Complete bilateral facial cleft (Tessier 4) with corneal staphyloma: a rare association

The oro-ocular cleft no. 4 according to the Tessier classification is one of the rarest facial cleft, and to this day, few cases have been reported in the literature. We describe the case of a 9-month-old girl with a complete bilateral facial cleft. On the right cornea protruded a hard lesion, a corneal staphyloma. We describe the 3 primary surgical steps used to restore the possibility of satisfactory feeding, to promote language acquisition, and to protect vision in the nonaffected eye. The psychological and social aspects of severe facial deformities in developing countries are also tackled.     

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。     

Incomplete midline cleft of the lower lip

Summary Midline clefts of the lower lip and mandible, and incomplete midline clefts of the lower lip are uncommon anomalies, with the latter being very rare. A case of incomplete midline lower lip cleft with a corresponding orbicularis oris muscle defect is present.     

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.     

Atypical first branchial cleft fistula: A case report

IntroductionFirst branchial cleft anomalies (FBCA) are rare. They have an estimated incidence of 1 in 100,000. Type I are those that embryologically duplicate the membrane (cutaneous) external auditory canal.The aim of this case is to describe an unusual path of a type II first branchial cleft fistula tract in a 3 years old child and its surgical management in the academic hospital of Casablanca.Case presentationThis case is about a 3 year old girl who presented to the Ear Nose Throat (ENT) consultation for recurrent right lateral cervical infection. Clinical examination found an unsightly scar attached to an orifice giving pus located near the right mandibular angle suggesting type II first branchial cleft anomaly.Surgical excision was performed under general anesthesia by the superficial parotidectomy approach, the facial nerve was identified and preserved. The fistula cord was dissected and followed, it went under the facial nerve and the parorid gland to end under the digastric muscle where we tied it up. The postoperative check-up did not show any complications. The follow-up period was 12 months; the clinical examination did not find any sign of recurrence.ConclusionFirst branchial cleft fistula are rare and can be in form of cyst or fistula. Its management is surgical excision keeping the tract cyst of the fistula intact with facial nerve preservation.     

Midline cleft of the lower lip associated with Robinow syndrome

Robinow syndrome (also named “fetal face syndrome”) includes a series of anomalies including mesomelic brachymelia, bifid terminal phalanges of the hands and feet, abnormalities of vertebrae and ribs, and hypoplastic external genitalia. A midline cleft of the lower lip and mandible is an extremely rare maxillofacial deformity. Seventy cases have so far been described to our knowledge. We report a patient with Robinow syndrome and midline cleft of the lower lip and mandible and describe the reconstruction of these anomalies. We propose that this anomaly should be added to the range of malformations associated with the syndrome.     

Bilateral cleft lip nasal deformity

Bilateral cleft lip nose deformity is a multi-factorial and complex deformity which tends to aggravate with growth of the child, if not attended surgically. The goals of primary bilateral cleft lip nose surgery are, closure of the nasal floor and sill, lengthening of the columella, repositioning of the alar base, achieving nasal tip projection, repositioning of the lower lateral cartilages, and reorienting the nares from horizontal to oblique position. The multiplicity of procedures in the literature for correction of this deformity alludes to the fact that no single procedure is entirely effective. The timing for surgical intervention and its extent varies considerably. Early surgery on cartilage may adversely affect growth and development; at the same time, allowing the cartilage to grow in an abnormal position and contributing to aggravation of deformity.Some surgeons advocate correction of deformity at an early age. However, others like the cartilages to grow and mature before going in for surgery. With peer pressure also becoming an important consideration during the teens, the current trend is towards early intervention.There is no unanimity in the extent of nasal dissection to be done at the time of primary lip repair. While many perform limited nasal dissection for the fear of growth retardation, others opt for full cartilage correction at the time of primary surgery itself. The value of naso-alveolar moulding (NAM) too is not universally accepted and has now more opponents than proponents. Also most centres in the developing world have neither the personnel nor the facilities for the same. The secondary cleft nasal deformity is variable and is affected by the extent of the original abnormality, any prior surgeries performed and alteration due to nasal growth.This article reviews the currently popular methods for correction of nasal deformity associated with bilateral cleft lip, it''s management both at the time of cleft lip repair and also secondarily, at a later date. It also discusses the practices followed at our centre.     

Analysis of anatomic variations in cleft hands

PURPOSE: This study presents an overview of the clinical and x-ray findings observed in 54 cleft hands of 31 patients. The emphasis was on a detailed analysis of typical malformation-associated x-ray patterns such as aplasia and synostosis, with findings arranged as a function of cleft location and of the Manske and Halikis classification. METHODS: The charts and radiographs of 31 patients with 54 cleft hands were reviewed retrospectively and compared with data reported in the literature. Important clinical and x-ray findings as well as the typical morphology of cleft hands were analyzed. Cleft hands were differentiated according to their location and according to the Manske and Halikis classification. In the different locations the deformities were arranged in teratologic sequences. RESULTS: In cleft hands syndactylies were seen in 30 of the 54 hands, most commonly between the ring and the small finger. In 3 hands polydactylies were noted. Analysis of the x-ray morphology showed 2 typical patterns: aplasia and synostoses. Location-specific teratologic sequences showed that radial cleft hands were bilateral more frequently and were associated significantly more frequently with cleft feet. Aplasias predominated in radial cleft hands, whereas synostoses were more common in central cleft hands. In terms of the Manske and Halikis classification the unilateral cleft hands often corresponded to type I whereas bilateral cleft hands with cleft feet mainly were type IV and type V deformities. Cleft hands with synostoses often were seen in types I to III whereas cleft hands with aplasias were classified most frequently as type V. CONCLUSIONS: Analysis of the patients' clinical data and x-rays showed differences between radial and central cleft hands, as well as between the different Manske and Halikis types.     

Simultaneous branchial cleft and thyroid disorders may present a management challenge

BACKGROUND: Cysts, sinuses or abscesses arising from second, third or fourth branchial cleft remnants may lie either within the body of, or in close proximity to the thyroid gland. Given their infrequent nature they may pose both diagnostic and management challenges for the treating surgeon when they occur in association with thyroid disorders. METHODS: This is a case series. All patients with concomitant thyroid disorders and a branchial cleft anomaly treated in the University of Sydney Endocrine Surgical Unit in the 10-year period 1994-2003 comprised the study group. Patient demographics, clinical presentation, imaging, surgical management, definitive histology and outcomes were documented. RESULTS: Six patients were identified with an age range of 3-76 years and a male : female ratio of 1:5. Five branchial cleft anomalies were left sided, one was right sided. Two patients had second cleft anomalies, both of which were initially thought to represent metastatic lymph nodes in association with thyroid cancer. A further two patients had third cleft abnormalities presenting as suppurative thyroiditis. The final two patients had fourth cleft abnormalities causing intraoperative management problems. CONCLUSIONS: Branchial cleft remnants and anomalies are rare but may occur in association with thyroid disease. They may pose a diagnostic and management dilemma either preoperatively, when mistaken for metastatic thyroid cancer, or intraoperatively when mistaken for a thyroid nodule.     

Treatment of laryngotracheoesophageal cleft

Four patients with laryngotracheoesophageal cleft were treated in our institution. Two of the patients survived and two died. Cleft levels ranged from partial 15 mm laryngotracheoesophageal to total with extended right bronchoesophageal cleft.     

开放式唇裂继发鼻畸形整形术

目的：探讨单侧唇裂继发鼻畸形的手术治疗。方法：应用跨鼻小柱的鼻翼软骨下缘切口开放式鼻整形进行唇裂继发鼻畸形矫正,鼻小柱侧缘切口继续向下延伸至鼻小柱基底,将鼻小柱基部皮肤向下翻开,充分松解和暴露鼻翼软骨,矫正中隔畸形,将鼻翼软骨向前上内侧复位,与健侧鼻翼软骨、鼻背侧软骨缝合固定,必要时进行切取鼻中隔软骨移植以抬高鼻尖以及加强鼻穹窿。结果：应用本方法治疗单侧唇裂继发鼻畸形47例,单纯进行鼻畸形矫正者11例,同时行唇畸形整形者36例,效果满意,其中有32例进行了6～24个月的随访,平均随访时间为11个月,鼻外形良好。结论：应用开放式鼻整形技术进行唇裂继发鼻畸形矫正,可在直视下充分松解并显露鼻翼软骨,准确复位及缝合固定,术后效果稳定,切口无明显瘢痕。     

The philtrum and unilateral cleft lip

Summary A unilateral cleft lip apparently does not lack a philtral ridge on the cleft side even if the elements are variable with the degree of cleft. The objective of primary repair should be to preserve the complete philtrum.     

新生儿先天性单侧唇裂修复术的临床研究

目的：探讨新生儿单侧唇裂修复的临床治疗效果。方法：选足月健康正常体重单侧唇裂患儿，在出生后48h内行唇裂修复术。对手术效果随访，并与婴幼儿期唇裂修复结果进行比较。结果：新生儿期修复单侧唇裂，效果理想，经3个月至4年随访．唇修复形态的优良率为92．8％，与婴幼儿组相比唇形态优良率无明显差别，但出血少，费用低，住院时间短，家长普遍满意。结论：对先天性单侧唇裂新生儿进行早期修复是安全的，且效果令人满意。     

Surgical repair of combined gastroschisis and sternal cleft

We present a case of prenatally diagnosed gastroschisis combined with a sternal cleft, as well as the successful surgical management of this unusual condition. Successful management of gastroschisis combined with sternal cleft has not been reported before in the literature.     

改良双侧唇裂修复术──延长前唇的唇肌功能整复法

目的：介绍一种新的双侧唇裂修复术延长前唇的唇肌功能整复法。方法：自１９８０年以来应用该法治疗１４６例，详细描述了手术步骤。结果：本法保留了较多的唇部组织，术后唇肌功能恢复较好，前唇延长较多，张力降低，唇弓、唇峰、唇珠形态自然美观。结论：本法定点设计简单、合理，术后静态及功能效果满意，值得推广应用。     

Isolated alar cleft associated with hamartoma

Isolated alar cleft associated with hamartoma is a very rare congenital anomaly. In this paper, this rare anomaly is presented with its clinical findings and the treatment.     

Symptomatic rathke's cleft cysts

Two unusual cases of symptomatic Rathke's cleft cysts are reported. In one, the cyst was entirely suprasellar with a normal sella turcica. The gross visual failure, hypothalamic disturbances, and hypopituitarism of the patient resolved after a transfrontal aspiration and partial excision of the wall of the cyst. The second patient was a pituitary dwarf with hypothalamic dysfunction, but with normal vision. The patient had a large intrasellar cyst that exhibited marked suprasellar extension with calcification of the capsular rim. After transsphenoidal aspiration and partial excision of the wall of the cyst, the patient achieved a satisfactory recovery. The differential features between Rathke's cleft cyst and craniopharyngioma are highlighted.     

Complete laryngotracheoesophageal cleft: management and repair

A newborn baby with a complete or type III laryngotracheoesophageal cleft from larynx to carina was managed with a bifurcated endobronchial tube prior to complete reconstruction in one stage. To prevent pressure on the posterior suture line, a tracheostomy tube was constructed from a REA tube originally designed to use for cleft palate repairs. Fundoplication prevented gastroesophageal reflux and allowed enteral feedings. The child is now monitored at home on a prolonged pharyngeal retraining program with intermittent plugging of his tracheostomy tube. This report describes the management and repair of the first long-term survivor with this severe defect.     

婴儿期唇裂鼻畸形早期修复的临床研究

目的:探讨在婴儿期早期修复唇裂,同期矫正鼻畸形的可行性。方法:采用Millard唇裂修复法修复唇裂,同期矫正鼻畸形,使移位的鼻翼软骨、鼻中隔软骨复位,以恢复正常的解剖关系。结果:30例患儿唇裂鼻畸形早期矫治,经6～8年随访,取得满意效果。结论:早期修复唇裂,同期矫正鼻畸形,使畸形的鼻翼软骨、鼻中隔恢复到正常的解剖位置并在此位置上生长发育,鼻畸形可明显改善。     

Tessier number two cleft

The number two craniofacial cleft as described by Tessier is an extremely rare cleft of unknown etiology with distinct soft tissue and bony characteristics. It traverses the alveolar arch at the level of lateral incisor, middle third of the alar rim, the junction between the nasal bone and the frontal process of maxilla and medial end of the eyebrow and continues cranially as a number 12 cleft. Two such patients with interruption of the middle third of alar rim and asymmetrical hypertelorism are described.