Breast Hypertrophy: A Real Pain in the Back.

BACKGROUND: Bilateral breast hypertrophy comes with signs and symptoms ranging from mild to debilitating. Bilateral breast reduction (BBR) is one of the most frequently performed plastic surgery procedures, and its effects on parameters such as spinal balance, paraspinal muscle function, and physical performance have not been thoroughly evaluated. The objective of this study was to evaluate the effects of BBR using advanced spine imaging modalities, and pain resolution. METHODS: A prospective, observational, cohort study was carried out at the McGill University Health Centre. The following measures were recorded preoperatively and postoperatively for each patient: patient questionnaires (BREAST-Q and Pain), magnetic resonance imaging, and EOS low-radiation spinal scan. RESULTS: Significant postoperative pain reduction was recorded, and there was up to 148% improvement in physical tests. Improvement in all questionnaire and BREAST-Q categories was documented. Preoperative and postoperative magnetic resonance imaging did demonstrate a statistically significant absence of permanent anatomical skeletal sequelae. Postoperative improvement in thoracic kyphosis was documented. CONCLUSIONS: Quality-of-life scores are uniformly improved following BBR. Key findings following BBR include significant pain reduction and no evidence of spinal skeletal change. This is a finding of major importance in view of the practice of many insurance companies/third-party payer and health care systems that use the Schnur scale. The Schnur scale associates a weight for resection with body size that is not directly predictive of pain relief. This may indicate the need for more precise or different guidelines based on these quantitative findings. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

The U-Turn Tournedos (UTT) Flap: A Technical Surgical Evolution for Deep Complex Calvarial Defects.

ABSTRACT: Patients treated for complex oncological calvarial defects are at a higher risk of severe complications (38%): infection, meningitis, dehiscence, and hardware/brain exposure. The patient cohorts at our center have led to the development of the "U-Turn" technical (UTT) addition of our previously reported turnover "tournedos" myocutaneous latissimus dorsi free flap. This allows for an improved ability to fill these large, round, complex defects, maintaining the safety of our original surgical technique, while improving aesthetic outcomes.A single-institution case series of complex microsurgical reconstructions for full-thickness oncologic calvarial defects using the UTT addition was reviewed. A free 30 cm latissimus dorsi myocutaneous flap was harvested, deepithelialized in-situ, and turned over with the dermal component laying on the avascular reconstructed dura. Both ends were positioned next to each other into a U shape and sutured together, creating a 15 cm round paddle.Fifty two complex microsurgical procedures for oncological calvarial defect reconstruction were performed. The 7 most recent were ideal for the UTT addition. There were no instances of microvascular thrombosis, infection, cerebral spinal fluid leak, or major wound healing problems. All procedures provided stable volume and full coverage, with all patients requiring debulking and contouring to achieve optimal aesthetic results. All flaps remained stable after debulking.The UTT addition takes the previously established "tournedos" latissimus dorsi free flap to another level of reconstruction, providing a larger volumetric filler, round shape, better defect filling, better durability, and better aesthetics, even in irradiated and/or infected calvarial chronic wound bed.

A Review of Frontal Orbital and Frontal Sinus Fractures and Associated Ocular Injuries - Level I Trauma Center - University Hospital Experience.

BACKGROUND: Traumatic frontal fractures result from high force injuries and can result in significant morbidity and mortality. The purpose of the current study is to evaluate our Montreal General Hospital (MGH) experience with frontal bone fractures. METHODS: A comprehensive review of our trauma database was performed. All adult patients (>18 years) presenting with a diagnosis of frontal sinus fracture were identified. A thorough retrospective electronic medical records search was performed and relevant data extracted. Specifically, all cases of ocular injury or sequelae were identified and an in-depth review was performed. RESULTS: Between 2008 and 2014, 10,189 trauma patients presented to the MUHC Level 1 trauma center. A total of 1277 patients presented with a facial fracture and 140 had a frontal sinus fracture. The mean age was 43.5 years, 90% were male and the mean hospitalization time was 16.2 days. A significant proportion of patients suffered concomitant craniomaxillofacial fractures including orbital (79%), maxillary (66%), nasal (64%), zygomaticomaxillary complex (34%), nasoorbitoethmoid (31%), Lefort types I-III (18%), and mandibular (8%). Associated cervical spine injuries were documented in 16% of patients. Ocular injuries were present in 30% of subjects. 26% of patients had some form of permanent sequelae from their trauma, mainly neurological. CONCLUSIONS: Due to the intimate association of the frontal bones with the brain and the orbits, frontal sinus fractures demand a sophisticated multidisciplinary craniofacial surgical approach. Given the high rate of ocular injury of 30% as well as severe systemic injuries, the authors propose a modified treatment algorithm for these complex cases.

The Effects of Breast Reduction on Back Pain and Spine Measurements: A Systematic Review.

The aim of this review article was to synthesize the literature on reduction mammaplasty and its effects on the spine. The particular focus was to find these few radiological studies and those investigating changes in spinal angles, posture, center of gravity, and back pain reduction. METHODS: We performed a thorough review of the literature, searching the Medline database for all relevant published data studying reduction mammaplasty and the spine. The search yielded 107 articles of which 11 articles met our specific inclusion criteria. The primary outcome measures of the studies and their respective results were tabulated, contrasted, and compared. RESULTS: The 11 cohort studies included in this review cover the period from 2005 to 2015 and focus on breast hypertrophy and spine. According to these 11 quantitative studies, breast hypertrophy causes objective, quantitative, measurable disturbances to women living with this condition. Reduction mammaplasty produces an unmistakable improvement in signs, symptoms, and quantifiable measures. Although the majority of included articles in this review described postoperative improvement in spinal angles, there remain discrepancies of results between them. CONCLUSIONS: The studies included in this review did offer a promising glimpse into the complex interaction between breast hypertrophy and the spine. However, future research initiatives can improve upon what these investigators have begun with more refined, objective, radiological evidence. More specifically, we aim to clarify some of the basic hypotheses in our center with the use of EOS.

Transcranial Blade Injuries and Principles of Their Safe Extraction.

Retained cranial blade injuries are uncommon events lacking standardized recommendations for appropriate surgical extraction. The authors present a case of a 30-year-old male who sustained a penetrating blade injury of the left orbit with intracranial extension through the skull base into the temporal lobe. The patient walked to the emergency room and remained alert. Clinically, the patient had only a small laceration of the left upper eyelid with no gross visual impairment.The radiological investigation confirmed the presence of a knife blade in the orbit. Intraoperative management included an intracranial approach and an extracranial craniofacial dissection for blade visualization and soft tissue protection, globe protection and to avoid any major bleeding. A thorough review of the penetrating cranial injuries literature is presented and a trauma management algorithm is offered for the care of similar injuries.

Botulinum Toxin A: A Novel Therapeutic Modality for Upper Extremity Chronic Regional Pain Syndrome.

BACKGROUND: Complex regional pain syndromes (CRPS) is a disease that is poorly understood. It is a chronic pain syndrome characterized by sympathetic disruptions as well as CNS sensitization. Botulinum Toxin-A has been shown to have efficacy in Raynaud's as well as other neuropathic pain disorders. Perhaps BTX-A warrants experimentation in the treatment of CRPS. METHODS: Patients with CRPS refractory to 2 or more regional sympathetic nerve blocks in 2007 were included in the study. Patient's were asked to rank their initial pain on a visual analog scale of 0 to 10 (10 being the worst pain). "Tenderness maps" were marked on patient's areas of most pain in 1 by 1 centimeter grids. Each box on the grid was injected with 10 IU of BTX-A after nerve blocks with 1% lidocaine. Treatment sessions occured on a monthly basis with VAS pain scores being re-assessed immediately before the new treatment. t Test, linear regression, and Cohen's D-test were used to analyze the correlation of the data. RESULTS: Study sample was 20 patients. Etiology of CRPS was 6 amputations, 4 crush injuries, 4 penetrating injuries, and 2 lacerations. Average pain reduction on VAS scale achieved was 2.05 points. Average percentage pain reduction was 22.94%. Cohen's D Test also showed a meaningful difference with a score of 1.01. Linear regression R2 = 0.491. Maximum pain reduction, on average, was achieved by treatment 9. CONCLUSION: Despite the esoteric etiology of CRPS, BTX-A has a well-demonstrated mechanism of effect. BTX-A should be further explored as a treatment modality for CRPS.

The Impact of Implant Location on Breast Cancer Characteristics in Previously Augmented Patients: A Systematic Literature Analysis.

BACKGROUND: There is a paucity of data comparing the oncologic properties of breast cancer among patients previously having undergone breast augmentation in either the subglandular or subpectoral planes. The objective of the present systematic review was to evaluate whether implant location influenced the characteristics of breast tumors in previously augmented women. METHODS: A systematic literature search was performed to identify relevant articles reporting tumor characteristics in augmented patients. The search included published articles in three electronic databases; Ovid MEDLINE, EMBASE, and PubMed. Comparative studies (subglandular vs. subpectoral) were included. RESULTS: Analysis of data pooled from the included studies showed that subglandular implants had a higher frequency of tumors between 2 to 5 cm (26.5% vs. 9.9%, P = 0.0130). Subglandular implants also had a higher frequency of stage 2 tumors (42.9% vs. 23.7%, P = 0.0308). There was no significant difference in lymphovascular invasion between the 2 groups. These results of this systematic review suggest that the prognosis of patients undergoing augmentation is unaffected by implant location (subpectoral vs. subglandular). CONCLUSIONS: With the absence of large randomized controlled trials, our study provides surgeons with an evidence-based reference to improve informed consent with regards to implant placement.

Single-Stage Breast Reconstruction Using an All-In-One Adjustable Expander/Implant.

BACKGROUND: When tissue expansion is necessary in breast reconstruction, a single-stage approach is possible using adjustable expander/implants, with or without the use of acellular dermal matrix. We aimed to present the senior author's single-stage experience over a period of 12 years using combined expander/implants in breast reconstruction. METHODS: This is a Single-institution, retrospective review of breast reconstruction with combined expander/implants from 2002 to 2014. Logistic regression was performed to evaluate the impact of multiple variables on long-term outcomes. RESULTS: A total of 162 implants in 105 patients were included in this study. Mean follow-up time was 81.7 months (SD, ± 39.2; range, 15-151). Complication rates were as follows: 0.62% extrusion, 1.2% mastectomy flap necrosis, 1.2% hematoma, 1.9% dehiscence, 2.5% seroma, 4.9% infection, and 15.4% deflation. The following associations were identified by logistic regression: adjuvant radiotherapy and capsular contracture (P = 0.034), tumor size and deflation (P = 0014), and smoking history and infection (P = 0.013). CONCLUSIONS: Overall, 81% of breasts were successfully reconstructed in a single stage. Single-stage reconstruction using all-in-one expander/implants reduces costs by eliminating the need for a second procedure under general anesthesia and can achieve results comparable with other alloplastic reconstructions reported in the literature.

Transverse Cervical Artery: Consistent Anatomical Landmarks and Clinical Experience with Its Use as a Recipient Artery in Complex Head and Neck Reconstruction.

BACKGROUND: Many head and neck reconstructions occur in patients with extensive history of surgery or radiation treatment. This leads to complicated free flap reconstructions, especially in choosing recipient vessels in a "frozen neck." The transverse cervical artery is an optimal second-line recipient artery in head and neck reconstruction. METHODS: Seventy-two neck sides in 36 cadavers were dissected, looking for the transverse cervical artery and transverse cervical vein. Anatomical location of these vessels, their diameter, and length were documented. A retrospective analysis on 19 patients who had head and neck reconstruction using the transverse cervical artery as a recipient artery was undertaken as well with regard to outcome of procedures, reason for surgery, previous operations, and use of vein grafts during surgery. RESULTS: The transverse cervical artery was present in 72 of 72 of cadaveric specimens, and was infraclavicular in two of 72 specimens. Transverse cervical artery length ranged from 4.0 to 7.0 cm, and the mean diameter was 2.65 mm. The transverse cervical vein was present in 61 of 72 cadaveric specimens, the length ranged from 4.0 to 7.0 cm, and the mean diameter was 2.90 mm. The transverse cervical artery averaged 33 mm from midline, and branched off the thyrocervical trunk at an average 17 mm superior to the clavicle. Transverse cervical artery stenosis was markedly less in comparison with external carotid artery stenosis. In a 20-year clinical follow-up study, the transverse cervical artery was the recipient artery in 19 patients. A vein graft was used in one patient, and no flap loss occurred in any of the 19 patients. CONCLUSION: The transverse cervical artery is a reliable and robust option as a recipient artery in free flap head and neck reconstruction.

CD109, a novel TGF-ß antagonist, decreases fibrotic responses in a hypoxic wound model.

Excessive extracellular matrix deposition that occurs in many fibrotic skin disorders such as hypertrophic scarring and scleroderma is often associated with hypoxia. CD109 is a novel TGF-ß co-receptor and TGF-ß antagonist shown to inhibit TGF-ß-induced extracellular matrix protein production in vitro. We examined whether CD109 is able to regulate extracellular matrix deposition under low oxygen tension in vivo using transgenic mice overexpressing CD109 in the epidermis. By creating dorsal bipedicle skin flaps with centrally located excisional wounds in these mice and their wild-type littermates, we generated a novel murine hypoxic wound model. Mice were sacrificed on 7 or 14 days post-wounding, and tissues were harvested for histological and biochemical analysis. Hypoxic wounds in both transgenic and wild-type mice showed increased levels of HIF-1α and delayed wound closure, validating this model in mice. Hypoxic wounds in CD109 transgenic mice demonstrated decreased collagen type 1 and fibronectin expression, and reduced dermal thickness on day 7 post-wounding as compared to those in wild-type mice and to non-hypoxic control wounds. These results suggest that CD109 decreases extracellular matrix production and fibrotic responses during hypoxic wound healing. Manipulating CD109 levels may have potential therapeutic value for the treatment of fibrotic skin disorders associated with poor oxygen delivery.

CD109 overexpression ameliorates skin fibrosis in a mouse model of bleomycin-induced scleroderma.

OBJECTIVE: Transforming growth factor ß (TGFß) is a profibrotic cytokine, and its aberrant function is implicated in several types of fibrotic pathologies including scleroderma (systemic sclerosis [SSc]). Multiple lines of evidence show that increased TGFß signaling contributes to progressive fibrosis in SSc by promoting fibroblast activation, excessive extracellular matrix (ECM) deposition, and dermal thickening. We have previously identified CD109 as a TGFß coreceptor and have shown that it antagonizes TGFß signaling and TGFß-induced ECM expression in vitro in human keratinocytes and fibroblasts. The aim of the present study was to examine the ability of CD109 to prevent skin fibrosis in a mouse model of bleomycin-induced SSc. METHODS: Transgenic mice overexpressing CD109 in the epidermis and their wild-type (WT) littermates were injected with bleomycin in phosphate buffered saline (PBS) or with PBS alone every other day for 21 days or 28 days. Dermal thickness and collagen deposition were determined histologically using Masson's trichrome and picrosirius red staining. In addition, collagen and fibronectin content was analyzed using Western blotting, and activation of TGFß signaling was examined by determining phospho-Smad2 and phospho-Smad3 levels using Western blotting and immunohistochemistry. RESULTS: Transgenic mice overexpressing CD109 in the epidermis showed resistance to bleomycin-induced skin fibrosis, as evidenced by a significant decrease in dermal thickness, collagen crosslinking, collagen and fibronectin content, and phospho-Smad2/3 levels, as compared to their WT littermates. CONCLUSION: Our findings suggest that CD109 inhibits TGFß signaling and fibrotic responses in experimental murine scleroderma. They also suggest that CD109 regulates dermal-epidermal interactions to decrease extracellular matrix synthesis in the dermis. Thus, CD109 is a potential molecular target for therapeutic intervention in scleroderma.

Delayed repair of lateral orbital wall and orbital floor fracture.

Timing of surgical management of facial fractures may differ from the current standard when an associated severe ophthalmologic injury exists; this may necessitate a delay in repair to protect the patient's vision. We set out to demonstrate that good functional and aesthetic outcomes can be achieved in cases where zygoma bone fracture repair had to be delayed for more than 21 days after injury with a specific surgical plan.Using the electronic database from Montreal General Hospital, a level I trauma center, a retrospective review of all facial fractures occurring from 1994 to 2009 was performed. Our review returned a total of 22,727 trauma patients admitted to Montreal General Hospital. Of these, 2672 sustained 1 or more facial fractures. Among the facial trauma patients, 945 patients' injuries included a zygoma fracture, 324 (35.3%) of which were managed operatively. Seven cases had their surgery delayed for more than 21 days after injury, 2 of which were managed using simple osteotomies and plate fixations, without the need for bone grafts. Both patients demonstrated a satisfactory outcome with improvement relative to their preoperative status. We provide a detailed description of our most recent case of a 73-year-old woman in whom the surgical repair of the lateral orbital rim and orbital floor was delayed for 10 weeks to allow healing of her associated traumatic retinal detachment.For patients with associated ocular injuries who cannot undergo orbital fracture repair within 21 days of the trauma, delayed surgery may still result in an acceptable aesthetic result.

Complex scalp, skull, and dural defect reconstruction using a turnover "tournedos" myocutaneous free flap.

BACKGROUND: The latissimus dorsi flap is one of the most commonly used flaps for calvarial defect reconstruction. In the setting of radiation and/or chronic infection and when skeletal reconstruction of the cranium is not recommended, standard calvarial reconstruction needs to be refined. The standard use of the latissimus dorsi only was associated with potential dead space over the dura, limited skin paddle size, and potential external contour irregularities. METHODS: In this study, we present our approach to complex calvarial reconstruction with free tissue transfer without bone grafting while avoiding contour deformities in 1 efficient surgical procedure. We propose the "tournedos" turnover de-epithelialized latissimus dorsi flap, which provides stable dermal and subdermal tissue that will not undergo atrophy over time over the dura. To reach an adequate aesthetic result, we used a uniform, unmeshed, thick split-thickness skin graft over the muscular portion of the tournedos flap. Patients who underwent this procedure, from March 1992 to March 2012, at McGill University Health Center and the Montreal Neurological Institute were included. RESULTS: Thirty-three complex microsurgical procedures for oncologic calvarial defect reconstructions were performed from March 1992 to March 2012. Among them, 6 patients benefited from the tournedos procedure. The average age was 74 years. Scalp defects sizes ranged from 4 × 10 to 16 × 18 cm (40-288 cm). All latissimus dorsi flap donor sites were closed primarily. Patients kept their donor-site drains for approximately 18 days (±5 days). One patient developed a seroma at the donor site after drain removal that was treated conservatively. All patients were satisfied with their reconstructions, and no secondary procedures were necessary. We present our most recent case with good photographic documentation. CONCLUSIONS: Our approach using the tournedos turnover de-epithelialized free flap provides durable and stable coverage for irradiated and/or previously infected calvarial defects. It is a safe procedure in those challenging complex cases and offers many advantages.

Management of severe craniofacial vascular malformation operated under bypass: evolution from a "no-flow" to a "low-flow".

BACKGROUND: Cardiopulmonary bypass (CBP) and circulatory arrest as an assist in the surgical excision of a severe facial vascular malformation were first described by Mulliken et al in 1979. Later on, its use had expanded for resection of intracranial vascular malformations. However, to date, there have not been any published series of these procedures being used in the resection of craniofacial vascular malformations. We sought to review the first 10 surgical procedures performed at McGill University Health Centre for large vascular malformations resection using hypothermic CBP with or without circulatory arrest. METHODS: All consecutive patients at the McGill University Health Centre who had a craniofacial vascular malformation resected with the aid of CBP were reviewed. A comparison of the classic midline sternotomy with cardiac arrest to percutaneous femoral bypass with hypothermic "low flow" was performed. Charts were reviewed for the operative intervention including bypass parameters and short- and long-term complications of the procedure. RESULTS: Cardiopulmonary bypass was used in 9 patients for 10 surgical procedures for the resection of a variety of craniofacial vascular malformations from 1987 to 2001. All lesions had sclerotherapy and embolization of the feeding vessels 72 to 96 hours preoperatively. The average age of our patients was 21 ± 13.4 years (2-37 years). Procedures were conducted via either an open bypass or a closed femoral approach. There were no mortalities. There were 2 major cardiac intraoperative complications and 1 major postoperative complication, which were managed with no sequelae. The average length of postoperative hospital stay was 10 days. All patients went on to full recovery. The blood transfusions varied from 10 U to 0 U for our last patient. CONCLUSIONS: The assistance and adjunct of CBP are a useful procedure in the resection of very large vascular malformations, in selected cases. There were no major long-term complications in this series. With the evolution of our approach, the use of complete circulatory arrest was not required in the majority of cases, and an adequate resection was usually possible with the low-flow state alone as we developed this technique with more experience through the process.

Spatial and temporal localization of WNT signaling proteins in a mouse model of distraction osteogenesis.

While the surgical procedure of distraction osteogenesis (DO) is very successful in the treatment of orthopedic conditions, its major limitation of slow bone formation in the distracted gap has prompted numerous attempts to understand and accelerate this slow bone formation. Interestingly, WNT/FZD signaling has been identified as a critical pathway in mediating bone formation and regeneration but has not yet been studied in the context of DO. The objective of this study was to determine the spatial and temporal localization of endogenous WNT signaling proteins at various times of bone formation in a wild-type mouse model of DO. In this study, the DO protocol performed on mice consisted of three phases: latency (5 days), distraction (12 days), and consolidation (34 days). Our immunohistochemical findings of distracted bone specimens show an increased expression of WNT ligands (WNT4 and WNT10A), receptors (FZD1 and 2, LRP5 and 6), ß-catenin, and pathway antagonizers (DKK1; CTBP1 and 2; sFRP1, 2, and 4) during the distraction phase, which were then down-regulated during consolidation. This is the first published report to show an activation of the WNT pathway in DO and could help identify WNT as a potential therapeutic target in accelerating bone regeneration during DO.

Intravenous heparin use in digital replantation and revascularization: The Quebec Provincial Replantation program experience.

BACKGROUND: No consensus exists among microsurgeons regarding the role of intravenous (IV) heparin in digital replantation/revascularization. The current experience of the Provincial Replantation Center in Quebec was reviewed over a 4-year period. METHODS: An initial retrospective review of all revascularized or reimplanted digits at our Replantation Center from April 2004 to April 2006 was conducted. Then, data of all patients treated at our center from January 08 to September 08 were prospectively collected. The two cohorts were compared with regards to demographics, injury characteristics, postoperative thromboprophylaxis medication as well as complication and success rates. Proportions were compared using χ(2) tests/Fisher's exact tests. Multivariate analysis was conducted with logistic regression. RESULTS: 175 digits were treated from April 2004 to April 2006, including 104 revascularizations and 71 amputations. IV heparin was used in 35.1% of the cases and was associated with a 3.59-fold (95% CI, 1.55-8.31) increase risk of developing a complication compared with cases where heparin was not used (P = 0.001). In 2008, 106 digits were treated. IV heparin was used in 14.6% of the cases and was not significantly associated with a higher complication rate compared with cases where heparin was not used (P = 0.612). Both cohorts' success rates were very similar (P = 0.557). The number of complications decreased from the first period (20.5%) to the second one (12.8%). CONCLUSION: Routine use of IV heparin following digital replantation and revascularization is not warranted. Surgical technique and type of injury remains the most important predictors for success in these complex procedures.

Utility assessment of body contouring after massive weight loss.

BACKGROUND: The number of surgical procedures performed for obesity and massive weight loss (MWL) is increasing. The authors set out to quantify the health state utility assessment of living with MWL that can occur after such procedures. METHODS: Utility assessments using the visual analog scale (VAS), time trade-off (TTO), and standard gamble (SG) were used to obtain utilities for MWL, monocular blindness, and binocular blindness from a sample of the general population and medical students. RESULTS: All the measures for MWL of the 100 volunteers (VAS, 0.79 ± 0.13; TTO, 0.89 ± 0.12; SG, 0.89 ± 0.15) were significantly different (p < 0.005) from the corresponding measures for monocular blindness (0.63 ± 0.18, 0.84 ± 0.17, and 0.86 ± 0.16, respectively) and binocular blindness (0.31 ± 0.17, 0.63 ± 0.28, and 0.66 ± 0.27, respectively) except for the SG utility measure comparing monocular blindness with MWL. Age was inversely proportional to the TTO utility scores for MWL (p < 0.05). Caucasian race and medical education were independent predictors of SG utility scores (p < 0.05). CONCLUSION: In a sample of the general population and medical students, SG utility assessments for MWL were comparable with those for monocular blindness. Utility assessment of living with MWL varied with race (VAS and SG) and education (SG). The sample population, if faced with MWL, would consent to undergo a procedure such as body contouring with an 11% chance of death and be willing to trade 4 years of their life.

Autogenous bone graft for expansion thoracoplasty in Adam Robert Wright syndrome: a case report and review.

We present a direct anterior sternal split expansion as a surgical option for a case of severe Thoracic Insufficiency Syndrome (TIS) in an arthrogryposis-like patient. This patient's clinical features were published as a newly described syndrome: Adam Robert Wright Syndrome. The patient born with this syndrome displays characteristic craniofacial abnormalities, severe thoracic insufficiency syndrome, cleft palate, limb contractures, arthrogryposis, pulmonary hypoplasia, cryptorchidism, ophthalmoplegia and retinopathy, with normal intelligence. His severe thoracic insufficiency necessitated an urgent life-saving surgical intervention for a progressively worsening sleep apnoea and respiratory distress. We present a review of published data of sternal expansion thoracoplasty from 1965 to 2007 found in the literature. We demonstrate that direct anterior sternal split thoracoplasty with autogenous rib grafts is an effective technique for the acute management of thoracic insufficiency syndrome in this specific case. This procedure provided our patient with symptomatic benefit. To our knowledge, this is the only reported surgical management of thoracic insufficiency syndrome demonstrating a statistical improvement in chest wall compliance and tidal volume. We show that direct anterior sternal split expansion is a surgical treatment option in some patients with thoracic insufficiency syndrome. Our surgical strategy for the management of severe thoracic insufficiency syndrome in Adam Robert Wright Syndrome provided symptomatic relief and favourable long-term results.