Sternal cleft and pectus excavatum: an overlooked congenital association?

BACKGROUND: Sternal cleft (SC), a rare thoracic malformation, is associated with pectus excavatum (PE) in 2.6-5% of cases. It remains unclear if these conditions are congenitally linked or if SC repair triggers PE. To investigate the potential higher frequency of PE in SC cases, we conducted a retrospective study of our SC patients. METHODS: We assessed PE incidence, progression, and management in SC patients treated at our institute from 2006 to 2022. When available, we collected pre-SC repair CT scan data, calculating the Haller Index (HI) and Correction Index (CI) and compared them to a selected control group. RESULTS: Among 8 SC patients, 7 had concomitant PE (87.5%), varying in severity. PE management ranged from observation to thoracoplasty, depending on its degree. We observed a significant pre-operative CI difference between SC and control group patients (p < 0.00001). In the last two SC repair cases, we attempted concurrent PE prevention or treatment. CONCLUSION: Our findings suggest an underestimated association between PE and SC in the existing literature. SC patients may exhibit a predisposition to PE from birth, which may become more apparent with growth after SC repair. Consequently, PE prevention or treatment should be considered during SC repair procedures.

Aesthetic Outcome of Primary Rhinoplasty of Saddle Nose Deformity in Naso-Orbital Ethmoidal Fractures in Asian Patients.

BACKGROUND: Saddle nose deformity following naso-orbital ethmoidal (NOE) fractures remain a challenging problem for the reconstructive surgeon. Early reduction and internal fixation allow for fracture stabilization but is unable to address the problem of the depressed nasal dorsum, especially after soft tissue shrinkage. The aim of this study is to evaluate the outcome of primary rhinoplasty in patients with NOE fractures. MATERIALS AND METHODS: From 2016 to 2019, 9 patients presented to our department with NOE fractures complicated by saddle nose deformity underwent primary nasal reconstruction at the time of their fracture fixation. Life size (1:1) frontal and lateral postoperative photographs were taken. Three objective measurements were made, including the nasofrontal angle, tip projection, and radix projection. These measurements were compared between normal persons (group 1), preoperative patients (group 2), and postoperative patients (group 3). Nose aesthetic assessment was carried out via a panel assessment using a Visual Analog Scale of 5. Patient satisfaction was further assessed subjectively by the patient themselves using the Visual Analog Scale. RESULTS: When comparing group 3 to 2, a significant reduction in the nasofrontal angles was found with an accompanying increase in the radix and tip projection ( P <0.05). No statistical significance between normal persons and postprimary rhinoplasty patients was noted between groups 1 and 3. Average patient satisfaction scored 3.86±1.07 compared with 3.63±0.84 by laypersons and 4±0.77 by specialists' panel. CONCLUSION: Primary nasal reconstruction may be an alternative method for achieving optimum results following NOE fractures preventing the development of secondary saddle nose deformity with a shortened nose which may potentially be more difficult to correct.

Reconstruction of Congenital Sternal Cleft: A Systematic Review of the Literature.

INTRODUCTION: Sternal cleft (SC) is a rare congenital deformity that results from failure of sternal bar fusion. Sternal cleft can be categorized as superior partial, inferior partial, or complete. Each form of SC can present as an isolated defect or in association with other congenital deformities, which presents a unique challenge for reconstructive surgeons. In our systematic review, we aim to summarize the published experience on repair of SCs and present a pragmatic approach to help guide reconstructive planning. METHODS: A systematic review was performed to identify all reported SC cases in literature that underwent sternal reconstruction. RESULTS: Seventy-one studies were identified from 1970 to 2019, which included a total of 115 patients. Superior partial SC was the most common SC variant, accounting for 65.2% (75/115) of all reported cases. There were 31 cases of complete SC (27.0%) and 9 cases (7.8%) of inferior partial SC; 49.6% of the patients (57/115) in our review had isolated SC without any other congenital deformities. Sixty-seven patients (60.3%) were treated with primary closure, with or without secondary maneuvers, such as chondrotomies, cartilage resection, or periosteal flaps. Alternative methods included interposition grafts, with autologous rib graft in 18 patients (15.8%), permanent mesh in 8 patients (7.0%), acellular dermal matrix in 5 patients (4.4%), sternal plate flap in 5 patients (4.4%), and nonthoracic autologous bone grafts in 4 patients (3.5%). CONCLUSIONS: Our review supports that primary closure should be attempted regardless of patient age. For wider sternal gap, reconstruction with an autologous local graft or flap should be considered. When the patient does not have sufficient autologous tissue for a successful sternal reconstruction, alloplastic or allograft interposition options are a reasonable choice.

Congenital Kyphosis: Progressive Correction With an Instrumented Posterior Epiphysiodesis: A Preliminary Report.

PURPOSE: Congenital kyphosis is a rare condition. In this case series we sought to identify the outcomes and complications of posterior instrumented fusion and the resultant epiphysiodesis effect in uniplanar congenital kyphosis in pediatric patients. METHOD: Pediatric patients were included if treated for a uniplanar congenital kyphotic deformity treated with posterior instrumented spinal fusion between October 2006 and August 2017, with a minimum of 2 years of follow-up. Patients were excluded if a coronal deformity >10 degrees was present. RESULTS: Six patients met the inclusion criteria. Mean age at surgery was 3.6 years. The mean kyphotic deformity before surgery was 49.7 degrees. All patients underwent posterior instrumented fusion with autogenous iliac crest graft and a cast or brace postoperatively. One patient showed a loss of motor evoked potential on prone positioning which returned to normal on supine positioning. No patient showed any postoperative neurological deficits. One patient was diagnosed with a wound infection which was successfully treated with oral antibiotics.By a follow-up of 5.4 years (range, 2.2 to 10.9 y) there was no failure of instrumentation. An epiphysiodesis effect (a difference of ≥5 degrees in the kyphotic deformity measured between the immediate postoperative and final follow-up lateral whole spine XR) of 16.2 degrees (range, 7.2 to 30.9 degrees) was seen in 5 patients. The mean annual epiphysiodesis effect was 2.7 degrees (95% confidence interval, 1.4-4.1 degrees). No kyphosis proximal to the instrumentation was observed for the duration of follow-up. CONCLUSION: Posterior instrumented fusion and epiphysiodesis is safe and effective. The epiphysiodesis effect occurs in 5/6 of cases, and our data suggests that the procedure is associated with an acceptable blood loss and a low incidence of neurological complications.

Determination of congenital absence of palmaris longus tendon with clinical examination and ultrasonography.

OBJECTIVES: The tendon of the palmaris longus is commonly used as a tendon graft in many reconstructive surgeries. Palmaris longus absence (PLA) was found in 15% among individuals worldwide. In this prospective study, we aimed to conduct an incidence study in which physical examination methods were confirmed by ultrasonography in PLA, and to evaluate the relationship of absence with age, gender, laterality and dominant hand. METHODS: The study included 490 cases. They were initially tested to evaluated by physical examination using the Schaeffer's and Hiz-Ediz test for the assessment of the palmaris longus tendon. Additional ultrasonography was performed to confirm its absence in 129 wrists of 78 cases whose tendons could not be visualized or palpated. RESULTS: The incidence of tendon absence was 13% by physical examination methods. According to the final results when we added ultrasonography to physical examination methods, the incidence of unilateral, bilateral and overall absence of the palmaris longus were 5%, 9% and 11% respectively. There was no statistically significant difference between individuals with and without PLA in terms of gender, side, age and dominant hand (p = 0.796, p = 0.622, p = 0.397 and p = 0.187, respectively). However, bilateral PLA was statistically significantly higher than unilateral in both genders (p = 0.011). CONCLUSIONS: We think that agenesis should be proven accurately by ultrasonographic examination for the final result before any surgical procedure with palmaris longus tendon. Furthermore measuring the diameter of the palmaris longus tendon by preoperative ultrasonography can be useful for surgeons who plan a procedure that requires specific measurements.

Tibial and femoral osteotomies in varus deformities - radiological and clinical outcome.

BACKGROUND: Varus deformities of the knee are frequently corrected by osteotomies, which should be performed at the level of origin. But in contrast to high tibial osteotomies (HTO), little data exists for distal femoral osteotomies (DFO). This study evaluates radiological and clinical outcomes after valgisation osteotomies in the proximal tibia and distal femur. METHODS: We used an observational cohort study design and prospectively performed preoperative long standing radiographs (LSR), lateral x-rays and clinical questionnaires (SF-36, Lysholm score, VAS). Postoperative LSR and lateral x-rays were obtained on average 18 months postoperative and postoperative clinical questionnaires at final visit (mean follow up 46 months). A subgroup analysis of the different surgical techniques (oHTO vs. cDFO) was performed, with regards to radiological and clinical outcomes. RESULTS: Finally 28 osteotomies with medial tibial opening (oHTO) or lateral femoral closing (cDFO) wedge osteotomies in 25 consecutive patients (mean age 40 years) were identified. There were 17 tibal and 11 femoral procedures. All osteotomies were performed at the origin of deformity, which was of different etiology. The average deviation of the final HKA compared to the preoperative planning was 2.4° ± 0.4°. Overall, there was a significant improvement in all clinical scores (SF-36: 61.8 to 79.4, p < 0.001; Lysholm-score: 72.7 to 90.4, p < 0.001; VAS: 3 to 1, p < 0.001). There was no significant correlation between surgical accuracy and outcome scores. CONCLUSION: Valgisation osteotomies lead to a significant improvement in all clinical scores with the demonstrated treatment protocol. An appreciable proportion of varus deformities are of femoral origin. Since cDFO provides comparable radiological and clinical results as oHTO, this is an important treatment option for varus deformities of femoral origin.

Short Face Dentofacial Deformities: Changes in Social Perceptions, Facial Esthetics, and Occlusion After Bimaxillary and Chin Orthognathic Correction.

The purpose of this study was to document changes in social perceptions and facial esthetics, and document occlusion outcomes in a series of short face (SF) dentofacial deformity (DFD) subjects. The investigators hypothesized that subjects would achieve positive change in social perceptions and facial esthetics, and maintain a long-term corrected occlusion after undergoing bimaxillary and chin osteotomies.A retrospective cohort study was implemented. Photographic records and occlusion parameters were studied preoperatively and >2 years after surgery. The first outcome variable was social perceptions of SF subjects, judged by laypersons. The second outcome variable was facial esthetics, judged by professionals. The third outcome variable was occlusion maintained long-term.Fifteen subjects met inclusion criteria. Mean age at operation was 33 years. Consistent facial contour deformities at presentation included deficient maxillary dental show and downturned oral commissures. As a group, there was improvement (P < 0.05) in 11 of 12 social perceptions, judged by laypersons, all subjects achieved correction of the facial esthetic parameters studied by professionals, and all subjects maintained a favorable occlusion long-term.In SF DFD subjects, bimaxillary and chin surgery proved effective to improve social perceptions, to correct facial contour deformities, and in achieving a long-term corrected occlusion.

Prevalence and anatomy of the axillary arch and its implications in surgical practice: A meta-analysis.

PURPOSE: The following research aimed to investigate the prevalence and anatomical features of the axillary arch (AA) - a muscular, tendinous or musculotendinous slip arising from the latissimus dorsi and that terminates in various structures around the shoulder girdle. The AA may complicate axillary lymph node biopsy or breast reconstruction surgery and may cause thoracic outlet syndrome. METHODS: Major electronic databases were thoroughly searched for studies on the AA and its variations. Data regarding the prevalence, morphology, laterality, origin, insertion and innervation of the AA was extracted and included in this meta-analysis. The AQUA tool was used in order to assess potential risk of bias within the included studies. RESULTS: The AA was reported in 29 studies (10,222 axillas), and its pooled prevalence estimate in this meta-analysis was found to be 5.3% of the axillas: unilaterally (61.6%) and bilaterally (38.4%). It was predominantly muscular (55.1% of the patients with the AA), originated from the latissimus dorsi muscle or tendon (87.3% of the patients with the AA), inserted into the pectoralis major muscle or fascia (35.2% of the patients with the AA), and was most commonly innervated by the thoracodorsal nerve (39.9% of the patients with the AA). CONCLUSION: The AA is a relatively common variant, hence it should not be neglected. Oncologists and surgeons should consider this variant while diagnosing an unknown palpable mass in the axilla, as the arch might mimic a neoplasm or enlarged lymph nodes.

Short Nose Correction: Septal Cartilage Combined With Ethmoid Bone Graft.

BACKGROUND: Multiple methods are employed to correct short nose deformities, with septal extension graft representing the first choice for Asians. However, the volume of the septal cartilage in many Asian patients is not sufficient to be used alone for such operation. The present work developed a new method combining the septal cartilage with ethmoid bone graft to overcome this issue in Asian patients with short noses. METHODS: Thirty-five women with short noses underwent septal extension graft from February 2015 to March 2017. The endoscopic technique was utilized to harvest the ethmoid bone to enhance the L-strut structure. An L-strut, comprising 0.8 cm segments of the caudal and dorsal cartilaginous septa, is left altered in order to harvest more cartilage for septal extension. The harvested the septal cartilage, approximately 1.0 mm thick and 16 to 20 mm long, underwent grafting on one side of the caudal septum. This was followed by alar cartilage fixation at the septal cartilage graft end. Finally, nose length, nasal tip projection and nasolabial angle were assessed before and after the surgery. RESULTS: Septal cartilage combined with ethmoid bone graft yielded an adequate nose lengthening and reduced nostril show, also in individuals showing extremely little septal cartilage. CONCLUSIONS: This new method is effective for short nose deformity correction in Asian patients. Combining the septal cartilage with ethmoid bone graft yields ideal outcome without overt complications, achieving commendable patient satisfaction.

Coexistence of Antrochoanal Polyp and Fibrous Dysplasia in the Maxilla.

The authors report an unusual case of coexistence of antrochoanal polyp (ACP) and fibrous dysplasia (FD) in the maxilla. To the best of our knowledge, this condition has never been reported in the literature. The complete removal of the ACP through endoscopic sinus surgery was attained and FD was ultimately diagnosed by histopathological examination. It is hypothesized that chronic inflammation or irritation due to FD has an effect on the occurrence of ACP.

Talus bipartitus: a systematic review and report of two cases with arthroscopic treatment.

PURPOSE: The aim of this study was to provide a literature review on talus bipartitus and to introduce an arthroscopic treatment option. METHODS: A systematic review of published case reports and small case series was performed. Medline, Embase, CINAHL, Google Scholar and Web of Science databases were searched for relevant publications. In addition, three cases of talus bipartitus treated in our institute were discussed. RESULTS: Eleven articles were identified, reporting on 23 patients, of whom one patient had a bilateral talus bipartitus. Fourteen were males (61%). The median age at presentation was 15.5 years (IQR 14-24.3). In 21 of the symptomatic cases (96%), the patient experienced ankle pain, and 13 had a restricted range of motion (54%). In our institution, two patients were treated arthroscopically and had excellent short- and long-term outcomes. CONCLUSION: Talus bipartitus is a rare anatomical anomaly. Symptoms are characterized by pain and restricted subtalar motion in young patients. Surgical treatment is focused on either fixation or excision of the bony fragment. Our two cases have demonstrated that an arthroscopic approach can be a safe and effective treatment option in patients with a symptomatic talus bipartitus. LEVEL OF EVIDENCE: IV.

Post-operative deformation and extrusion of the discoid lateral meniscus following a partial meniscectomy with repair.

PURPOSE: The purpose of this study was to investigate the morphology of the discoid lateral meniscus sequentially following a partial meniscectomy with repair using magnetic resonance imaging (MRI). METHODS: Nine patients with a symptomatic discoid lateral meniscus with a peripheral tear were enrolled in this study, and a partial meniscectomy with repair was performed arthroscopically. An MRI examination was performed 2 weeks after surgery (before weight bearing was permitted) and again 6 months after surgery (when sporting activities could resume). The width, height and distance of the discoid lateral meniscus were measured. The distance was defined as the distance between the edges of the discoid lateral meniscus and the tibia. RESULTS: The width of the anterior, middle and posterior segments significantly decreased from 2 weeks to 6 months after surgery. The height of the middle and posterior segments significantly increased from 2 weeks to 6 months after surgery, whereas the height of the anterior segment did not significantly change. The distance of the anterior, middle and posterior segments significantly decreased from 2 weeks to 6 months after surgery. CONCLUSION: The discoid lateral meniscus exhibited deformation and extrusion from 2 weeks to 6 months after a partial meniscectomy with repair. Therefore, the function of load transmission might not be maintained appropriately after surgery. LEVEL OF EVIDENCE: IV.

Complex Truncal Masses in the Setting of CLOVES Syndrome: Aesthetic and Functional Implications.

BACKGROUND: Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal abnormalities (CLOVES) is a complex overgrowth syndrome with dramatic aesthetic and functional implications. The truncal masses characteristic of CLOVES syndrome are described as vascular malformations or lipomatous lesions with variable vascular components. Herein, we describe our single-institution experience with surgical excision of CLOVES-related truncal masses and discuss future directions in treatment of these complex anomalies. METHODS: A single-institution retrospective review was performed for patients diagnosed with CLOVES syndrome. Patients undergoing excision of truncal vascular malformations were included. Outcome measures included perioperative characteristics [estimated blood loss (EBL), specimen size/anatomic location, blood-product requirement], as well as length-of-stay [LOS], and complication profile. Mean follow-up was 23.4 months (range 4.2-44). RESULTS: Three consecutive patients were reviewed, accounting for 4 surgical operations. One patient underwent two operations for two distinct masses. All lesions were located on the upper back or flank with various degrees of muscular involvement. One patient required no transfusions with an uneventful 2-day hospitalization. The remaining three patients had an EBL ranging from 1500 to 6450 mL, requiring 9-13 units of packed red blood cells and 5-8 units of fresh frozen plasma during LOS (averaging 5 days). Mean weight of resected masses was 6.26 lbs (range 2.04-12 lbs) and mass dimensions ranged between 1778.9 and 15,680 cm3. One patient with recurrence was subsequently treated with a combination of sclerotherapy and rapamycin, leading to significant mass reduction. CONCLUSIONS: Management of CLOVES syndrome requires a collaborative and multimodal approach. Although surgical debulking is one treatment option, non-invasive medical modalities and sclerotherapy should be considered prior to surgical resection. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Lateral Ligament Reconstruction for Ball-and-Socket Ankle Accompanying Lateral Ankle Instability: A Case Report and Literature Review.

The ball-and-socket ankle joint is a rare deformity characterized by the loss of concavity in the trochlear surface of the talus with rounding of the articular surfaces of the distal fibula and tibia. Frequently, tarsal coalitions, fibular hypoplasia, and shortening of the limb accompany this deformity. To date, no data have been reported on surgical treatment of lateral ankle joint instability and peroneal tendon dislocation concomitant with a ball-and-socket ankle joint. In the present study, we report the case of a 43-year-old male patient with right lateral ankle joint instability and peroneal tendon dislocation in a ball-and-socket ankle joint, with accompanying tarsal coalition. This was surgically treated by lateral ankle joint ligament reconstruction and tenodesis.

Isolated sternal cleft in a patient with atrial septal defect: A rare sole association.

Sternal cleft (SC) is a rare form of chest wall deformity with an incidence of <0.15%. It occurs because of failure of fusion of the sternal bars about the 7-9th week of intrauterine life. It could occur in isolation or as a part of a syndromic complex. This condition exposes the vital mediastinal structures to a huge risk of injury, aside from cosmetic issues. Treatment in the neonatal period is most favourable, though other modalities are available in the later stages of life, but it can be challenging. We present the case of a 2-month-old female infant with a superior SC with a single association of secundum atrial septal defect. She was asymptomatic. She was treated by primary repair to protect the underlying mediastinal structures as well as for cosmetic reasons. Such a repair resulted in good clinical outcome.

The use of a pedicle screw-cortical screw hybrid system for the surgical treatment of a patient with congenital multilevel spinal non-segmentation defect and spinal column deformity: a technical note.

INTRODUCTION: This technical note presents, to the authors' knowledge, the first reported case of a hybrid pedicle-cortical screw system for instrumented fusion in a patient with congenital vertebral column deformity. CASE: Cortical screws were navigated using stereotactic guidance to extend a prior non-segmented fusion mass, facilitating instrumentation in a circumstance with completely distorted anatomy. This technique provided a safe trajectory with excellent cortical purchase in an anatomically deformed spine. DISCUSSION: Cortical screw fixation may serve to be helpful in augmenting pedicle screw fixation and in circumstances in which the bone quality is suboptimal or the pedicles are compromised. Cortical screw fixation is a relatively new technology, but it may prove to be invaluable in providing an adjunct to pedicle screw constructs in anatomically distorted or osteoporotic spines.

Repair of inferior sternal cleft using a titanium plate in an infant with pentalogy of Cantrell.

Repair of a sternal cleft should be performed as early as possible, and the ideal material for this treatment is autologous tissue. We report the use of a titanium plate to repair the sternal cleft in a five-month-old patient with pentalogy of Cantrell because of the size of the defect and the limited availability of autologous tissue.

The Chondroepitrochlearis Muscle: A Rare Cause of Axillary Vein Thrombosis and Lymphedema.

The chondroepitrochlearis muscle is a rare anomaly of the pectoralis major crossing over the neurovascular bundle in the axilla. Often associated with other supernumerary muscles like the arch of Langer, it has been reported in the past as a cause of restriction of arm abduction, cosmetic defects and compression of the ulnary nerve. This case report describes the first known vascular complication due to a chondroepitrochlearis muscle, causing intermittent compression of the axillary vein and thrombosis, associated with pain, upper limb lymphedema, and impaired movements. The diagnosis was suspected from the medical history and confirmed by palpation and dynamic ultrasonography. Surgery was performed to divide the muscle slip with the help of lymphofluoroscopy to prevent damage to the lymphatic structures. Pain and impairment of movement disappeared within a few days after surgery and lymphedema decreased significantly.

Accessory Soleus: A Case Report of Exertional Compartment and Tarsal Tunnel Syndrome Associated With an Accessory Soleus Muscle.

An accessory soleus muscle is a rare anatomic variant that frequently presents as an asymptomatic soft tissue swelling in the posteromedial ankle. Less frequently, the anomalous muscle can cause pain and swelling with activity. We present the case of a 17-year-old male with exertional compartment syndrome and associated tarsal tunnel syndrome secondary to a very large accessory soleus muscle. After surgical excision, the patient was able to return to full activity with complete resolution of symptoms.

Symptomatic Os Subtibiale Associated with Chronic Pain Around the Medial Malleolus in a Young Athlete.

An os subtibiale is a rare accessory bone located below or behind the medial malleolus. Herein we present a rare case of a painful os subtibiale in a young triathlete who presented with pain, redness and swelling below his left medial malleolus. Plain radiographs and three-dimensional computed tomography revealed a well-defined oval bone distal to the left medial malleolus. After conservative treatment failed, the ossicle was excised in an open surgery with complete resolution of symptoms. This case report emphasizes the need for clinical awareness of different anatomical variations of the bones of the foot.