal cavity (arrow). No bony destruction seen. No enhancement is
seen within the mass s/o benign etiology.
Malignant:
Rhabdomyosarcoma
A to C. Computed tomography (CT) images of a 2-year-old boy shows large
expansile heterogenously enhancing soft tissue mass in left maxillary sinus
with surrounding aggressive type of bony destruction (arrow) and extension
in the nasal cavity is noted s/o of a malignant etiology.
287Chapter 20 Pediatric Sinonasal Disorders: Imaging
Benign
(Figs. 20.23A to C)
A
A
Malignant
(Figs. 20.24A to C)
B
B
CC
Section 5 Congenital/Pediatric Diseases288
ILLUSTRATIVE CASES
Sinus Masses (Contd..)
Nonaggressive benign sinus masses.
Ossifying Fibroma
A. Plain radiograph shows well-marginated, sclerotic epansile mass in left
maxillary and ethmoid regions (arrows).
B and C. Computed tomography (CT) image shows mixed soft tissue mass with
areas of bone density and thick bony walls in left maxillary and ethmoid sinuses
obliterating the superior left nasal cavity (arrows). It also shows relatively
well-dened margins.
Fibrous Dysplasia
A. Plain radiograph shows expansion and sclerosis of maxillary sinus with
relatively maintained contour (arrow). Margins of the sclerotic region are
merging with the surrounding bone in a patient of brous dysplasia (FD).
B and C. Computed tomography (CT) images show bony expansion of the
left ethmoid and sphenoid sinuses which show ground glass matrix (arrows).
Involvement of the left middle turbinate presented as an intranasal mass.
289Chapter 20 Pediatric Sinonasal Disorders: Imaging
Ossifying Fibroma
(Figs. 20.25A to C)
A
A
Fibrous Dysplasia
(Figs. 20.26A to C)
B
B
CC
21
CHAPTER
Pediatric Sinonasal Disorders:
Surgical Perspective
Prem Sagar, Shuchita Singh
• Congenital Malformations of the
Nose
– Developmental Errors of Anterior
Neuropore
– Developmental Errors of the
Central Midface
▪ Aplastic and Hypoplastic
Anomalies
▪ Atretic Anomalies
▪ Hyperplastic and Duplication
Anomalies
▪ Craniofacial Clefts
– Developmental Errors of the
Nasobuccal Membrane
▪ Choanal Atresia
• Rhinosinusitis
– Acute Bacterial Rhinosinusitis
– Chronic Bacterial Rhinosinusitis
• Chronic Adenoid Hypertrophy
• Specic Lesions
CONGENITAL MALFORMATIONS OF THE NOSE
Congenital malformations of the nose may be categorized as developmental
errors of the anterior neuropore, errors of central midface, and errors of the
nasobuccal membrane. Imaging features of these disorders are detailed in
Chapters 20 and 23.
Developmental Errors of Anterior Neuropore
During rst trimester, a dural projection extends through foramen cecum,
prenasal space up to the ectoderm at the tip of the nasal bones. As the
foramen cecum closes, this extension gets detached from the nasal bones and
gets retracted into the cranium. Dierent type of errors in this developmental
process leads to common congenital malformations as enumerated in
Table 21.1.
■ Encephaloceles may be meningoceles or meningoencephaloceles
depending on the inclusion of meninges only or meninges along with
brain tissue, respectively.
■ Encephaloceles in sinonasal region may be sincipital (more common)
or basal encephaloceles. Dierent types of sincipital and basal encephaloceles are summarized in Tables 21.2 and 21.3, respectively.
Table 21.1: Common developmental errors of the anterior neuropore.
Type of error Pathophysiology Schematic representation
Dermoid cyst/
sinus/stula
Glioma Premature closure of
Encephalocele Premature closure of
Posterosuperiorly
retracted ectoderm
through foramen
cecum
foramen cecum with
entrapped glial tissue
(without any patent
communication with
CNS)
foramen cecum with
entrapped meninges
± brain parenchyma
communicating with
CNS
291Chapter 21 Pediatric Sinonasal Disorders: Surgical Perspective
Table 21.2: Characteristics of sincipital encephaloceles.
Clinical location of the
Type Skull base defect
Nasofrontal Between orbits and nasal + frontal
bones
Nasoethmoid Foramen cecum, deep to nasal
bones
Naso-orbital Deep to nasal and frontal bones
through defect in medial orbital wall
encephalocele
Supercial to nasal bones as
glabellar mass
Nasal dorsum
Orbital mass
Table 21.3: Characteristics of basal encephaloceles.
Type Skull base defect Clinical location of the encephalocele
Transethmoidal Cribriform plate Nasal cavity
Sphenoethmoidal Between posterior ethmoid
cells and sphenoid sinus
Transsphenoidal Craniopharyngeal canal Nasopharynx
Spheno-orbital Superior orbital ssure Orbit
Nasal cavity
■ e clinical presentation and treatment of these three types of anterior
neuropore errors are summarized in Table 21.4 (Fig. 21.1).
Section 5 Congenital/Pediatric Diseases292
Table 21.4: Clinical presentation and treatment protocol of
dierent types of anterior neuropore errors.
Type of error Symptoms Signs Treatment
Dermoids Midline pit or swelling
at the rhinion (Fig.
21.1), which may
have intermittent
caseous discharge or
infections
Gliomas Extranasal mass
(most commonly
at the glabella),
intranasal mass
arising from middle
turbinate or septum
or as both extra- and
intranasal mass
Encephaloceles Mass lesion at
locations as
described previously
Cysts are rm,
lobulated and
noncompressible,
do not enlarge
on coughing or
crying and do not
transilluminate
Nonpulsatile, do
not increase in size
with coughing or
crying and do not
transilluminate
Pulsatile, bluish,
compressible
masses, which
expand on crying
or coughing and
transilluminate
brilliantly
Complete surgical
excision
Early surgical excision
Surgery
Fig. 21.1: Classical clinical picture of nasal dermoid, note the hair follicle coming out of the
midnasal lesion along with the broad nasal bridge.
■ Nasal dermoids may present along with aural atresia or deformities,
hydrocephalous, cleft lip and palate, hypertelorism, hemifacial
microsomia, branchial arch anomalies, and mental retardation.
■ Dermoids with intracranial extension is not uncommon and is detected
by computed tomography (CT) and magnetic resonance imaging (MRI).
Developmental Errors of the Central Midface
Aplastic and Hypoplastic Anomalies
■ Arhinia is congenital absence of external nose and airway, along with
paranasal sinuses and olfactory bulbs.
• Distinctive “dish-face” deformity with hypertelorism or hypotelorism
and normal labial as well as philtrum development.
• May be associated with anophthalmia or hypoplasia of the eye and a
high-arched palate.
• Surgery involves reconstruction of the external nose and correction of
the high-arched palate.
■ Heminasal aplasia is the agenesis of unilateral nostril (Fig. 21.2).
• Occurs in isolation or in combination with ipsilateral facial anomalies.
• Radiology may show associated absence of cribriform plate.
• Staged surgical correction.
■ Hypoplastic conditions include columellar agenesis, complete or partial
nasal bone agenesis, hypoplastic anterior nasal spine, nasomaxillary
hypoplasia, underdeveloped nasal dorsum, and hypoplastic nares.
293Chapter 21 Pediatric Sinonasal Disorders: Surgical Perspective
Fig. 21.2: Clinical picture of a patient with heminasal aplasia with absence of right half of
the nose.
Section 5 Congenital/Pediatric Diseases294
Atretic Anomalies
■ Patients present with nasal obstruction, respiratory distress, and feeding
diculties along with cyanotic spells, which are temporarily relieved on
crying.
■ Inability to pass a nasal suction catheter is a reliable test to detect nasal
stenosis, but nasal endoscopy conrms the diagnosis.
■ e CT scan provides information about the site, extent, and nature
(membranous or bony) of the stenosis, which is crucial prior to surgical
correction.
■ Mild forms may be managed conservatively using saline nasal drops,
topical decongestants, humidication, and frequent suctioning.
■ Nasal pyriform aperture stenosis results due to bony overgrowth of the
nasal process of maxilla causing narrowed nasal inlet.
• Clinical examination reveals narrow anterior nasal passage with bony
thickening medially.
• Patients may also have hypotelorism and a at nasal bridge along with
a central mega-incisor.
■ Midnasal stenosis occurs either due to unequal growth of the lateral nasal
walls or excessive folding of the nasal septum.
Hyperplastic and Duplication Anomalies
■ Polyrhinia is the presence of two external noses, which arise from two
pairs of normally developing nasal placodes.
• May be associated with bilateral choanal atresia.
• Correction involves excision of medial portions of each external nose.
■ Supernumerary nostril is an extra-nasal opening either lateral, medial, or
superior to the normal nostril.
• May be unilateral or bilateral and may or may not connect with the
nasal cavity.
• Surgically excised.
■ Proboscis lateralis is a rare craniofacial malformation in which external
nose fails to develop on one side and is replaced by a tubular structure
emanating from the medial canthus.
• May occur in isolation or in association with a wide spectrum of
anomalies of ipsilateral paranasal sinuses and nasolacrimal system,
absent olfactory nerve, olfactory lobe, and cribriform plate on the
aected side.
• Surgery is done after complete facial growth.
Craniofacial Clefts
■ e craniofacial clefts are very rare and the hallmark features are ocular
hypertelorism, broad-nasal root, ill-dened nasal tip, anterior cranium
bidum occultum, median clefting of the nose, lip and palate, and
unilateral orbital clefting or notching of the nasal ala.
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