Nasal airflow and olfactory function after the repair of cleft palate (with and without cleft lip).

OBJECTIVE: The objective of this study was to examine nasal airflow and olfactory functions in patients with repaired cleft palate compared with matching normal controls. STUDY DESIGN: The all-cleft group consisted of 25 patients with hard palate cleft comprising 15 patients with unilateral cleft palate and lip (UCLP); 2 with CP but no cleft lip (UCLP subgroup) and 8 patients with bilateral cleft lip and palate (BCLP subgroup). All had had surgical correction of the palate in infancy. The control group consisted of 20 nonaffected orthodontic patients. The median age of both groups was 14 years. The tests included the following: (1) nasal airflow measured by anterior rhinomanometry, (2) smell threshold for isoamyl-acetate determined using a 3-way forced choice method, (3) a self-administered questionnaire regarding the subjective perception of smell sense function, and (4) orthonasal and retronasal smell identification (correct/incorrect) and hedonics using visual analog scale (VAS). RESULTS: The respective test results follow. (1) When compared with the control group, the total airflow in the UCLP subgroup was significantly lower especially on the affected side; while in the BCLP subgroup it was lower than in the control group bilaterally. No significant difference was found between the cleft side of UCLP and BCLP subgroups. (2) The smell threshold of the UCLP subgroup was significantly higher than that of the control group and BCLP subgroup. No significant differences were found between right and left nostrils within the BCLP patients and between them and the control group. (3) No difference was found between the groups regarding the subjective perception of smell. (4) No significant differences were found between the UCLP and BCLP subgroups and between the all-cleft group and the control group, except for one item, regarding orthonasal and retronasal smell identification and hedonics. CONCLUSION: Although nasal airflow is significantly lower and the smell threshold higher on the cleft side, the day-to-day function of the sense of smell of cleft patients is similar to that of normal controls.     

Left atrioventricular valve repair technique in partial atrioventricular septal defects

BACKGROUND: The aim of our study was to evaluate the effect of chordal transfer around the cleft on left atrioventricular valve competence in the late postoperative period. METHODS: Forty-four adult patients underwent surgical correction of partial atrioventricular septal defect between 1983 and 1997. Fenestration was found in 8 patients (18.2%) and cleft, in 35 (79.5%). There was no chordal support of the free edges of the left superior and left inferior leaflets around the cleft in 18 patients. Two chordae were mobilized from the left lateral leaflet and reimplanted into the tip of the left superior and left inferior leaflets around the cleft. RESULTS: At 5 years postoperatively, left atrioventricular valve insufficiency was severe in 5 patients and moderate in 11 patients who had had cleft closure alone. In contrast, severe valvular insufficiency was present in only 1 patient in the group with chordal transfer (p < 0.05). Reoperation was done in 5 patients with isolated cleft closure. Left AV valve replacement was performed in 1 patient. CONCLUSIONS: Chordal transfer plus cleft closure with interrupted sutures significantly reduces early and late left atrioventricular valve incompetence and also decreases the rate of reoperation.     

Facial morphology and occlusion at the stage of early mixed dentition in cleft lip and palate patients treated with delayed closure of the hard palate

Cephalometric radiographs and dental study casts were analyzed in a group of 23 seven-year-old cleft lip and palate patients, 16 with unilateral and 7 with bilateral cleft. The patients' primary surgical procedures had been completed except for closure of the cleft in the hard palate. For comparison, similar records from another group of patients, 18 with unilateral and 8 with bilateral cleft lip and palate, were studied. In these cases the cleft of the hard palate had been repaired in infancy, using a vomer flap procedure. The results indicated that midfacial growth and dental occlusion of the unilateral cleft sample was significantly better in patients whose closure of the hard palatal cleft had been delayed to the stage of mixed dentition than where repair had been performed with a vomer flap in infancy. No differences were found, however, between similar subgroups with bilateral cleft lip and palate.     

Bilateral oblique facial cleft

An extremely rare case of bilateral oblique facial cleft is presented. This should be classified as a bilateral oroocular Type I cleft according to Boo-Chai's classification and a bilateral No. 4 cleft according to Tessier's classification. Except for this deformity, the patient was normal and healthy. Corrective surgical procedures were carried out between the ages of 4 months and 18 months to repair the left and right clefts, remove an abscess caused by the remnant of the lacrimal canal, and repair the cleft palate. The patient is now making a good recovery. Bone graftings or craniofacial osteotomies may be recommended at a later age.     

Repair of the Left AV Valve in Atrioventricular Septal Defect in Adults

A bstract Background : This study examined the septal cleft and septal commissure of the left atrioventricular (AV) valve, which are two different anatomical structures. Methods : We presented 36 cases of adult partial atrioventricular septal defect. A distinction was made between patients based on the anatomy of the anterior leaflet of the left AV valve. The left AV valve appeared to be normal or to have minimal radial openings from the free edge of the anterior leaflet of the left AV valve in 10 patients (28%). There was a septal commissure structure in 8 (22%), and a septal cleft structure in 18 (50%) patients. In the commissure type anatomy, leaflet coaptation was usually adequate and no or mild degree of left AV regurgitation existed preoperatively. Cleft type structure usually was associated with some degree of left AV regurgitation. Attempts were made to close the septal clefts and leave the septal commissures unsutured during the repair of the partial AV septal defects. Results : We have not found any increase of left AV regurgitation in patients with commissures during the follow-up period. Closure of the cleft successfully eliminated regurgitation. Long-term results for septal cleft and septal commissure after repair of partial AV septal defect were excellent with survival of 100% and freedom from reoperation of 100% at mean 6.5 years. Conclusions : Septal cleft and septal commissure should be considered two different structures. Repairing procedures for left AV valve abnormalities associated with partial AV septal defect should only be done in patients who have cleft type of leaflet structure.     

Unrepaired Clefts in the Hard Palate: Speech Deficits At the Ages of 5 and 7 Years and Their Relationship to Size of the Cleft

The purpose of the study was to investigate the relationship between the size of oronasal openings in the hard palate and speech deficits. Audiotape recordings and plaster casts were taken according to standard procedures at 5 and 7 years of age from 22 consecutive children born with complete unilateral cleft lip and palate treated at Sahlgrenska University Hospital, Göteborg, Sweden. The soft palate had been repaired before the age of 12 months, whereas the cleft in the hard palate was left unrepaired, to be closed later. Perceptual judgements of nine speech variables at 5 and 7 years of age were correlated with measures of the area of the residual cleft in the hard palate. "Retracted oral articulation" (to velar place) found in nine of the 22 children correlated significantly with the area of the cleft at the age of 5 years but not later. The establishment of this particular speech error seems to be related to the size of an oronasal opening.     

Atelectasis after induction of general anesthesia in an infant with cleft palate

We report an infant with cleft palate who developed atelectasis as a result of aspiration of food residue after induction of general anesthesia. A 12-month-old girl with cleft palate was scheduled for palatoplasty. The trachea was intubated after 6.5-hour fasting. Breath sounds were not audible on the left. Chest X-ray demonstrated complete atelectasis of the left lung. Bronchoscopy revealed that the left primary bronchus was blocked by a white stick. Pathological examination showed that the white stick consisted of milk residue with abundant candida species. It was speculated that the milk residue accumulated in the cleft palate had been aspirated during induction of general anesthesia.     

Unrepaired clefts in the hard palate: speech deficits at the ages of 5 and 7 years and their relationship to size of the cleft.

The purpose of the study was to investigate the relationship between the size of oronasal openings in the hard palate and speech deficits. Audiotape recordings and plaster casts were taken according to standard procedures at 5 and 7 years of age from 22 consecutive children born with complete unilateral cleft lip and palate treated at Sahlgrenska University Hospital, G?teborg, Sweden. The soft palate had been repaired before the age of 12 months, whereas the cleft in the hard palate was left unrepaired, to be closed later. Perceptual judgements of nine speech variables at 5 and 7 years of age were correlated with measures of the area of the residual cleft in the hard palate. "Retracted oral articulation" (to velar place) found in nine of the 22 children correlated significantly with the area of the cleft at the age of 5 years but not later. The establishment of this particular speech error seems to be related to the size of an oronasal opening.     

A case of an atypical cleft

A 16-month-old male infant with an atypical cleft palate is presented. He had an incomplete cleft palate, on the left side, the soft palate was shorter than the right. In addition it was swollen. Preoperative examination revealed muscular dysgenesis and mucosal hyperplasia on the left side. An intravelar veloplasty and V-Y pushback procedure was performed with a satisfactory result.     

Transverse facial cleft: A series of 17 cases

Introduction:

Transverse facial cleft (Tessier type 7) or congenital macrostomia is a rare congenital anomaly seldom occurring alone and is frequently associated with deformities of the structures developing from the first and second branchial arches. The reported incidence of No. 7 cleft varies from 1 in 60,000 to 1 in 300,000 live births.

Material and Methods:

Seventeen patients of transeverse facial cleft who presented to us in last 5 years were included in the study. Their history regarding familial and environmental predispositions was recorded. The cases were analysed on basis of sex, laterality, severity, associated anomalies and were graded according to severity. They were operated by z plasty technique and were followed up for 2 years to look for effectiveness of the technique and its complications.

Result:

Out of the seventeen patients of transverse cleft, none had familial predilection or any environmental etiology like antenatal radiological exposure or intake of drugs of teratogenic potential. Most of the patients (9/17) were associated with hemifacial microsomia and 1 patient was associated with Treacher Colin''s Syndrome. Out of the 6 cases of Grade I clefts, 4 were isolated transverse clefts and of the 10 patients of Grade II clefts, 7 were associated with hemifacial microsomia. We encountered only one case of Grade III Transverse Cleft which was not only associated with hemifacial microsomia but also had cardiac anomaly. Out of the 17 cases, 15 were operated and in most of them the outcome was satisfactory.KEY WORDS: Macrostomia, No. 7 cleft, transverse facial cleft     

唇腭裂1386例发病特点回顾性分析

目的：通过对唇腭裂患者临床资料进行回顾性统计分析,研究该病的发病特点,为唇腭裂的预防及治疗提供临床参考。方法：统计分析2007年1月～2010年1月手术治疗的1386例唇腭裂患者,分别从患者诊断、性别、年龄、出生地,唇腭裂裂型分布情况等方面进行回顾性调查。结果：本组病例中,单纯唇裂356例（25．69％）,唇裂合并腭裂580例（41．84％）,单纯腭裂450例（32．47％）;男809例,女577例,男：女=1．40：1;单侧唇裂伴或不伴腭裂明显多于双侧者,两者之比为5．67：1,其中左侧多于右侧（1．82：1）。结论：唇腭裂发病以唇裂合并腭裂居多,男性发病多于女性,在腭裂患者中女性发病高于男性;单侧发病多于双侧,左侧多于右侧。     

Fourteen-year follow-up of a Tessier number 4 cleft

A 3-month-old girl of Japanese parents presented with a left oblique facial cleft (Tessier number 4 cleft). It extended from the area lateral to the Cupids bow, through the cheek on the lateral side of the ala nasi, to the medial side of the lacrimal punctum of the lower eyelid. As a result, the left lower eyelid was split, resulting in marked ectropion. There was another cleft present on the medial side of the left upper eyelid. The left ala nasi was normal morphologically but was raised upwards. The cleft extended into the secondary palate. The nasal septum was preserved, and left choanal atresia was present. CT examination revealed hypoplasia of the left maxillary bone and sinus. A total of seven operations was performed during the 14-year follow-up period; the end result was satisfactory. Artificial bone (apatite ceramics) was implanted on the anterior wall of the maxilla, when the child was aged 6 years, and to the orbital floor when she was 13. CT examination (at 13 years) revealed that the artificial bone implanted at age 6 was correctly oriented and had fused to the anterior wall of the maxilla and zygoma. Resorption had been minimal.     

Left or right hand dominance in children with cleft lip and palate.

The relationship between unilateral cleft lip, bilateral cleft lip, palatal clefting and left-right hand dominance was studied in 337 patients with cleft lip and/or palate aged between 3 and 14 years. There was no statistically significant difference in the laterality of handedness between different types of cleft nor between unilateral left and right sided cleft lip with or without cleft palate.     

Repair of left atrioventricular valve cleft defects with patch augmentation

Operations for left atrioventricular valve insufficiency after repair of an atrioventricular septal defect can be challenging. Repair techniques largely depend on closure of the residual cleft in the anterior leaflet in conjunction with a posterior annuloplasty. Profound deficiencies in anterior leaflet tissue can make primary cleft closure impractical. A simplified technique, in which cleft closure is supported by triangular-shaped patch material, is presented along with results in 13 patients.     

Clinical and epidemiologic studies of nonsyndromic cleft lip and palate in china: analysis of 4268 cases

The aim of our study was to review clinical and epidemiologic characteristic of the nonsyndromic cleft lip and cleft palate over a period of 10 years at West China Stomatological Hospital, Sichuan University. Four thousand two hundred sixty-eight nonsyndromic cleft lip and cleft palate cases were retrospectively analyzed according to the following variables: general information, cleft type, maternal age, familial history, as well as associated malformations, et al. In these cases, 1075 of which were cleft lip, 1985 were cleft lip with cleft palate; 1208 were isolated cleft palate. There were more men than women in the cleft lip and cleft lip with cleft palate group, while there were more women than men in the isolated cleft palate group. There were significantly more unilateral cleft lip cases than cleft lip on both sides; the cases of cleft lip on the left side were more than that of cleft lip on the right side. The cases with inherited history accounted for 6.68% of all the cases. Most infection cases occurred among the deleterious factors and the complications experienced during the first 3 months after the pregnancy. In all of the 14 twin cases, only 1 of the babies was affected. There were totally 152 cases with other associated malformations. Patients with cleft lip born in November to January were less than those born in the other three quarters of the year. Patients of A blood group composed a higher proportion than the control group. Our data may provide references for appropriate resource use, cleft lip and cleft palate prevention programs, and counseling programs with China-specific data.     

Management of the left atrioventricular valve in the repair of complete atrioventricular septal defects.

Left atrioventricular valve regurgitation in atrioventricular canal defects is usually due to malalignment of the edges of the cleft or to annular dilatation. Intraoperative assessment and correction of left atrioventricular valve incompetence is critical for successful outcome in the surgical management of complete atrioventricular canal defects. Although some have elected not to suture the cleft in the setting of minimal incompetence, we have found that this often results in significant left atrioventricular valve insufficiency, necessitating reoperation. From January 1982 through December 1990, 105 patients with complete atrioventricular canal underwent definitive repair. Repair was performed with a single pericardial patch technique in 86 patients (82%). Intraoperative assessment of left atrioventricular valve competence was performed in all cases. Ninety-six patients (91%) required suturing of the cleft and 63 (60%) required annuloplasty to establish satisfactory competence of the left atrioventricular valve. The overall early mortality rate was 10.5% (11/105 patients). From 1986 to 1990, the early mortality rate decreased to 7.7% (6/78 patients). In a mean follow-up of 39 months (range 1 to 106 months), late survival was 96% (90/94 operative or early survivors). Reoperation was performed on eleven (11.5%) patients; six (6.3%) for failure of the atrioventricular valve repair, three for patch dehiscence, and two for residual ventricular septal defects. These data demonstrate that routine approximation of the cleft and aggressive use of left atrioventricular valve annuloplasty is safe and results in an excellent outcome with a low incidence of reoperation for failure of left atrioventricular valve repair.     

A case of Rathke's cleft cyst in association with a ruptured aneurysm of the anterior cerebral artery mimicking pituitary apoplexy

We present a rare case of a Rathke's cleft cyst in association with a ruptured aneurysm of the anterior cerebral artery (ACA). A 44-year-old man suffered from sudden onset of headache. Initial computed tomographic (CT) scan revealed a high-density mass lesion in the suprasellar region and a diffuse high-density area in the basal cistern. Using emergent magnetic resonance imaging (MRI), we found a cyst showed homogeneously high and iso-intensity on T1 and T2-weighted image, respectively. The cyst showed no enhancement of the cyst wall, but on Gd-DTPA, it was shown to compress the normal pituitary gland. Angiography showed an aneurysm at the A1 portion of the left ACA. Based on/these findings, we were able to diagnose Rathke's cleft cyst and a ruptured aneurysm. An operation was performed through the interhemispheric approach. The suprasellar cystic mass compressed the optic nerves and chiasm upward. Neck clipping of the aneurysm and opening of the cyst were performed. We confirmed the cause of the subarachnoid hemorrhage as being a ruptured aneurysm at the A1 portion of the left ACA. Histological diagnosis was Rathke's cleft cyst. Postoperative course was uneventful. There has been only one reported case of Rathke's cleft cyst in association with a ruptured aneurysm. When we encounter a case presenting subarachnoid hemorrhage with suprasellar mass and intracerebral aneurysm, we must discriminate between ruptured aneurysm and pituitary apoplexy in the acute stage as the cause of the subarachnoid hemorrhage. If the mass is Rathke's cleft cyst, we speculate that the cause of the subarachnoid hemorrhage is a ruptured aneurysm, because there are no reports of Rathke's cleft cyst with subarachnoid hemorrhage.     

33例颅面裂Tessier分类诊断与治疗体会

目的介绍颅面裂的Tessier分类诊断与手术治疗体会。方法按Tessier分类法对33例颅面裂患者进行分类，并根据畸形类型及轻重程度制定手术方案；共施术29例，手术方法包括单纯眶内侧壁截骨与鼻翼软骨修整、局部皮瓣转移、内眦固定、真皮移植、骨移植、组织代用品置入、吻合血管的游离组织瓣移植等。结果33例中属0号颅面裂1例、0—14号颅面裂3例、1号颅面裂1例、2-12号颅面裂2例、3号颅面裂3例、4号颅面裂1例、5／6号复合颅面裂1例、7号颅面裂18例、6／7／8号复合颅面裂2例、11号颅面裂1例；29例接受手术的患者，术后面部形态明显改善，无并发症发生。结论①Tessier颅面裂分类法有助于发现与主诉体征相伴的其它颅面畸形，对作出全面正确的诊断及制定手术方案具有重要的指导作用。②在Tessier颅面裂中以7号颅面裂较为常见。③Tessier颅面裂有不同的类型，且每一类型的颅面裂又有不同的严重程度，而且不同类型及不同严重程度的颅面裂又可见于同一患者，需根据患者具体情况制定个性化的手术方案。     

先天性颅面裂隙畸形的诊断和分类

目的 对先天性颅面裂隙畸形进行分类和诊断，有利于区分畸形的部位和程度，为手术修复提供指导。方法 用Tessier颅面裂分类的基本方法，对81例先天性颅面裂隙畸形进行分析，再根据畸形发生的部位、性状、CT扫描等临床检查结果，按照受累的范围，选用S(皮肤)、T(皮下组织)、O(颅面骨)等字母组合表示畸形部位和性状，选用阿拉伯数字的大小表示畸形的严重程度，作为Tessier分类的补充。结果 以STO分类法分析81例病例，眶下方畸形以Ⅲ、Ⅳ号裂多见(24．70％)，眶上方畸形以IX、X号裂多见(38．27％)，各种组织的受累程度并未表现出一定的规律性。结论STO分类法是Tessier分类法的补充，可为颅面裂隙畸形的修复提供依据。     

Left atrioventricular valve regurgitation after repair of incomplete atrioventricular septal defect

BACKGROUND: Excellent surgical results have been reported for repair of incomplete atrioventricular septal defect; however, left atrioventricular valve regurgitation (ltAVVR) is a major cause of late morbidity. We reviewed our entire experience with incomplete atrioventricular septal defect in order to investigate long-term results of ltAVVR after repair and determine the factors influencing the progression of ltAVVR in late follow-up. METHODS: Between 1983 and 2002, 61 patients underwent surgical repair of incomplete atrioventricular septal defect, including 7 patients with intermediate forms. The age of operation ranged from 1 month to 62 years old (median 5.3 years old). Thirteen patients were less than 2 years old, including 7 infants, while there were 15 adult patients. All patients underwent patch closure of the ostium primum defect. Before 1995, the cleft was left open in 7 patients and partial closure of the cleft was done in 41 patients, whereas complete closure of the cleft was performed in 9 patients since 1996. Preoperative and postoperative ltAVVR at hospital discharge and late follow-up were graded 0-IV by echographic evaluation. RESULTS: There was 1 early death and 4 late deaths with a 91% 10-year actuarial survival rate. Preoperative ltAVVR grade was I in 25 patients, II in 31 patients, III in 4 patients, and IV in 1 patient. Postoperatively, ltAVVR deteriorated in 3 patients. Left AVVR decreased in 21 patients, whereas in 37 patients it remained the same at hospital discharge. Consequently, ltAVVR remained grade II in 18 patients, grade III in 2, and there was no patient with grade IV. During the long-term follow-up, 24 patients were noted to have increased ltAVVR, including grade III in 8 patients and grade IV in 4. Reoperations for ltAVVR were required in 5 patients (8.3% of hospital survivors); valve replacement in 3 patients and valve repair in 2. Actuarial freedom from reoperation for ltAVVR was 91% at 10 years, whereas actuarial freedom from postoperative ltAVVR grade III or more was 89% at 5 years and 78% at 10 years. Multivariate analysis indicated that postoperative ltAVVR grade II or more at hospital discharge (p = 0.0032, odds ratio = 7.41, 95%CI: 1.95-28.10) was the only independent variable related to late ltAVVR, whereas age at operation, preoperative grade of ltAVVR, and the method of cleft repair were not significant risk factors. CONCLUSIONS: Left AVVR is still a significant risk in long-term follow-up. Because the postoperative grade of ltAVVR is the only independent risk factor for late ltAVVR, more efforts should be focused on left atrioventricular valve repair so as to minimize residual regurgitation, even mild regurgitation.