CLINICAL STUDY
Congenital Lateral Cleft Palate of Unknown Etiology Ahmet Kahraman, MD, Serdar Yu¨ce, MD,y O¨mer Faruk Koc¸ak, MD,y Yasin Canbaz, MD,y and Dag˘han Is¸ik, MDz Abstract: The term cleft palate (CP) refers to midline defects extending from the prealveolar ark to the uvula, and these defects present with varying degrees. CP may be complete, incomplete, unilateral, bilateral, or submucosal. It is often observed with cleft lip (CL). In various studies, the incidence of isolated CP has been reported as 1.3 to 25.3 per 1000 births.1,2 As a result of deterioration of the anatomical structure of the palate, illnesses such as regurgitation, respiratory tract infections, otitis, and speech disorders may occur. These defects are often observed in the midline and are rarely laterally localized. Cleft palates with lateral localization are outside the natural midline cleft closure line and cause clinical complaints similar to other types of CP. Two cases of laterally localized CP have previously been published in the literature.3,4 The case presented here is the third known case to be reported. Key Words: Abnormal cleft palate, etiology, kongenital, lateral cleft palate (J Craniofac Surg 2015;26: 1332–1333)
CASE
T
he presented case was an 18-month-old male patient who was the fourth child of a family living at an altitude of 1800 m. The patient had an oblique-lateral cleft soft palate extending from the junctional region of the hard palate and soft palate to the left oropharyngeal region (Fig. 1A). A median palatine line (raphe) was observed, and there was no cleft in the uvula. The mucosa of the hard palate had a natural appearance. The patient’s general physical examination did not show any abnormality. The patient’s psychomotor development was normal. There was no abnormality during the gestation of the patient. The mother had no history of drug use, infection, or exposure to x-rays during pregnancy. The patient’s family, including his other siblings, had no history of similar complaints. The patient was operated under general anesthesia. The oral mucosa and nasal mucosa were separated by an incision along the junctional line. A cystic structure filled with a gel-like fluid was observed at the distal third of the cleft region (close to the uvula) From the Mustafa Kemal University Faculty of Medicine, Department of Plastic, Reconstructive and Aesthetic Surgery, Hatay, Turkey; yYuzuncu University Faculty of Medicine, Department of Plastic, Reconstructive and Aesthetic Surgery, Van, Turkey; and zKatip Celebi University Faculty of Medicine, Department of Plastic, Reconstructive and Aesthetic Surgery, Izmir, Turkey. Received May 19, 2014. Accepted for publication September 9, 2014. Address correspondence and reprint requests to Ahmet Kahraman, MD, Medicine School of Uludag University, Department of Plastic and Reconstructive Surgery, Bursa, Turkey. E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001714
1332
FIGURE 1. (A) The patient had an oblique-lateral cleft soft palate. (B) During the first year of follow-up, the cleft region appeared to recover without any problems.
(Fig. 2A). The cyst was excised with its capsule (Fig. 2B). Palate muscle dissection was performed on both sides of the palate. No muscle residue was observed in the left lateral wall of the cleft region. The muscles on the right side of the palate were separated from their insertion. A muscular structure that was on the right side of the cleft was observed to be connected to the muscles beside the intact cleft (Fig. 3A). The mucosa on the nasal side was closed. Then, according to the normal anatomic position, the palate muscles
FIGURE 2. (A) A cystic structure filled with a gel-like fluid was observed at the distal third of the cleft region (arrow). (B) The cyst was excised with its capsule (circumscrıbed). (C) The mucosa on the oral side was repaired, and the line on the side of the cleft was closed.
FIGURE 3. (A) A muscular structure that was on the right side of the cleft was observed to be connected to the muscles beside the intact cleft. (B) According to the normal anatomic position, the palate muscles on the cleft side.
The Journal of Craniofacial Surgery
Volume 26, Number 4, June 2015
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 4, June 2015
on the cleft side were fixed to the soft tissue under the periosteum and to the mucosa of the bone on the side of the cleft using sutures (Fig. 3B). Last, the mucosa on the oral side was repaired, and the line on the side of the cleft was closed (Fig. 2C). During the first year of follow-up, the cleft region appeared to recover without any problems (Fig. 1B).
DISCUSSION The pathological anatomy of the CP was first described by Veau. Uvula bifida is the most common form of CP and affects approximately 2% of the population. The mean incidence of isolated CL is 2.1 cases per 1000 live births among Asians, 1 case per 1000 live births among white people, and 0.41 cases per 1000 live births among black people. The incidence of isolated CP is the same in all races and ranges from 0.45 to 0.50 in 1000 live births. The frequency of the occurrence of isolated CP, CL, and CL/CP is not increased in the relatives of patients. This indicates that isolated CP is genetically a different entity from CL and CL/CP. The secondary palate extends from the incisive foramen to the uvula. It becomes embryologically apparent between the 8th and 12th weeks of gestation due to apoptosis of the median edges of the palatine raphe. Because the head moves from a flexion position into the extension position, the lateral palatine ridges in the vertical position connect with each other at the midline due to the movement of the tongue to the inferior position. If apoptosis is insufficient, cystic residues, referred to as Epstein pearls, filled with keratin can be observed at the midline raphe. These cysts may occur rarely at the level of the soft palate. The downward growth of the nasal septum due to the merging of the medial nasal ridges causes full closure through fusion of the nasal septum with the palate.5 During this period, due to reasons such as micrognathia, mechanical factors that prevent the movement of the tongue may cause CP by preventing the merger of the lateral palatal raphe.6 During the embryological period, the right palatine raphe becomes horizontal to the left palatine raphe; for this reason, left palatine clefts are more common. Many experimental studies have shown that exposure to agents such as hypoxia, alcohol, insulin, tretinoin, corticosteroids, anticonvulsant drugs, phenobarbital, carbon monoxide, salicylates, and cigarettes during pregnancy may induce the formation of CP.7
#
2015 Mutaz B. Habal, MD
Congenital Lateral Cleft Palate
In the presented case, there was no evidence of an abnormal finding during the maternal period. The patient had no micrognathia or any other congenital abnormality. The existence of the fusion in the midline suggests that during the embryological merger, fusion was completed but separation occurred again for unknown reasons. On the other hand, the existence of an inclusion cyst at the cleft line is an indication of an apoptotic disorder, and it provides clues as to why the merger did not take place. As a result of this, why the cleft line did not occur in the midline but instead shifted laterally is not well understood. In previously published cases, a definitive conclusion regarding the etiology was not reached. Although the author Is¸ik the cleft formation to tongue movement disorder, he could not explain why the cleft formed laterally.3 In the presented case, the birth of the patient at an altitude of approximately 1800 m suggests a possible effect of hypoxia. The authors also considered trauma during the birth of the patient as a possible cause of the cleft. However, there was no evidence to confirm this claim. Because of the small number of cases on this subject, the authors could not explain the etiology of the lateral palatal cleft. However, the authors hypothesize that a more detailed evaluation is required during the pre- and postoperative periods in future cases.
REFERENCES 1. Mossey PA, Little J, Munger RG, et al. Cleft lip and palate. Lancet 2009;374:1773–1785 2. Mossey P. Epidemiology underpinning research in the aetiology of orofacial clefts. Orthod Craniofacial Res 2007;10:114–120 3. Isık D, Bulut O, Bekerecıoglu M. Congenital lateral cleft palate and lateral palatal synechiae. Scand J Plast Reconstr Surg Hand Surg 2008;42:325–327 4. Erdogan B, Atabay K, Akoz T, et al. Congenital lateral cleft palate: a new anomaly? Plast Reconstr Surg 1990;86:766–767 5. Pantaloni M, Hollier L. Cleft palate and velopharyngeal incompetence. SRPS 2001;9:1–7 6. Hopper RA, Cuttıng C, Grayson B. Cleft lip and palate. In: Thorne CM, ed. Grabb and Smith’s plastic surgery, 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007;204:201–225 7. Randall R, La Rossa D, et al. Cleft palate. In: Weinzweig J, ed. Plastic surgery secret. Philadelphia, PA: Hanley & Belfus, Inc; 1999:66–67
1333
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Congenital Lateral Cleft Palate of Unknown Etiology Ahmet Kahraman, MD, Serdar Yu¨ce, MD,y O¨mer Faruk Koc¸ak, MD,y Yasin Canbaz, MD,y and Dag˘han Is¸ik, MDz Abstract: The term cleft palate (CP) refers to midline defects extending from the prealveolar ark to the uvula, and these defects present with varying degrees. CP may be complete, incomplete, unilateral, bilateral, or submucosal. It is often observed with cleft lip (CL). In various studies, the incidence of isolated CP has been reported as 1.3 to 25.3 per 1000 births.1,2 As a result of deterioration of the anatomical structure of the palate, illnesses such as regurgitation, respiratory tract infections, otitis, and speech disorders may occur. These defects are often observed in the midline and are rarely laterally localized. Cleft palates with lateral localization are outside the natural midline cleft closure line and cause clinical complaints similar to other types of CP. Two cases of laterally localized CP have previously been published in the literature.3,4 The case presented here is the third known case to be reported. Key Words: Abnormal cleft palate, etiology, kongenital, lateral cleft palate (J Craniofac Surg 2015;26: 1332–1333)
CASE
T
he presented case was an 18-month-old male patient who was the fourth child of a family living at an altitude of 1800 m. The patient had an oblique-lateral cleft soft palate extending from the junctional region of the hard palate and soft palate to the left oropharyngeal region (Fig. 1A). A median palatine line (raphe) was observed, and there was no cleft in the uvula. The mucosa of the hard palate had a natural appearance. The patient’s general physical examination did not show any abnormality. The patient’s psychomotor development was normal. There was no abnormality during the gestation of the patient. The mother had no history of drug use, infection, or exposure to x-rays during pregnancy. The patient’s family, including his other siblings, had no history of similar complaints. The patient was operated under general anesthesia. The oral mucosa and nasal mucosa were separated by an incision along the junctional line. A cystic structure filled with a gel-like fluid was observed at the distal third of the cleft region (close to the uvula) From the Mustafa Kemal University Faculty of Medicine, Department of Plastic, Reconstructive and Aesthetic Surgery, Hatay, Turkey; yYuzuncu University Faculty of Medicine, Department of Plastic, Reconstructive and Aesthetic Surgery, Van, Turkey; and zKatip Celebi University Faculty of Medicine, Department of Plastic, Reconstructive and Aesthetic Surgery, Izmir, Turkey. Received May 19, 2014. Accepted for publication September 9, 2014. Address correspondence and reprint requests to Ahmet Kahraman, MD, Medicine School of Uludag University, Department of Plastic and Reconstructive Surgery, Bursa, Turkey. E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001714
1332
FIGURE 1. (A) The patient had an oblique-lateral cleft soft palate. (B) During the first year of follow-up, the cleft region appeared to recover without any problems.
(Fig. 2A). The cyst was excised with its capsule (Fig. 2B). Palate muscle dissection was performed on both sides of the palate. No muscle residue was observed in the left lateral wall of the cleft region. The muscles on the right side of the palate were separated from their insertion. A muscular structure that was on the right side of the cleft was observed to be connected to the muscles beside the intact cleft (Fig. 3A). The mucosa on the nasal side was closed. Then, according to the normal anatomic position, the palate muscles
FIGURE 2. (A) A cystic structure filled with a gel-like fluid was observed at the distal third of the cleft region (arrow). (B) The cyst was excised with its capsule (circumscrıbed). (C) The mucosa on the oral side was repaired, and the line on the side of the cleft was closed.
FIGURE 3. (A) A muscular structure that was on the right side of the cleft was observed to be connected to the muscles beside the intact cleft. (B) According to the normal anatomic position, the palate muscles on the cleft side.
The Journal of Craniofacial Surgery
Volume 26, Number 4, June 2015
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 4, June 2015
on the cleft side were fixed to the soft tissue under the periosteum and to the mucosa of the bone on the side of the cleft using sutures (Fig. 3B). Last, the mucosa on the oral side was repaired, and the line on the side of the cleft was closed (Fig. 2C). During the first year of follow-up, the cleft region appeared to recover without any problems (Fig. 1B).
DISCUSSION The pathological anatomy of the CP was first described by Veau. Uvula bifida is the most common form of CP and affects approximately 2% of the population. The mean incidence of isolated CL is 2.1 cases per 1000 live births among Asians, 1 case per 1000 live births among white people, and 0.41 cases per 1000 live births among black people. The incidence of isolated CP is the same in all races and ranges from 0.45 to 0.50 in 1000 live births. The frequency of the occurrence of isolated CP, CL, and CL/CP is not increased in the relatives of patients. This indicates that isolated CP is genetically a different entity from CL and CL/CP. The secondary palate extends from the incisive foramen to the uvula. It becomes embryologically apparent between the 8th and 12th weeks of gestation due to apoptosis of the median edges of the palatine raphe. Because the head moves from a flexion position into the extension position, the lateral palatine ridges in the vertical position connect with each other at the midline due to the movement of the tongue to the inferior position. If apoptosis is insufficient, cystic residues, referred to as Epstein pearls, filled with keratin can be observed at the midline raphe. These cysts may occur rarely at the level of the soft palate. The downward growth of the nasal septum due to the merging of the medial nasal ridges causes full closure through fusion of the nasal septum with the palate.5 During this period, due to reasons such as micrognathia, mechanical factors that prevent the movement of the tongue may cause CP by preventing the merger of the lateral palatal raphe.6 During the embryological period, the right palatine raphe becomes horizontal to the left palatine raphe; for this reason, left palatine clefts are more common. Many experimental studies have shown that exposure to agents such as hypoxia, alcohol, insulin, tretinoin, corticosteroids, anticonvulsant drugs, phenobarbital, carbon monoxide, salicylates, and cigarettes during pregnancy may induce the formation of CP.7
#
2015 Mutaz B. Habal, MD
Congenital Lateral Cleft Palate
In the presented case, there was no evidence of an abnormal finding during the maternal period. The patient had no micrognathia or any other congenital abnormality. The existence of the fusion in the midline suggests that during the embryological merger, fusion was completed but separation occurred again for unknown reasons. On the other hand, the existence of an inclusion cyst at the cleft line is an indication of an apoptotic disorder, and it provides clues as to why the merger did not take place. As a result of this, why the cleft line did not occur in the midline but instead shifted laterally is not well understood. In previously published cases, a definitive conclusion regarding the etiology was not reached. Although the author Is¸ik the cleft formation to tongue movement disorder, he could not explain why the cleft formed laterally.3 In the presented case, the birth of the patient at an altitude of approximately 1800 m suggests a possible effect of hypoxia. The authors also considered trauma during the birth of the patient as a possible cause of the cleft. However, there was no evidence to confirm this claim. Because of the small number of cases on this subject, the authors could not explain the etiology of the lateral palatal cleft. However, the authors hypothesize that a more detailed evaluation is required during the pre- and postoperative periods in future cases.
REFERENCES 1. Mossey PA, Little J, Munger RG, et al. Cleft lip and palate. Lancet 2009;374:1773–1785 2. Mossey P. Epidemiology underpinning research in the aetiology of orofacial clefts. Orthod Craniofacial Res 2007;10:114–120 3. Isık D, Bulut O, Bekerecıoglu M. Congenital lateral cleft palate and lateral palatal synechiae. Scand J Plast Reconstr Surg Hand Surg 2008;42:325–327 4. Erdogan B, Atabay K, Akoz T, et al. Congenital lateral cleft palate: a new anomaly? Plast Reconstr Surg 1990;86:766–767 5. Pantaloni M, Hollier L. Cleft palate and velopharyngeal incompetence. SRPS 2001;9:1–7 6. Hopper RA, Cuttıng C, Grayson B. Cleft lip and palate. In: Thorne CM, ed. Grabb and Smith’s plastic surgery, 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007;204:201–225 7. Randall R, La Rossa D, et al. Cleft palate. In: Weinzweig J, ed. Plastic surgery secret. Philadelphia, PA: Hanley & Belfus, Inc; 1999:66–67
1333
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
