International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 3 , Pages 149-152, September 2011

Combined sphenoid and frontal sinus aplasia accompanied by bilateral maxillary sinus hypoplasia in a child with sinusitis

  • Arzu Tatlıpınar

      Affiliations

    • Haydarpaşa Numune Research and Training Hospital, 1st ENT Clinic, Üsküdar, İstanbul, Turkey
    • Corresponding Author InformationCorresponding author at: Barboros mah, Fesleğen sok, Ağaoğlu My World Southside Sitesi, B3 Blok, Daire: 20 Batıataşehir, İstanbul, Turkey. Tel.: +90 216 6884540/532 227 66 57; fax: +90 212 2112500.
    • ,
  • Onur Dede

      Affiliations

    • Haydarpaşa Numune Research and Training Hospital, 1st ENT Clinic, Üsküdar, İstanbul, Turkey
    • ,
  • Erkan Esen

      Affiliations

    • Haydarpaşa Numune Research and Training Hospital, 1st ENT Clinic, Üsküdar, İstanbul, Turkey
    • ,
  • Tanju Gökçeer

      Affiliations

    • Haydarpaşa Numune Research and Training Hospital, 1st ENT Clinic, Üsküdar, İstanbul, Turkey
    • ,
  • Tayfun Akoğlu

      Affiliations

    • Haydarpaşa Numune Research and Training Hospital, Radiology Clinic, Üsküdar, İstanbul, Turkey

Received 9 February 2010; received in revised form 9 June 2010; accepted 12 June 2010. published online 22 July 2010.

Article Outline

Abstract 

Paranasal sinus agenesis is unusual clinical condition. The frontal sinus agenesis is more frequently seen, whereas agenesis of sphenoid sinus is extremely rare. Agenesis of both frontal and sphenoid sinus is very unique. This case presents a 13-year-old child with combined sphenoid and frontal sinus agenesis accompanied by bilateral maxillary sinus hypoplasia with scoliosis and coccyx abnormality.

Keywords: Sinusitis, Agenesis, Coccyx, Hypoplasia

 

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1. Introduction 

The paranasal sinuses begin their development as an evagination of the mucosa from the nasal cavities during the third and fourth fetal months. They undergo major expansion after birth, along with the development of facial cranium and teeth [1]. Although the sphenoid sinuses can be identified in sections of the fetus at 4 months, at birth the sinus remains small and is little more than an evagination of the sphenoethmoid recess. After the 3rd year, invasion of the sphenoid bone is more rapid, and by the age of 7, the sinus has extended posteriorly to the level of sella turcica. By the age of 12, the sphenoid pneumatization reaches its final form and size equivalent to the adult, although further enlargement into the basis sphenoid may occur in the adult [2]. The frontal sinus originates as anterosuperior outpouchings from the frontal recess of the nose. It is merely a slowly developing extension of the anterior ethmoidal sinus. At birth, it is undeveloped and nonaerated. The earliest pneumatization occurs at or shortly after age 2 years. The sinuses achieve their final size after puberty. Development of the frontal sinus is quite variable. Aplasia is present unilaterally in 15% and bilaterally in 5% of adults [3]. The maxillary sinus is the first paranasal sinus to form. At birth, the rudimentary aerated sinus is 6–8cm3 in volume. Asymmetry in size and shape of the sinus is common. Hypoplasia is unilateral in 7% and bilateral 2% of adults. Partial or complete opacification of the maxillary sinus in the first few years of life is normal [4].

The underdevelopment or agenesis of the paranasal sinuses is unusual clinical condition that refers more often to the frontal sinus and rarely to the other sinuses. Agenesis of sphenoid sinuses is uncommon phenomenon. Based on their anatomical studies, Wertheim and Grünwald reported in the early 1900s that agenesis of the sphenoid sinuses can occur in 1–1.5% of cases [5]. Combination of agenesis and hypoplasia of sinuses is extremely rare.

We report a 13-year-old child presenting chronic sinusitis with combined sphenoid and frontal sinus aplasia accompanied by bilateral maxillary sinus hypoplasia. She has also scoliosis and coccyx abnormality. To the best of our knowledge, this is the only case in the literature regarding multiple sinus agenesis and hypoplasia with scoliosis and coccyx abnormality.

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2. Case report 

A 13-year-old child who presented with symptoms of chronic sinusitis was referred for CT examination. She was complaining of postnasal discharge and chronic headache for years. Although she had medical treatment several times, her symptoms did not relieve. CT examination on coronal and axial planes showed bilateral absence of the sphenoid and frontal sinuses and bilateral maxillary sinus hypoplasia (Fig. 1a and b). Bilateral ethmoidal and maxillary sinusitis was also detected on CT examination. She was referred to pediatrics and orthopedics departments. Since these consultations revealed cervicothoracal scoliosis and coccyx abnormality on direct graphies (Fig. 2a), detailed imaging modalities were ordered. Cranial CT was normal. Lumbar spinal MRI revealed that lumbar lordosis was flattened and scoliosis was evident at inferior lumbar vertebral level (Fig. 2b). A prominent posterior angulation was observed at sacrum and coccyx on MRI. No haematological abnormality was detected on laboratory examinations. The patient had no craniofacial anomaly and neurologic deficit. There was no physical limitation and pain during her physical activities. Family history was not remarkable for congenital skeletal malformations. Patient was evaluated for cystic fibrosis and sweat test found to be normal.

  • View full-size image.
  • Fig. 1. 

    (a) Consecutive axial CT slices of paranasal sinuses demonstrate the lack of pneumatization in expected locations of sphenoid and frontal sinuses and bilateral hypoplastic maxillary sinuses. Bilateral soft tissue densities in maxillary sinuses are noted. (b) Sequential CT slices of paranasal sinuses in coronal plane shows the same findings in a.

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3. Discussion 

The underdevelopment or aplasia of the paranasal sinuses is rare phenomenon that refers mainly to the frontal and secondarily to the maxillary sinuses [3]. This occurs more frequently in syndromes of craniosynostosis, osteodysplasia (Melnick-Needles), as well as in cases of Down's syndrome (hypoplasia of the frontal sinus) [6]. Sphenoid sinuses vary in size and shape. The average measurements of the sphenoid sinus are vertical height, 2cm; transverse breadth, 1.8cm; anteroposterior depth, 2.1cm [7]. Pneumatization can extend into the greater wing, pterygoid processes and rostrum and encroach on the basilar part of occipital bone. Agenesis of sphenoid sinuses is extremely rare phenomenon. Previous researchers have reported that sphenoid sinus agenesis occurs in 1–1.5% of the population [5], [8]. However, these studies, most of which date back to first half of the 20th century lack the support of CT. There are only a few case reports in the literature regarding sphenoid sinus agenesis [2], [9], [10]. Degirmenci et al identified three sphenoid sinus agenesis cases in approximately 4500 paranasal CT examinations during 5-year period [10]. The diagnosis of sphenoid sinus hypoplasia is potentially important in patients in whom transsphenoidal hypophysectomy is contemplated. Maxillary sinus hypoplasia is an uncommon condition that may be misdiagnosed as chronic sinusitis [11]. Bolger et al. found the prevalence of unilateral hypoplastic maxillary sinus to be 10.4% on coronal CT scans [12]. Eggesbo et al. reported the developmental anomalies of paranasal sinuses in cystic fibrosis patients by comparing them with non-cystic fibrosis controls with inflammatory sinusitis. They reported five frontal sinus aplasia (4%) and bilateral maxillary sinus hypoplasias (5%) in their 126 controls aged 12 years or older. No sphenoid sinus aplasia was reported in patients or controls from all age groups in their study [13]. Small sphenoid sinuses have previously been reported in cystic fibrosis patients [14], [15], [16], [17].

To the best of our knowledge there is only one case report in the literature presenting agenesis of multiple paranasal sinuses identified by CT. In this case report Haktanır et al presented a 25-year-old man with a pre-diagnosis of sinusitis. His coronal and axial CT scans revealed combination of agenesis of sphenoid and frontal sinus and bilateral ethmoid and maxillary sinus hypoplasia [18].

Our case is unique in that the patient not only has multiple paranasal sinus agenesis and hypoplasia but also has scoliosis and coccyx abnormality. She has cervicothoracal scoliosis and her coccyx is retroverted instead of anteverted position (Fig. 2a and b). In spite of this coccyx anomaly the patient has no pain or physical limitation. We reviewed the literature and we could not find such a case or syndrome in the literature.

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4. Conclusion 

Awareness of paranasal sinus agenesis and hypoplasias is important for the surgeon to avoid possible intraoperative complications, plan operation accordingly and improve success of management strategies.

Our case is important in that it represents very unique entity regarding combined sphenoid and frontal sinus aplasia accompanied by bilateral maxillary sinus hypoplasia with scoliosis and coccyx abnormality.

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Conflict of interest 

Authors do not have any financial relationship or interest.

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References 

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PII: S1871-4048(10)00047-X

doi:10.1016/j.pedex.2010.06.002

International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 3 , Pages 149-152, September 2011

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