Nasal Clefts Research Articles

Alar Rim Triangular Flap for Congenital Nasal Cleft Repair in Pediatric Patients.

According to Tessier classification, number 1 and number 2 craniofacial clefts involve the nasal ala. Congenital nasal cleft is not common and is difficult for reconstruction. Notches in the medial one-third of either nasal ala are typical manifestations in these patients. Herein, we introduce a alar rim triangular flap, which is indeed a local flap, for the treatment of isolated nasal cleft due to congenital deformities in pediatric patients. The authors conducted a retrospective cohort study including 10 consecutive pediatric patients undergoing this surgery. This alar rim triangular flap including 2 triangles was existing nasal tissue near the cleft. The alar rim defect was covered through local tissue re-arrangement. The authors reviewed the photographs and clinical medical notes of these patients carefully. Self-reported satisfactions of patients (or children's parents) with the scar morphology and correction effect of this procedure were evaluated as well at postoperative every follow-up. All the cases were followed up regularly, and the average follow-up time was 22 months (ranged from 13-38 months). All the nasal clefts were reconstructed successfully. The alar rim triangular flap survived with no flap loss. The wound created by this procedure healed primarily. No alar retraction, nasal obstruction or step-off deformities were observed during postoperative follow-up. There were no patients unsatisfied with the outcome of the scar morphology and correction effect of this operation. The newly designed alar rim triangular flap in this study can be an alternative treatment for correcting isolated congenital nasal cleft with optimal clinical outcome. Level 4.

Reconstruction of Congenital Alar Rim Defects (Tessier Number 1 Cleft) Using Laterally Based Alar Subunit Rotation Advancement Flap.

Alar rim defects develop in most cases as a result of burns, trauma, or tumor excision. Congenital alar rim defects are rare, with an incidence of 1 in 20,000 to 40,000 live births. Tessier classification is the most commonly used classification system for craniofacial defects. Facial clefts involving the nose are categorized as types 0, 1, 2, and 3, whereas cranial clefts with nose lesions are categorized as types 11, 12, 13, and 14. The patterns of nasal clefts are extremely variable, ranging from a simple notch of alar margin to complex craniofacial cleft involving the lip, nose, eyelid, brow, forehead, and underlying bone. This study was conducted at Fayoum University Hospital on 8 male patients who present with congenital alar rim defects (Tessier number 1 cleft). Surgeries were performed under general anesthesia with orotracheal intubation. A full-thickness incision was created along the whole alar subunit, keeping the ala attached only laterally (laterally based alar full-thickness flap). The alar flap was advanced and rotated medially and inferiorly and sutured to the anatomical place in 2 layers, skin and mucosa. Follow-up was done at least 1.5 years postoperatively. The study was conducted on 8 male patients presenting with congenital alar rim defects (Tessier number 1 cleft). The mean age of patients was 14.2 years, and the cleft was unilateral in 4 patients (50%) and bilateral in 4 patients (50%). The patients were assessed both aesthetically and functionally and followed at 1.5 years postoperatively. The aesthetic outcome was assessed according to both patients' and author's evaluations of the scar appearance, alar symmetry, nostril symmetry, and general satisfaction. Laterally based alar subunit rotation advancement flap is a reliable option for reconstruction of congenital alar rim defects (Tessier number 1 cleft) with the advantages of being an easy single-stage procedure with good tissue matching and nostril symmetry and only limitation of extremely wide defects with deficient tissues between the ala and tip.

Surgical Technique for Nasal Cleft Repair.

Congenital nasal cleft is a very rare yet challenging deformity to reconstruct. Atypical craniofacial clefts that involve the nasal ala are designated as number 1 and number 2 under the Tessier classification system. These clefts typically present as notches in the medial one-third of either nasal ala and may be accompanied by a malpositioned cartilaginous framework. Nasal clefts are smaller and far less common than familiar clefts of the lip and palate, but they pose equally challenging reconstructive planning. Our described technique relies on usage of existing nasal tissue near the cleft. Local tissue rearrangement using a laterally based rotational alar flap, a medially based triangular flap, and a nasal wall advancement flap restores normal anatomy and provides an aesthetically pleasing result. Five children with isolated nasal cleft were treated by the senior author (A.M.) between 2010 and 2017. All patients presented with clefts of the soft tissue with no underlying cartilaginous involvement. There were no postoperative complications. Excellent aesthetic outcome was achieved in all patients. Isolated nasal cleft can be properly corrected with the described procedure in a single stage and with optimal result.

Strategy for Nasal Reconstruction in Atypical Facial Clefts.

Summary:It is difficult to put forward a strategy for the treatment of nasal clefts due to the rarity and diversity of anatomical aberrations of these cases contrary to the common nasal affection in cleft lip and palate patients, which differ in severity rather than differing in the morbid anatomy. This simple strategy for correction of these nasal clefts will hopefully help surgeons to achieve better results. In the mean time I intended to describe the morbid anatomy of these cases by choosing examples of each morbid anatomy.

CT and MR findings of the respiratory epithelial adenomatoid hamartoma in the nasal olfactory clefts

Objective To study the CT and MR characteristic features of the respiratory epithelial adenomatoid hamartoma of olfactory clefts. Methods (1)The CT and MRI findings of 29 patients with histologically proved respiratory epithelial adenomatoid hamartoma in the olfactory clefts were retrospectively reviewed. All patients underwent CT and 8 of them underwent MRI. Location, CT and MRI features, and associated findings of the disease were reviewed; (2)The CT findings, olfactory clefts width, total nasal distance, and the ratio of OC to the total nasal distance of the case patients (29 cases) and the control patients (33 patients with sinusitis) were compared to investigate the correlation of the olfactory clefts distance and the incidence of respiratory epithelial adenomatoid hamartoma in olfactory clefts. Results All patients were associated with sinusitis, and 23 had sinonasal polyps, 1 had papilloma. On nonenhanced CT, the OC lesions with the OC widening were isodense to gray matter in all cases, and the lesions caused the adjacent bony expansion and absorption rather than erosion; 15 cases were bilateral diseases and 14 were unilateral; The olfactory clefts width of the case patients and the control patients were (1.03±0.24) cm, (0.71±0.17) cm, respectively. There was statistically significant difference (t=4.963, P 0.05) for the total nasal distance, and was significant difference (t=6.029, P<0.01) in the ratio of OC to the total nasal distance between the two groups. On T1WI, the disease appeared isointense in 6 patients and slightly hypointense in 2 patients compared with gray matter. On T2WI, the lesions revealed heterogeneous isointense in all patients. Regular cribriform pattern was found on MR T2WI and enhanced TlWI. Conclusions The unilateral or bilateral olfactory cleft opacification in chronic sinusitis patients with or without sinonasal polyposis, with involved OC widening and the adjacent bony walls compressed and remodeled may highly suggests the presence of REAH in the OC. The lesions showed inhomogeneous isointense signal on T2WI images, regular cribriform pattern enhancement are typical imaging feature of this entity. Key words: Hamartoma; Respiratory mucosa; Tomography, X-ray computed; Magnetic resonance imaging

Patterns and management of congenital nasal clefts

Objective The aim of the study was to study different patterns of congenital nasal clefts and different methods of their management trying to put forward a road map for their treatment. Background Because of the rarity of atypical facial clefts, little is written with respect to patterns and plans of management. This study will spot light on atypical facial clefts affecting the nose (atypical nasal clefts) with respect to different patterns and plans of management. Patients and methods This was a retrospective and short prospective study that was conducted in the Department of Plastic Surgery, Menoufia University, from 2001 to 2014. The study included 26 patients with atypical nasal clefts. Those patients were classified according to Tessier classification into Tessier type 0, 1, 2, and 3. All patients were evaluated for the type of facial clefts, pattern of nasal involvement, and associated congenital anomalies. In all, 20 of them underwent surgical repair in one or more stages according to complexity of the deformity. The study describes different plans of reconstruction used for management. Results The statistical analysis of data was descriptive and focused on description of different patterns of Tessier type. It showed that the highest incidence was Tessier 0, which included 17 cases found in eight patterns, followed by five cases in Tessier 1 in three patterns, two cases in Tessier type 2 in two different patterns, and two cases in Tessier type 3 in two patterns. The analysis of data showed that the most common associated congenital anomalies were cleft lip and hypertelorism. With respect to management, analysis of data showed the variation in number of operations, timing of first operation, and type of surgical intervention according to complexity and pattern of deformity. Conclusion Atypical nasal clefts can be found in a very wide range of patterns, ranging from simple esthetic deformity to complete agenesis or bifidity of the nose. Management of these cases requires good analysis of the pattern of nasal cleft and we provided a treatment algorithm for each pattern.

Rare nasal cleft in a patient with holoprosencephaly due to a mutation in the ZIC2 gene

Holoprosencephaly (HPE) is a spectrum of midline malformations of the prosencephalon generally reflected in a continuum of midline facial anomalies. Patients with mutation in the ZIC2 gene usually present a normal or mildly dysmorphic face associated with a severe brain malformation. Here we present a rare unilateral nasal cleft (Tessier cleft n. 1) with holoprosencephaly in a patient with a ZIC2 mutation. The male newborn presented with alobar HPE, microcephaly, ocular hypertelorism, upslanting palpebral fissures, a bulky nose with a left paramedian alar cleft. Mutational screening for HPE genes revealed the occurrence of a frameshift mutation in the ZIC2 gene. The mutation was inherited from the father who presented only mild ocular hypotelorism but had an affected child with HPE from his first marriage. The occurrence of oral clefts is common in patients with HPE, but unusual in patients with mutation in the ZIC2 gene. To our knowledge, clefts of the nasal alae have been reported only once or twice in patients with ZIC2 mutations. In documented patients from the literature, only 2% of individuals with described pathogenic mutations in the ZIC2 gene (3/171) presented facial clefts, one of them a nasal cleft, while common oral clefts were observed in 27% of individuals (7/26) described with nonpathogenic ZIC2 mutations or presenting a concomitant mutation in another HPE gene. When compared with the general population, nasal clefts are common in ZIC2 mutations and these mutations must be searched for in undiagnosed cases.

Reconstruction of Nasal Cleft Deformities Using Expanded Forehead Flaps: A Case Series.

Reconstruction of the nasal clefts is a challenging task considering the nasal anatomic complexity and their possible association with craniofacial defects. The reconstruction of these defects needs extensive amounts of soft tissue that warrant the use of forehead flaps. Often presence of cranial defects and low hairline compromise the amount of tissue available for reconstruction warrenting tissue expansion. To evaluate the efficacy of tissue expansion in reconstruction of congenital nasal clefts. 9 patients with congenital nasal clefts involving multiple sub units were taken up for nasal reconstruction with expanded forehead flaps. The average amount of expansion needed was 200ml. The reconstruction was performed in 3 stages. Expanded forehead flaps proved to be best modality for reconstruction providing the skin cover needed for ala, columella and dorsum with minimal scarring at the donor site. Expansion of the forehead flap is a viable option for multiple sub unit reconstruction in congenital nasal cleft deformities.

Isolated cleft of the ala nasi: A report of seven cases

Craniofacial clefts other than cleft lip & palate are reported to be 1.4 to 4.9 per 100,000 live births. Of these, clefts of nose are usually associated with other clefts. Isolated cleft of Ala is rare, 0.7% of all clefts reported by Monasterio. In an analysis of photographic records of 3,500 consecutive patients with craniofacial clefts including cleft lip & palate registered with us between 1985- 2012 which were accessed through our data base, 13 patients with nasal clefts were identified, seven out of which had Isolated cleft of the Ala. All were treated by a rotation flap of the Ala with good results with the longest follow up of 14Yrs. The authors have emphasised the rarity of the condition and presented a simple surgical procedure for correction. In the opinion of the authors this very simple procedure which can be performed by the junior surgeon gives a good long term result in the management of cleft Ala.

Rotation-Transposition Correction of Nasal Deformity in Tessier Number 1 and 2 Clefts

To formulate a standardized procedure for repair of the nasal component of Tessier number 1 and 2 clefts. The procedure was performed from 1998 to 2007 in 13 patients with congenital nasal clefts of different degrees of expression corresponding to Tessier 1 and 2. The patients' ages ranged from 3 months to 28 years. There were 10 male and three female patients. In the absence of any standard published technique for these rare defects, we devised our own method, which we find uniformly applicable to all such cases. We use a composite muco-chondro-cutaneous lateral alar flap to recreate the alar rim. The resulting defect on the lateral nasal wall is then covered with a transposition flap from the dorsum. An alar rim z-plasty was added in cases where notching was evident. In all cases, no problem of flap viability was encountered and all healed well with minimal scarring. The postoperative results were satisfactory and have remained stable over an average 6-month follow-up period. We recommend this technique to be used for the correction of nasal deformity associated with Tessier clefts number 1 and 2. We feel that this technique is relatively simple and easily reproducible.

Prenatal Diagnosis of Partial Trisomy 3p (3p21→pter) and Partial Monosomy 11q (11q23→qter) Associated with Abnormal Sonographic Findings of Holoprosencephaly, Orofacial Clefts, Pyelectasis and a Unilateral Duplex Renal System

Patients with partial trisomy 3p seldom present major dysmorphic features, and holoprosencephaly occurs in only 10% of the cases with partial trisomy 3p. It has been suggested that multiple genetic hits or environmental exposures are required for the clinical expression of holoprosencephaly. At 16 weeks of gestation, prenatal sonography identified a fetus with holoprosencephaly, orofacial clefts, pyelectasis, and a unilateral duplex renal system. Amniocentesis revealed the karyotype of 46,XX,der(11)t(3;11)(p21;q23)pat with partial trisomy 3p (3p21-->pter) and partial monosomy 11q (11q23-->qter). The pregnancy was subsequently terminated. Postnatally, the proband showed hypotelorism, a depressed nasal bridge, orofacial clefts and holoprosencephaly-premaxillary agenesis. The present case provides evidence that partial trisomy 3p/monosomy 11q can be a genetic cause of holoprosencephaly and del(11)(q23-->qter) is associated with a duplex renal system.

Developmental nasal anomalies

Despite the complex embryological development of the nose and surrounding structures, significant developmental nasal anomalies are rare. We therefore present our experience in the management of such anomalies (excluding choanal atresia) over the past 10 years. Fifty cases were surgically treated; two nasopharyngeal cysts, four hairy polyps, two meningoencephalocoeles, seven gliomata, 20 dermoids, two capillary haemangiomata, four fibromas, one fibromyxyoma, one mucocoele, one granuloma, one lipoma, two nasal aplasias, two nasal clefts and one nasal web. All cases presented with nasal obstruction and/or as a nasal mass. Computerized tomography and magnetic resonance imaging aided diagnosis determining the extent of intracranial involvement. Various surgical techniques were used ranging from simple excision to the lateral rhinotomy and Goodman's external rhinoplasty approach. Only five patients (10 per cent) suffered complications; two CSF leaks and three recurrences, all treated successfully.

Nasal clefts.

Our 20-year experience with the treatment of 154 nasal clefts corresponding to numbers 1, 2, 3, and 0 of Tessier's classification is presented. The different varieties of each type of cleft are discussed, and the clinical features and therapeutic problems of each are described. The various operative procedures used in the last two decades are analyzed, and we evaluate the long-term evolution in relation to the aesthetic results and facial growth. Our present criteria for treatment are presented.

Today's status of facial cleft surgery (author's transl)

Cleft lips, jaws and palates numerically represent the major part of facial clefts. These abnormalities are evolutionary and they are characterized by a continually recurring anatomic pattern. The necessity of reconditioning form and function implies the application of surgical therapy. Regarding the one-sided closure or suturing of cleft lips, priority is nowadays given to the angular-type incisions, whereas the linear-type incision is still reserved for the double-sided cheiloschisis. In the following, there will be indicated the approved techniques and methods of suturing the cleft palate and a new method will be discussed related to the reciprocal Z-type plastic operation. In the case of speech-improving operations, excellent results can be achieved by implanting autologous costal cartilages into the posterior wall of the pharynx. Apart from a brief mutioning of transverse and diagonal facial clefts, the provision of nasal clefts with composite grafts is finally recommented.

Rare clefts of the face

Summary A total of seven rare clefts of the face is presented: two median clefts of the lip, two median clefts of the nose, one transverse, and one oblique facial cleft in combination with a cleft of the lip, alveolus and palate, as well as a malformation of the nose associated with cleft of lip, alveolus and palate. Based on the experience gained in the management of these cases, and reports offered in the literature, an attempt has been made to establish a therapeutic regime. For this purpose, three groups were classified: The first group consisted of median clefts of the upper lip, transverse facial clefts, and clefts of lower lip and tongue, in which a final plastic closure is possible. The second group comprises such clefts in which an early plastic repair is absolutely indicated on account of functional reasons, and in which, however, later corrective surgery may be necessary, for example, in oblique facial clefts. In the third group, in which the nasal clefts belong, repair must be deferred at all costs until facial growth is complete.

Rare clefts of the face

A total of seven rare clefts of the face is presented: two median clefts of the lip, two median clefts of the nose, one transverse, and one oblique facial cleft in combination with a cleft of the lip, alveolus and palate, as well as a malformation of the nose associated with cleft of lip, alveolus and palate. Based on the experience gained in the management of these cases, and reports offered in the literature, an attempt has been made to establish a therapeutic regime. For this purpose, three groups were classified: The first group consisted of median clefts of the upper lip, transverse facial clefts, and clefts of lower lip and tongue, in which a final plastic closure is possible. The second group comprises such clefts in which an early plastic repair is absolutely indicated on account of functional reasons, and in which, however, later corrective surgery may be necessary, for example, in oblique facial clefts. In the third group, in which the nasal clefts belong, repair must be deferred at all costs until facial growth is complete.

Fowl Cholera: Immune Response in Turkeys

SUMMARY Emulsified water-in-oil fowl cholera bacterins prepared from 3 strains of formalin-killed Pasteurella multocida induced a high degree of immunity in turkeys against homologous but not heterologous challenge, demonstrating at least 3 immunogenic types of P. multocida associated with fowl cholera. However, both vaccinated and unvaccinated turkeys that survived challenge exposure were immune when re-exposed to different immunogenic strains, demonstrating an immune response to live P. multocida different from that to killed. A one-ml dose of bivalent bacterin was more effective than a 0.5-ml dose in protecting turkeys against experimentally induced fowl cholera. In a comparison of exposure methods, the commonly used method of swabbing nasal clefts of turkeys with a culture of P. multocida was a more severe challenge of immunity than contact exposure challenge with infected birds. In using a challenge culture of low virulence, the intramuscular (IM) route of inoculation was better than either of the other two methods for challenging the immunity of turkeys.

THE EPIDEMIOLOGY OF FOWL CHOLERA

1. Strains of-P. avicida from "spontaneous epidemics" of fowl cholera, when introduced intranasally in fixed doses into specially bred chickens, induced fatal fowl cholera in about 40 per cent but did not survive in the nasal clefts of resistant birds nor spread to normal contacts. 2. Strains of P. avicida from "spontaneous endemics" of fowl cholera, when introduced similarly into chickens, failed to kill but did survive in the nasal clefts of inoculated birds and spread readily to normal contacts. 3. "Laboratory" variants of the epidemic strains of P. avicida failed to kill or survive in the test birds and did not spread to contacts.