Outcomes for expanded polytetrafluoroethylene strip in frontalis suspension surgery.

PURPOSE: This study evaluates surgical outcomes and complication rates of frontalis suspension with expanded polytetrafluoroethylene (ePTFE). METHODS: This retrospective cohort study reviewed all patients undergoing frontalis suspension surgery using ePTFE as the sling material from January 1 2012 to March 3 2020 by a single surgeon at a single academic center. Two different surgical techniques were evaluated in the placement of the sling material. Demographic, clinical, and operative data were extracted. Outcome data including postoperative lid height, reoperation, and complication rate were extracted for the cohort and compared between the two surgical techniques. Descriptive statistics were utilized. RESULTS: Sixty-four eyes from 49 unique patients were included in this study. Forty-three (67.2%) patients had isolated congenital blepharoptosis; 14 (21.9%) had blepharophimosis, ptosis, and epicanthus inversus syndrome (BPES); and 2 (3.1%) had cranial nerve III palsy. Fifty-one (79.7%) patients had no prior blepharoptosis surgery. Lid crease incision and stab incision techniques were utilized for 24 (37.5%) and 40 (62.5%) eyes, respectively. Overall, 21 (32.8%) eyes required reoperation with ePTFE to achieve appropriate eyelid height or contour. Only one patient experienced implant infection, requiring removal of ePTFE sling after a second reoperation. There were no cases of implant exposure or granuloma formation noted during the study period. CONCLUSION: An ePTFE strip soaked in cefazolin prior to utilization in surgery is a viable material for frontalis suspension surgery, with a lower infectious or inflammatory complication rate than previously reported. However, reoperation rate was still relatively high.

Association between blepharophimosis-ptosis-epicanthus inversus syndrome and lacrimal system anomalies.

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a congenital eyelid syndrome. Several associations, including the horizontal displacement of the puncta, canalicular stenosis, and ectropion have been so far described. Herein, we report a one-year-old boy presented to the Oculoplastic Clinic of Farabi Eye Hospital with complaint of watery discharge from both eyes since his birth. Based on the general appearance, the diagnosis of BPES was made. Mild tear regurgitation from the inferior punctum was noted. Detailed examination showed bilateral superior punctal agenesis with coloboma of both upper eyelids and lateral displacement of the inferior puncta. Multiple unsuccessful attempts of probing were suggestive of the presence of NLDO. The patient was managed by performing canaliculodacryocystorhinostomy. Osteotomy was performed to pass the canalicular and nasolacrimal obstruction followed by a successful canaliculoplasty. Finally, the lacrimal drainage system was intubated with a mono-Crawford from the inferior punctum into the nasal cavity. On the 1st-month follow-up visit, the complaint of watery discharge was resolved. This is an extremely rare report of nasolacrimal duct and sac anomaly in a patient with blepharophimosis. Thus, we recommend the evaluation of the nasolacrimal drainage system in these patients after the first month of birth.

Surgical outcome of epicanthus and telecanthus correction by C-U medial canthoplasty with lateral canthoplasty in treatment of Blepharophimosis syndrome.

PURPOSE: To evaluate the surgical outcome of epicanthus and telecanthus correction by C-U medial canthoplasty with lateral canthoplasty in Blepharophimosis Syndrome. PATIENTS AND METHODS: This was a retrospective single arm interventional study including 18 eyes of 9 patients with Blepharophimosis-ptosis-epicanthus inversus syndrome who presented to oculoplastic clinic, ophthalmology department, Qena university hospital in the period of between July 2020 to April 2021. All the patients had BPES with epicanthus and telecanthus. All cases were subjected to by C plasty with medial and lateral canthoplasty for correction of epicanthus and telecanthus correction followed by frontalis suspension surgery to correct the co-existing blepharoptosis. RESULTS: The study included 9 cases of BPES, 6 boys and 3 girls, the mean age was 5.4 ± 1.5 in the study group, all patients had a positive family history for BPES. After surgery, the mean IICD decreased from 38.44 mm preoperatively to 32.8 mm postoperatively, with a mean difference of 6.2 mm (P <  0.001). Likewise, the mean PFL increased from 20.78 mm preoperatively to 26.63 mm postoperatively, with a mean difference of 5.8 mm (P <  0.001). Epicanthus skin fold disappeared in all cases and medical canthus could be seen with well healed difficulty seen scars. CONCLUSION: C-U medial canthoplasty with lateral canthoplasty in Blepharophimosis Syndrome was found to be an effective procedure in the correction of epicanthus and telecanthus.

A Novel Medial Canthal Reconstruction Technique in Children With Blepharophimosis Syndrome.

PURPOSE: There are different techniques for medial canthoplasty in blepharophimosis syndrome where individuals have epicanthus inversus and telecanthus leading to typical facial appearances. These methods have potential problems with scarring, epistaxis, and extrusion of metal plates. METHODS: The authors describe a novel technique of medial canthal reconstruction using titanium microplates with microscrew fixation to the anterior lacrimal crest, with effective, safe, and reproducible results. RESULTS: Seven children with blepharophimosis syndrome underwent medial canthus reconstruction surgery at a single center with the collaboration of a pediatric oculoplastic surgeon and craniofacial plastic surgeon to improve eye opening through correction of the lid contours and telecanthus. CONCLUSIONS: This is a safe, effective, and reproducible technique, with minimal morbidity and rapid postoperative recovery in children. It produces cosmetically acceptable scars and a strong stable reconstruction of the medial canthal insertion.

Goldenhar syndrome with blepharophimosis and limb deformities: a case report.

BACKGROUND: Goldenhar syndrome has variable presentations and can affect multiple regions of the body. Diagnoses are based on clinical manifestations. The association of Goldenhar syndrome with blepharophimosis and limb deformities has not previously been reported. Here, we describe a patient who was diagnosed with Goldenhar syndrome in association with blepharophimosis, ocular hypertelorism, hearing loss and limb deformities. CASE PRESENTATION: A 10-year-old male was first referred to our ophthalmology clinic on 2009-2-11 for ocular hypertelorism and microphthalmia when he had chin-up position. In the first ophthalmic examination, his palpebral fissure length was 19 mm on the right and 20 mm on the left, both palpebral fissure height was 4 mm, the inner intercanthal distance was 63 mm, both upper margin reflex distances were - 1 mm, the myodynamia of the levator palpebrae muscle was 2 mm on the right and 3 mm on the left, and his visual acuity was 20/40 on the right and 20/32 on the left. A physical examination revealed the patient had developed limb deformities in his hands, wrists, elbows and shoulders along with hearing loss. The patient was diagnosed with Goldenhar syndrome because his clinical presentations included ocular hypertelorism, hearing loss, and multiple acral joint deformities. He underwent a first operation in 2009 and a second in 2015. The second operation achieved a satisfactory result in which the horizontal fissure length was 28 mm on both sides, both palpebral fissure height was 10 mm, the inner intercanthal distance was 30 mm, and both of the upper margin reflex distances were 4 mm. He continued to wear hearing aids as usual. His hearing loss and joint deformities were slated for long-term follow-up at his parents' request. CONCLUSION: The patient, diagnosed with Goldenhar syndrome in association with blepharophimosis, ocular hypertelorism, hearing loss and limb deformities, underwent two operations and achieved a satisfactory result. The patient was submitted to long-term follow-up observations and symptomatic treatments that vary with age and systemic associations, as needed. When treating patients with Goldenhar syndrome, ophthalmology specialists should cooperate with a multi-disciplinary team of clinicians and reach agreement regarding the appropriate systemic and comprehensive treatments.

Characterization of endocrine features and genotype-phenotypes correlations in blepharophimosis-ptosis-epicanthus inversus syndrome type 1.

OBJECTIVE: Blepharophimosis syndrome (BPES) is an autosomal dominant genetic condition resulting from heterozygous mutations in the FOXL2 gene and clinically characterized by an eyelid malformation associated (type I) or not (type II) with premature ovarian failure. The distinction between the two forms is critical for female patients, as it may allow to predict fertility and to plan an appropriate therapy. Identifying an underlying causative mutation is not always predictive of the clinical type of BPES since genotype-phenotype correlations are not yet fully delineated. Here, we describe the clinical and hormonal phenotypes of three female patients with BPES type 1 from two novel families, correlate their phenotypes with identified mutations, and investigate the effects of hormone replacement therapy (HRT). METHODS: Clinical, biochemical, and genetic evaluation were undertaken in all the patients and genotype-phenotype correlation was analyzed. The effects of substitutive hormonal therapy on secondary sexual characteristics development and induction of menarche were evaluated. RESULTS: All patients presented with primary amenorrhea or other signs of ovarian dysfunction. Two distinct mutations, a missense p.H104R change and an in-frame p.A222_A231dup10 duplication in the FOXL2 gene were identified. Observed phenotypes were not in accordance with the prediction based on the current genotype-phenotype correlations. HRT significantly improved secondary sexual characteristics development, as well as the induction of menarche. CONCLUSIONS: This study highlights the importance of early recognition of BPES and emphasizes the need of personalized therapy and follow-up in female patients carrying distinct FOXL2 mutations.

Surgical Approach, Findings, and Eight-Year Follow-Up in a Twenty-Nine Year Old Female With Freeman-Sheldon Syndrome Presenting With Blepharophimosis Causing Near-Complete Visual Obstruction.

The authors describe the surgical approach, findings, and 8-year follow-up in a 29-year-old woman, with severe Freeman-Sheldon syndrome, presenting with congenital blepharophimosis of both upper eyelids resulting in near-complete functional visual obstruction. To avoid possible Freeman-Sheldon syndrome-associated complications of malignant hyperthermia, difficult vascular access, and challenging endotracheal intubation, the surgery was completed under local anesthesia without sedation, and anatomical and functional correction was immediate and remained stable at 8-year follow-up. Unlike many congenital craniofacial syndromes, which frequently involve life-long impairments, important implications exist for plastic surgeons to facilitate opportunities for patients to overcome functional limitations.

The Partnership of Medical Genetics and Oral and Maxillofacial Surgery When Evaluating Craniofacial Anomalies.

A medical geneticist who has an interest in craniofacial anomalies forms a natural partnership with an oral and maxillofacial surgeon, which facilitates patient care. Using complementary diagnostic and therapeutic skills, the search for a recognizable pattern can lead to a syndrome diagnosis. After the initial examination, there is usually genetic testing to confirm the clinical diagnosis. Once established, care coordination and genetic counseling can be provided for the parents and the patient. Enrolling the patient into a research study could be helpful to understand the diagnosis but, in some circumstances, might not have immediate clinical relevance. A multidisciplinary craniofacial team is generally necessary for long-term management. This article discusses illustrative patients evaluated from 2007 through 2011 with the senior oral and maxillofacial surgeon at the Massachusetts General Hospital (Leonard B. Kaban, DMD, MD). These include single patients with the Nablus mask-like facies syndrome and auriculo-condylar syndrome and a series of 20 patients with Gorlin syndrome followed by a multispecialty team. A successful collaboration between a medical geneticist and an oral and maxillofacial surgeon optimizes the treatment of patients with craniofacial anomalies.

A modified staged surgical intervention for blepharophimosis-ptosis-epicanthus inversus syndrome: 125 cases with encouraging results.

BACKGROUND: Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant condition characterized by typical eyelid malformations that include blepharophimosis, ptosis, epicanthus inversus, and telecanthus. METHODS: We retrospectively reviewed 125 consecutive BPES patients who underwent staged surgical intervention from July 2003 to December 2011. All patients underwent initial medial and lateral canthoplasties, followed by blephroptosis correction 6 to 12 months afterward. The parameters that were studied included horizontal palpebral fissure length (PFL), vertical interpalpebral fissure height, inner intercanthal distance (IICD), the ratio of IICD to PFL, and frontalis function (FF). Facial photographs were taken preoperatively and postoperatively. Paired and group t tests were used for statistical analysis to evaluate surgical outcomes. RESULTS: After consecutive operations, the mean PFL increased from 19.5 to 25.7 mm (mean difference, 6.2 mm; P < 0.01). The mean interpalpebral fissure height increased from 3.4 to 8.5 mm (mean difference, 5.1 mm; P < 0.01). The mean IICD decreased from 38.0 to 30.9 mm (mean difference, 7.1 mm; P < 0.01). The mean FF was 7.3 mm for BPES patients approximately 5 years old and 10.4 mm for patients approximately 7 years old. There was no difference between children who underwent muscle flap suspension and healthy children of the same age (P > 0.05). CONCLUSIONS: The modified staged surgical intervention, including Y-V flap, von Ammon, and frontalis muscle flap suspension, provided effective results both in function and cosmesis for BPES. The FF was not weakened by surgery.

Cosmetic Comparison Between the Modified Uchida Method and the Mustarde Method for Blepharophimosis-Ptosis-Epicanthus Inversus Syndrome.

PURPOSE: Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare, congenital, surgically challenging disease. We undertook an objective, functional, and cosmetic comparison between the modified Uchida procedure and the Mustarde procedure on Asian patients with BPES. DESIGN: This is a retrospective, comparative, interventional case series with the description of 2 surgical techniques. PARTICIPANTS: Twenty consecutive Japanese patients with BPES were studied. METHODS: The patients were assigned to either the modified Uchida or the Mustarde procedure on the basis of preoperative intercanthal distance (ICD) ratio (ICD/palpebral fissure width). Intercanthal distance and margin reflex distance were measured from photographs before and after surgery. Classifications were made according to epicanthal fold score and cosmetic score using a visual analog scale (VAS). MAIN OUTCOME MEASURES: The main outcome measures were postoperative improvement in ICD ratio and the VAS. RESULTS: Of the 20 patients, 5 underwent the modified Uchida procedure and the other 15 underwent the Mustarde procedure. The preoperative median ICD ratio was 2.0 (range, 1.9-2.5) and 2.5 (range, 2.0-3.4) for the modified Uchida and Mustarde procedures, respectively, and decreased to 1.5 (range, 1.5-1.7) and 1.6 (range, 1.5-1.8) postoperatively. The mean ICD reduction rate was 35.7% (SD, 1.0%) in the Mustarde group and 22.1% (SD, 1.3%) in the modified Uchida group (P < 0.001).The mean VAS for the modified Uchida and Mustarde procedures was 5.9 (SD, 1.3) and 2.8 (SD, 1.1), respectively, which was statistically significant (P = 0.0166). CONCLUSIONS: Both the modified Uchida and Mustarde procedures could effectively shorten the ICD; however, cosmetic results were significantly superior using the modified Uchida method.

A Modified One-Stage Early Correction of Blepharophimosis Syndrome Using Tutopatch Slings.

PURPOSE: To investigate the efficacy of a one-stage early correction of blepharophimosis-ptosis-epicanthus inversus syndrome (BPES), using bovine pericardium derived membrane (TUTOPATCH(®)) for the frontalis suspension. METHODS: We prospectively studied 12 eyes from 6 patients (median age 14 months) affected by BPES with severe ptosis. All patients were submitted to a one-stage early correction of ptosis (frontalis suspension with TUTOPACH(®)) and telecanthus and epicanthus inversus. Upper margin reflex distance (MRD), nasal inner intercanthal distance (IICD), horizontal fissure length (HFL), and IICD/HFL ratio were evaluated using photographs. RESULTS: The Wilcoxon signed-rank test showed a statistically significant difference between pre- and post-operative MRD, IICD, HFL, and the IICD/HFL ratio. CONCLUSION: An early TUTOPATCH-assisted frontalis suspension, together with the correction of telecanthus and epicanthus inversus, is an effective procedure for BPES cases with severe ptosis.

Facial abnormalities in Nablus mask-like facial syndrome: multidetector computed tomography findings.

Nablus mask-like facial syndrome (NMLFS) is a rare microdeletion syndrome characterized by a mask-like facial appearance. NMLFS has been reported in only 6 patients and has a recognizable facial appearance, along with other clinical features. The first case of NMLFS has been described by Teebi in 2000, in a 4-year-old Palestinian boy. Three years later, Salpietro et al reported a second example of NMLFS in a 21-month-old girl. The same patient recently came to our hospital to undergo a computed tomography (CT) study to evaluate the degree of development of the zygomatic-maxillary region for orthodontic treatment and orthognathic surgery. To the best of our knowledge, no reports have previously illustrated the maxillofacial CT findings of NMLFS in the radiologic data. We report the multidetector CT (MDCT) facial characteristics/abnormalities of this syndrome, emphasizing the usefulness of multiplanar reformations (MPRs) in preoperative planning.

[Pay attention to patient's visual function in oculoplastic surgery for congenital blepharophimosis syndrome].

Blepharophimosis is characterized by the presence of a narrowed horizontal palpebral aperture, ptosis, epicanthus inversus, and telecanthus. Due to patient's eye deformity, both of his or her appearance and psychological development are affected. In some cases, blepharophimosis not only influence the appearance but also the visual development. Therefore, patients with blepharophimosis require to be evaluated the visual function before the surgery in order to prevent amblyopia. In this article, we conclude that we should pay close attention to patient's visual function before and after the surgery. The timing of surgery must weigh the balance between early surgery to prevent amblyopia and late surgery for more reliable ptosis measurement, which provides a better surgical outcome.

Bilateral Candida and atypical mycobacterial infection after frontalis sling suspension with silicone rod to correct congenital ptosis.

In this case report, the authors describe an unusual complication of a frontalis sling suspension with silicone rods. A 5-year-old girl with blepharophimosis syndrome underwent frontalis sling suspension using an open sky technique. Four weeks after surgery, she was noted to have pustules over both upper eyelids and eyebrows. Cultures from the surgical sites grew Mycobacterium chelonae and Candida parapsilosis. Intravenous antibiotics and antifungals and sling explantation were curative. One month after sling explantation, the patient maintained an adequate marginal reflex distance 1. Atypical mycobacterial and Candida infection should be considered in the differential diagnoses of postoperative infection after frontalis sling suspension with silicone rods.

Double jeopardy: Blepharophimosis syndrome with congenital nasolacrimal duct obstruction in twins.

PURPOSE: Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is an autosomal dominant disorder characterized by bilateral ptosis with poor levator function, epicanthus inversus and shortened horizontal palpebral fissures. METHOD: Eighteen -month -old twin sisters presented with history of watering from their left eyes since birth. Examination revealed features of BPES with left congenital nasolacrimal duct obstruction in both. RESULT: the twins underwent therapeutic probing, which was successful in one and failed in the other. the second child was subsequently managed by external dacryocystorhinostomy. CONCLUSION: To our knowledge, this is the first report of Blepharophimosis syndrome with congenital nasolacrimal duct obstruction occurring in twin sisters.

A new method of medial epicanthoplasty for patients with blepharophimosis-ptosis-epicanthus inversus syndrome.

PURPOSE: To describe a medial epicanthoplasty technique using the skin redraping method and review the surgical outcome in patients with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). DESIGN: Retrospective, noncomparative, interventional case series with the description of a new surgical technique. PARTICIPANTS: Sixteen consecutive Asian patients with BPES. METHODS: The charts of patients with BPES who underwent medial epicanthoplasty using the skin redraping method were reviewed retrospectively. Preoperative and postoperative inner intercanthal distance (IICD), interpupillary distance (IPD), horizontal palpebral fissure length (HPFL), and visibility of the scar were measured. The ratio of the IICD to IPD (IICD ratio) was calculated. MAIN OUTCOME MEASURES: Postoperative improvement in IICD ratio and the visibility of the surgical scar. RESULTS: The preoperative median IICD ratio was 1.65 (range, 1.49-1.83) and decreased to 1.27 (range, 1.02-1.48) postoperatively. The median reduction in IICD ratio was 21.7% (range, 16.7%-38.2%) (P<0.001, Wilcoxon signed-rank test). Fourteen patients (87.5%) had no visible scarring or scarring only visible under close inspection. Two patients (12.5%) had a more apparent scar, but no patient had severe scarring that required revision. CONCLUSIONS: Medial epicanthoplasty using the skin redraping method is an effective technique in the treatment of epicanthus inversus and telecanthus in patients with BPES, with excellent cosmetic outcomes.

Vector control in internal midface distraction using temporary anchorage devices.

Le Fort III and monobloc distraction osteogenesis serve as the primary surgical treatment for children with severe midface hypoplasia. The orbital retrusion and class III malocclusion of patients with midface hypoplasia is best addressed with bodily advancement of the midface segment parallel to the cephalometric Frankfort horizontal plane. Use of internal distraction devices allows for advancement of the midface without extensive external hardware but comes at the cost of less vectorial control, resulting in a distraction vector that can cause a clockwise rotation of the entire midface or frontofacial component creating hollow appearing orbits. To counteract this clockwise rotation, we have developed a technique using orthodontic microimplants to anchor interarch class III relationship elastics. We report our experiences with this technique on a cadaveric model and as a case series of 17 patients who underwent midface distraction. A Le Fort III distraction procedure was carried out on a cadaver, and the orbital height was measured at 0-, 10-, and 20-mm distraction advancement with and without elastics in a class III relationship. Improvement of both subjective hollow appearance of the orbits and objective measurement of the orbital height with class III relationship elastics demonstrated the efficacy of class III relationship elastics in counteracting the clockwise rotation of the midface segment. A review of 17 patients with midface or frontofacial hypoplasia treated with Le Fort III or monobloc distraction with simultaneous microimplant anchored class III relationship elastics revealed correction of malocclusion and improved midface projection without significant increase in vertical height of the orbits.

[Blepharophimosis ptosis epicanthus inversus syndrome (BPES) (corrected)]. / Das Blepharophimose-Ptosis-Epikanthus-inversus-Syndrom (BPES)--im deutschsprachigen Raum in der Regel als Waardenburg-Syndrom bekannt.

The blepharophimosis ptosis epicanthus inversus syndrome (BPES, also known as Waardenburg syndrome) was probably first reported by Ammon in 1841 and discribed more fully by Vignes in 1889. Its primary effects on the soft tissue of the midface are blepharophimosis, ptosis, epicanthus inversus and telecanthus. It starts with the epicanthic folds at about the age of 3-4 years, followed by the correction of the ptosis about 9-12 months later. Early surgery may be necessary for amblyopia. In 1995 the gene locus was identified as 3Q23. BPES is due to a mutation within a single gene, the FOXL2 gene. In female patients an early childhood ovarian insufficiency must be excluded.

A case of blepharophimosis: Freeman Sheldon syndrome.

PURPOSE: Important implications exist for ophthalmologists when considering possible early surgical intervention for potential amblyogenic anatomical abnormalities. The authors discuss the risks and benefits from an ophthalmological perspective of different interventions and review the genetic testing that confirmed the diagnosis. OBSERVATIONS: The authors describe the findings and management of an infant with Freeman Sheldon syndrome presenting with blepharophimosis of both eyelids resulting in inability to open both eyes during the first several days of life. Although the mode of inheritance for Freeman Sheldon syndrome (formerly known as Whistling Face Syndrome) is often autosomal dominant, our patient had no known family history of congenital abnormalities or consanguinity. However, genetic testing confirmed a heterozygous variant in MYH3, consistent with autosomal dominant Freeman Sheldon Syndrome. When our patient required gastrostomy (G-tube_placement, we performed an exam under anesthesia (EUA)). As is typical for Freeman Sheldon syndrome patients, intubation was difficult and complicated by pneumothorax. Eye-opening improved slightly after several weeks of life; however, the decision was made to proceed with eyelid surgery to prevent deprivation amblyopia. Surgery is scheduled for a future date. Additionally, the patient had congenital nasolacrimal duct obstruction of the left eye; however, a probing and irrigation failed because of obstruction from the abnormal facial anatomy. CONCLUSIONS AND IMPORTANCE: Patients with Freeman Sheldon syndrome are at increased risk for complications from anesthesia and surgery. Risks and benefits should be strongly considered and discussed with parent(s)/guardian(s) prior to any surgical intervention. Genetic testing of the MYH3 gene can confirm the diagnosis.

Correction of the lower eyelid malpositioning in the blepharophimosis-ptosis-epicanthus inversus syndrome.

PURPOSE: Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is an autosomal dominant complex eyelid malformation. The authors aim to offer an explanation for the lower eyelid malformation and propose a novel surgical approach to correct it. METHODS: An observational and interventional case series of 10 consecutive, molecularly proven BPES patients who underwent surgical repair of the lower eyelid malformation. During surgery detailed anatomical examination and surgical repositioning of the medial canthal tendon was performed. All the patients were followed up regularly after the surgery and assessed for epiphora. RESULTS: All patients exhibited a marked asymmetry in the attachment of the lower and upper eyelid to the medial canthal tendon, with the lower eyelid being much less attached. This resulted in an abnormal downward concavity with a temporal ectropion and a temporally displaced lower eyelid. Consequently, the inferior punctum was displaced temporally. All patients underwent a novel surgical technique to remediate this, namely, inserting a 4.0 nylon suture between the tarsal plate of the lower eyelid and the medial canthal tendon during telecanthus surgery. This simple additional surgical step corrected not only the position of the lower eyelid but also its abnormal downward concavity, the temporal ectropion and the lateral displacement of the inferior punctum. None of the authors' patients had lasting epiphora. CONCLUSION: Lateral displacement of the inferior punctum is an important hallmark in the diagnosis of BPES. The authors demonstrate an anatomical explanation for the complex lower eyelid malformation and also propose a novel surgical technique to correct this. During surgical repair of the telecanthus and blepharophimosis, specific attention should be paid to reattachment of the lower eyelid to the medial canthal tendon. This understanding improves clinical diagnosis and surgical treatment of BPES patients.