Craniofacial growth is a dynamic process balancing form and function. This equilibrium is easily disturbed at various levels by passive or active change of one of the mechanisms involved, prenatally or postnatally. The impact of the interaction with the adhering muscles on the developing midfacial skeleton has been recognised in recent years (Stricker et al. 1990). A spectrum of midfacial malformations may be observed as part of a syndrome or as solitary deformity which may point to an interaction between growth of the nasal septum and the premaxilla-maxilla. One of the most well-known congenital facial deformities is the cleft lip, alveolus and palate. In adult human skulls with facial clefts, which were known to be untreated, a specific pattern of growth disturbances could be observed (van Limborgh 1964; Atherton 1967). Unilateral clefts showed a deviation of the premaxilla to the non-cleft side, whereas the maxillary part on the cleft side had collapsed medially and fell behind compared to the non-cleft side (retrognathism). In addition, these skulls showed a specific malformation of the nasal septum with (a) a deviation of the perpendicular plate to the cleft side and (b) a disjunction between perpendicular plate and vomer (Fig. 35.9). The vomer, only connected to the palatal margin of the cleft, tends to a more horizontal position to meet the deviated perpendicular plate, suggesting a broadening of the nasal floor on the non-cleft side. From this study, it was concluded that these growth anomalies of the maxilla and nasal framework were part of a cleft syndrome, specific for each type of cleft (Verwoerd et al. 1989a, b). Collapse of the upper alveolar arch and maxillary retrognathism were also observed in adult members of a North-Borneo tribe who were born with cleft lip, alveolus and palate and did not undergo surgical treatment (Innis 1962). In a study on lateral cephalograms of untreated unilateral cleft patients with superimposed images of cleft and non-cleft side, the abnormal position of the maxilla could not confirmed, most probably due to the overprojection of the non-affected side (Capelozza et al. 1993).

Whether a combination of midfacial anomalies associated with a cleft alveolus and palate might represent a syndrome of craniofacial anomalies developing as reaction to a cleft was investigated in growing rabbits (Verwoerd et al. 1979a). Unilateral clefts of alveolus and palate, produced surgically in young growing rabbits, appeared to affect the further development of nose and (pre)maxilla into adulthood (Fig. 35.10a). On the cleft side the molar complex, lacking a connection with the growing septum, does not move forward and will show – in the adult stage – a retroposition (retrognathism) compared to the non-cleft side. Here, the premaxilla, maxilla and nasal bones will grow to normal length and gradually rotate and deviate to the non-cleft side as previously reported for human skulls (van Limborgh 1964). The results of these animal experiments indicate that the cleft syndrome as identified in ‘untreated’ human skulls with facial clefts should be considered an adaptation to the altered developmental mechanics in the developing midfacial skeleton.

Another interesting observation has been done when in young rabbits the premaxillary-maxillary suture was resected and replaced by non-sutural bone (Fig. 35.10b). Following this intervention normal lengthening of the ipsilateral upper jaw failed. Secondary effects were a progressive deviation of the premaxilla to the nongrowing side and an excessive forward shift of the molar complex on the operated side. These observations confirm disappointing midfacial development as observed after osteoplasty of an alveolar cleft in young children. The outcome of the above-mentioned experiments refers to the role of the cartilaginous nasal septum in postnatal development of the upper jaw. The growing and lengthening of the cartilaginous nasal septum, connected on both sides to the premaxilla-maxilla, seems to be responsible for lengthening of the upper jaw and, secondly, for a shift in anterior direction of the upper jaw relative to the cranial base. In the presence of a unilateral cleft, the ‘mechanical’ balance between both sides is disturbed.

Although the results of orthodontic and surgical treatment of facial clefts are improving, the secondary cleft nose in these patients can demonstrate some of the following features: asymmetry of tip, columella, nostril, ala and nostril floor; deflection of the caudal part of the septum cartilage to the non-cleft side; stenosis of the vestibule on the cleft side; hypoplasia of the maxilla on the cleft side; variable collapse of the maxilla with asymmetry of the piriform aperture ; underdevelopment of the maxilla with retroposition of the anterior nasal spine; deviation of the cartilaginous and bony nasal dorsum; and deviation of the posterior part of the nasal septum to the cleft side (Verwoerd et al. 1995).

Nasal congenital anomalies are extremely rare and range from bifidity of the nasal tip or dorsum to nasal aplasia with or without proboscis (Stricker et al. 1990). Reports on nasal dysplasia, which is mostly indicating a unilateral malformation, are scattered through the literature. The nasal cavity is missing and pneumatisation of the maxillary, ethmoidal and frontal sinuses has failed. Exploration reveals no cartilage, but just solid bone. The affected side of the maxilla is hypoplastic and regularly associated with other malformations such as cleft lip/palate or coloboma. The last variety to be mentioned is a duplication of the nasal dorsum, which can occur in different forms and as part of a syndrome (Fig. 35.11).

Various studies refer to the importance of the nasal septum for midfacial development. In humans, intrauterine exposure to warfarin appeared to cause early calcification of the septal cartilage; subsequent nasal and midfacial hypoplasia was demonstrated in a cephalometric study, suggesting that even in utero midfacial growth is retarded by interference with the nasal septum (Howe et al. 2004).

The neonate’s nose may show a slight or more pronounced deviation, with a luxation of the lower septal border into the nasal cavity, acquired during the passage through the birth canal (Fig. 35.12). In most cases this anomaly will restore spontaneously but infrequently the deformity has to be corrected by manipulation of the neonatal nasal septum which is still mostly cartilaginous from sphenoid to columella. Already in 1978, Gray found some type of septum anomaly in 57 % of 2,380 neonates (Gray 1978). Septal deformity was explained by Takahashi to be an inevitable condition resulting from the autonomic growth force of the septum cartilage (Takahashi 1988).

Any kind of mechanical or surgical trauma at a young age, destroying the septodorsal cartilage, initiates an irreversible disturbance of the cartilage from its genetically based developmental orientation. A lesion may vary from fracture and dislocation, with later deviation, to haematoma and abscess formation and loss of septum cartilage, with various associated sequelae. The regional differences in thickness of the septum cartilage, described above, seem to explain that fractures preferably occur at sites with the least mechanical strength and thus the thinnest parts of the septum. Septal deviations could, therefore, be related to the ‘weak’ areas (Mladina 2000). Dislocation of the caudal end of the septum, the most frequent disorder in young children, is caused by a fracture running through the thin area lying cephalic of the columellar rim. The thin area above the thick basal rim is another vulnerable fracture-prone region. This preference of specific fracture lines was described by several authors in children as well as adult patients; it was demonstrated in the so-called C-fracture running horizontally and superiorly along the thick basal rim of the septum, then more horizontally through the thin portion of the perpendicular plate, while ending in the thinner zone just under the nasal dorsum (Harrison 1979; van Velzen et al. 1997). Another weak point is the area of thin cartilage, semi-parallel to the caudal rim and just anterior to the connection between septum and upper lateral cartilages, which may easily fracture when the caudal end of the septum gets dislocated (viz. Fig. 35.2c).

Although injuries of the nose appear to occur frequently in young children – and the incidence seems still to be growing – only a relative small number is seen by the general practitioner, and even less are diagnosed by an ORL specialist. Consequently the long-term follow-up of these cases is rare and certainly no routine. In the years following such a ‘minor’ trauma, however, a progressive deformity of the midface may be observed with increasing deviation or distortion of the nose, eventually in combination with an underdevelopment of the maxilla, while the final effects can only be defined after the adolescent growth spurt.

After a traumatic accident, fractured parts of the bony midface and the septum will lead to, often disproportionate, disfigurement of the facial profile. In particular, the less obvious lesions of the cartilaginous septum are seldom diagnosed and during further growth the fractured parts will bend and dislocate increasingly, thus leading to spine formations, local overlapping edges and finally to severe deviations (Manning 1999). A midfrontal trauma may cause multiple fractures with larger and smaller pieces of cartilage resulting in a highly distorted septum and finally an underdeveloped and distorted nose (Fig. 35.13). The direction of deviations is mostly unpredictable, because it depends on the direction of the deforming force and on the reaction of the traumatized tissues involved. Captivating evidence for the significance of the nasal septum in midfacial growth is supplied by a rather large study of 21 monozygotic twins ranging in age from 18 to 22 years by Grymer (Grymer et al. 1991). In only one of each twin pair, a deformity was found in the anterior part of the septum. The nose of the affected siblings was demonstrated by cephalometry to be shorter than in their ‘normal’ other half.

From many human case studies described in the literature, it is evident that the sequelae of a nasal injury are frequently a septum haematoma and, due to contamination and infection, a subsequent septum abscess. A haematoma may mostly, when not treated adequately, and an abscess may invariably cause destruction of the nasal cartilage leading to septum perforation or a submucosal defect of the septum cartilage. Saddling deformity with loss of tip support and growth retardation with a short nasal dorsum are then regular findings (Fig. 35.14a, b). Grymer and Bosch reported about one twin sibling, who endured septum destruction due to a septum abscess at the age of 7 years and was followed up with its twin for more than 10 years (Grymer and Bosch 1997). Despite the treatment with homologous cartilage from the tissue bank, the affected boy developed a severe saddle nose with upward displacement of the anterior part of the maxilla, decreased nasal projection and a retrognathical position of the maxilla compared to the unaffected sibling. This observation suggested that the nasal septum has a great influence on midfacial development determining vertical as well as anteroposterior maxillary growth. And secondly, that bank cartilage grafting is not adequate in preventing malformations of the midface during growth. Patients with a history of facial trauma develop progressive nasal deformation and/or nasal obstruction but the younger the age of the child the more impact such a lesion may have in the long run (Pirsig 2000). Also the extent of the injury – fracture, haematoma, abscess and perforation, surgical treatment – will play a role in the final result after the adolescent growth spurt. And these results are often disappointing, thus many of these patients have to undergo a second or even more surgical procedures later in life (Derkay 1999).

From a series of 241 children with a history of nasal injury, 40 % was diagnosed with pathological septal findings, with a longest follow-up period after trauma of 10 years (Blahova 1985). Septum deviation associated with snoring and abnormal rhinomanometric values appeared in 55 % of the patients. In a group of 16 paediatric patients, ranging from 2 to 14 years, major sequelae (62,5 %) like dorsum, tip or pyramid deformation, septum deviation with nasal obstruction, functional vault deformity and septum perforation were observed, whereas the other patients (37,5 %) all showed minor sequelae without any airway compromise (Alvarez et al. 2000). Another consecutive series of 20 children (2 months–15 years) were admitted to the hospital for treatment of nasal trauma followed by nasal abscess (12 patients) universally associated with destruction of the septum cartilage (Canty and Berkowitz 1996). Nasal obstruction, however, was the most common symptom (19 patients). Recently a structured review of PubMed, EMBASE and the Cochrane Databases comprising 81 citations, dating from 1920 up to now, regarding nasal septal abscess was published (Alshaikh and Lo 2011). It was concluded that it is a serious condition that necessitates urgent surgical management in order to prevent severe complications, and even the growing child should be treated with early reconstruction essential for normal development of the midface. In this whole sequence, the lack of knowledge of the developmental mechanics is part of the problem.