BACKGROUND:Cleft lip and palate are among the most common congenital craniofacial conditions and require prolonged, coordinated care across surgical, orthodontic, speech, audiologic, and psychosocial domains. National pathway documents emphasize early access, centralization of complex care, and structured multidisciplinary follow-up. The Smile House Network was developed in Italy to operationalize a hub-and-spoke model for cleft care. METHODS:We performed a descriptive analysis of the Smile House Network's publicly reported 2023 activity volumes, extracting center-level counts of primary and secondary cleft-related surgical procedures and associated multidisciplinary outpatient care (orthodontic therapy, speech therapy, and psychological counseling). These results were contextualized and compared against national pathway evidence from AGENAS, including Italian population-based indicators (2017-2019) on the timing of primary cleft repair and case volumes. RESULTS:In 2023, the Smile House Network reported 342 primary surgical procedures and 180 secondary procedures (total 522). Secondary surgery included 41 alveolar bone grafts, 26 orthognathic procedures, 28 revisions, 17 labioplasties, and 38 rhinoplasties. Across the network, multidisciplinary care volumes included 2459 orthodontic therapy encounters/patients, 1372 speech therapy encounters/patients, and 409 psychological counseling encounters/patients. Activity was concentrated in hubs for surgery and in selected sites for high outpatient volumes, reflecting differentiated hub-and-spoke functions. Compared with national indicators, the network's primary surgical volume approximated the expected annual volume of primary repairs derived from Italian birth cohorts, supporting the feasibility of a coordinated national model. CONCLUSIONS:The Smile House Network's 2023 volumes demonstrate a mature, system-level implementation of multidisciplinary cleft care, combining substantial surgical throughput with high-volume allied-health services. When interpreted alongside AGENAS pathway evidence, these data support the scientific and organizational rationale for a structured hub-and-spoke network to deliver timely primary repair, manage long-term sequelae, and standardize longitudinal follow-up.

Lipodystrophy (LD) indicates a group of rare disorders, with generalized or partial loss of white adipose tissue (WAT) often associated with metabolic derangements. Heterogeneity/wide spectrum of the disease and lack of biomarkers make diagnosis often difficult. MicroRNAs are important to maintain a correct WAT function and WAT is a source of circulating miRNAs (cmiRs). miRNAs from 320 family were previously detected in the WAT and variably associated to the metabolic syndrome. Our aim was then to investigate if LD can result in altered abundance of cmiRs-320. We collected samples from a cohort of LD subjects of various subtypes and from age matched controls. Use of quantitative PCR determined that cmiRs- 320a-3p, 320b, 320c, 320e are upregulated, while 320d is downregulated in LD. CmiRs-320 power as classifiers was more powerful in the most extreme and defined forms of LD, including the generalized and the Dunnigan subtypes. cmiR-320a-3p showed significant inverse relationships with plasma leptin (P < 0.0001), typically low in LD. The hepatic enzymes gamma-glutamyl transferase (GGT), aspartate aminotransferase (AST), alanine aminotransferase (ALT) and the marker of inflammation C-reactive protein (CRP) were inversely related to cmiR 320d (P < 0.05, for CRP and GGT; P < 0.01, for AST and ALT). Gene ontology analysis revealed cell-cell adhesion as a process regulated by 320 miRNAs targets, thus disclosing a novel route to investigate origin of WAT loss/dysfunction. In conclusion, cmiRs-320 constitute novel biomarkers of LD, abundance of miR320a-3p is inversely associated to indicators related to WAT function, while downregulation of cmiR-320d predicts an altered hepatic profile and higher inflammation.

Mesangiogenic progenitor cells (MPCs) have been isolated from human bone marrow (BM) mononuclear cells. They attracted particular attention for the ability to differentiate into exponentially growing mesenchymal stromal cells while retaining endothelial differentiative potential. MPC power to couple mesengenesis and angiogenesis highlights their tissue regenerative potential and clinical value, with particular reference to musculoskeletal tissues regeneration. BM and adipose tissue represent the most promising adult multipotent cell sources for bone and cartilage repair, although discussion is still open on their respective profitability. Culture determinants, as well as tissues of origin, appeared to strongly affect the regenerative potential of cell preparations, making reliable methods for cell isolation and growth a prerequisite to obtain cell-based medicinal products. Our group had established a definite consistent protocol for MPC culture, and here, we present data showing MPCs to be tissue specific.

Laminopathies are disorders caused by LMNA gene mutations, which selectively affect different tissues and organ systems, and present with heterogeneous clinical and pathological traits. The molecular mechanisms behind these clinical differences and tissue specificity have not been fully clarified. We herein examine the case of a patient carrying a heterozygous LMNA c.1634G>A (p.R545H) variant with a mild, transient myopathy, who was referred to our center for the suspicion of lipodystrophy. At physical examination, an abnormal distribution of subcutaneous fat was noticed, with fat accumulation in the anterior regions of the neck, resembling the fat distribution pattern of familial partial lipodystrophy type 2 (FPLD2). The R545H missense variant has been found at very low allelic frequency in public databases, and in silico analysis showed that this amino acid substitution is predicted to have a damaging role. Other patients carrying the heterozygous LMNA p.R545H allele have shown a marked clinical heterogeneity in terms of phenotypic body fat distribution and severity of organ system involvement. These findings indicate that the LMNA p.R545H heterozygous variant exhibits incomplete penetrance and highly variable expressivity. We hypothesized that additional genetic factors, epigenetic mechanisms, or environmental triggers might explain the variable expressivity of phenotypes among various patients.

Cleft lip and/or palate patients (CLP) undergo corrective surgery that can affect facial growth. The aim of this study was to analyze facial growth and maxillary development of CLP subjects after surgery according to P.I.S.A. technique (Peri osteoplasty Improves Symmetry and Aesthetic). Cephalometric tracings of 55 patients were performed, thirty-three of which belonged to the test group, while the lasting twenty subjects were part of the control group. The test group was formed by cleft lip and/or palate patients after surgical repair according to P.I.S.A. technique. The control group included patients unaffected by this malformation, with an Angle's first class, selected from the Michigan Growth Study sample. Facial growth and upper maxilla development analysis was carried out by comparing the data obtained from the cephalometric traces of cleft patients who performed an early surgery, with the values of the same parameters measured in non-cleft subjects, providing the normal values. The results of this study showed, in the test group, a maxillary and mandibular bi-retrusion, a more negative facial convexity, the absence of a marked discrepancy in the skeletal relationships, a slight tendency towards hyper-divergence. .

Blepharophimosis, ptosis, and epicanthus inversus syndrome (BPES) is due to heterozygous FOXL2 intragenic mutations in about 70% of the patients, whereas total or partial gene deletions account for a minority of cases. Alteration of FOXL2 regulatory elements has been rarely described in patients with BPES. In this study, a prepubertal girl with BPES due to a 197-kb de novo deletion of the regulatory elements upstream of FOXL2 is reported. This girl presented with additional clinical features such as a soft cleft palate and microcephaly; thus, this copy number variant might have other somatic effects. The present deletion encompasses 2 coding genes (MRPS22 and COPB2), whose homozygous mutations have been associated with microcephaly. In our case, the sequences of the non-deleted allele were normal, ruling out a compound genetic defect. Normal levels of new biomarkers of ovarian reserve (anti-müllerian hormone, inhibin B) likely indicate an early diagnosis of type 2 BPES, but an evolutive gonadal damage will be excluded only by long-term follow-up. Additional reports of microdeletions upstream of FOXL2 are needed to better define the underlying genetic mechanism and the related phenotypic spectrum; the ability of the new hormonal markers to predict ovarian function in adolescence and adulthood should be confirmed.

Sir: We read with great interest the article from Khansa et al. entitled “Evidence-Based Scar Management: How to Improve Results with Technique and Technology.”1 The authors performed a literature review of high-level studies analyzing methods to prevent or improve hypertrophic scars, keloids, and striae distensae. They conclude that, for the treatment of existing hypertrophic scars, silicone, pulsed-dye laser, carbon dioxide laser, corticosteroids, 5-fluorouracil, bleomycin, and scar massage have high efficacy, whereas onion extract and fat grafting seem to have low efficacy. For keloid scars, effective adjuncts to excision include corticosteroids, mitomycin C, bleomycin, and radiation therapy. The appearance and symptoms of established hypertrophic scars may be improved with injection of substances that cause scar atrophy, pulsed-dye laser treatment, pressure garments, and scar massage. We can choose nonsurgical treatment or surgical treatment to achieve the purpose of repair and reconstruction of scar contracture deformity after considering the factors of function and appearance.2 In our opinion, surgical treatment should be taken into consideration by the plastic surgeon. In general, surgical revision of scars should be delayed for at least 12 months.3 The various surgical techniques of revising the scar are as follows: fusiform elliptical excision, Z-plasty, S-plasty, W-plasty, geometric broken-line closure, and V-Y and Y-V advancement techniques.4 The plastic surgeon should be experienced with each of the surgical revision technique and apply these methods as appropriate.5 With this letter, we propose our personal surgical technique (the A-plasty) to revise the scar, in alternative to the previously described techniques, with a different drawing (Fig. 1). The initial drawing represents “A” along the excision line; subsequently, the A is deprived of its triangular component, and only the design of trapezoids along the excision line is kept. We commonly perform the A-plasty in scar revision. Among the reasons why it is useful in scar revision is its ability to make the revised scar not lie in or parallel to a crease line or relaxed skin tension line, as opposed to a W-plasty or Z-plasty.Fig. 1.: A-plasty scar revision technique. The red line is the incision line and the blue line indicates the final outcome.The A-plasty could be an excellent alternative, to avoid a zig-zag incision, especially in delicate anatomical areas, such as the face (Fig. 2). The technique is very easy to execute and can be used in both web and linear contractures. It offers a new option for the correction of linear scar contracture that is safe, simple, and effective. As with any technique, careful preoperative planning and meticulous execution lead to great results.Fig. 2.: Preoperative drawing for a scar revision on the face.DISCLOSURE None of the authors has a financial interest in any of the products, devices, or drugs mentioned in this communication. Nicola Freda, M.D.Alessandro Giacomina, M.D.Gian Luca Gatti, M.D.Plastic and Reconstructive SurgerySanta Chiara HospitalPisa, Italy

INTRODUCTION: Complete clefts lip and palate represent a long journey for patients, families and the cleft team. We have to face a lack of function, symmetry and shape. There are involved soft tissue, muscles, cartilages, bone and all of them are displaced, distorted or missed. In more than 40 years of experience in our department we developed a strong surgical protocol that has a its center the cheilo-rhyno-plasty and periosteoplasty procedure. Within 3 hours procedure we try to restore every single part involved in order to achieve better results and less need of further procedures. MATERIALS AND METHODS: We performed a modified Tennison technique for UCLP and the Mulliken one on BCLP; we used to perform some principles from the early treatment of the nose by Mulliken and, furthermore, we introduced a carefully excision of a semilunar skin flap at the base of the displaced alar cartilage in order to improve the symmetry of the nose. Moreover, in order to restore the lack of maxillary bone, we performed a periosteoplasty described by Massei. The average age was 2,5-3 months. Photographs are taken pre, post operation and at each follow ups. RESULTS: We performed the procedures on UCLP cases and on BCLP ones, from January 2010 to December 2014. We achieved satisfied results in symmetry, muscular reconstruction, correct philtrum shape, length and position and, moreover, a proper shape and structure for the nasal pyramid. Periosteoplasty procedure has decreased the need of bone graft in the 70% of cases. Main complications are: 0,1% of post operative bleeding, 15% of skeletal class III malocclusion. CONCLUSIONS: We do believe that an earlier surgical approach represent the best way to achieve better aesthetic results and less need of further operation. Periosteoplasty procedure is our milestone to decrease the need of bone graft.

The authors present a clinical report of deforming mucocutaneous leishmaniasis of the nose in a native American woman, left untreated for 25 years. The nose was reconstructed using the local tissue displaced as flaps, and using cartilage grafts taken from the nasal septum and the ear shell. To the best of the authors' knowledge, the literature offers just 1 report on a similar patient.

Popliteal pterygium syndrome is a condition characterized by skin webs on the popliteal fossa, which may impair mobility unless surgically repaired. Affected individuals may also have syndactyly on the fingers and/or toes. Most people with this disorder present cleft lip and cleft palate and they can have syngnathia, that is a congenital adhesion between maxilla and mandible by fibrous bands, which affects the opening of the mouth. The case that we report is about a 2-month-old male, who presented skin webs bilaterally on the popliteal fossa, syndactyly between the IV and the V toe of the right foot and between the III and the IV toe of the left foot, and genital malformations. He was born with complete bilateral cleft lip and complete cleft palate on the left side and incomplete cleft palate on the right side and syngnathia with 4 fibrous bands between the mandibular arch and the maxilla arch on the right side, which affected the opening of the mouth. The case of our patient is very interesting because there have been few reported patients affected by popliteal pterygium syndrome with syngnathia.

INTRODUCTION:Cleft lip and palate is the most frequent congenital craniofacial deformity. In this article, the authors describe their experience with cleft lip and palate repair.METHODS:Data regarding patients presenting with primary diagnosis of cleft lip and/or palate, between 2009 and 2015, were reviewed. Details including demographics, type of cleft, presence of known risk factors, surgical details, and follow-up visits were collected. Documented complications were reported. Caregivers' satisfaction was assessed with a survey. The survey used to assess satisfaction with cleft-related features was based on the cleft evaluation profile (CEP). In addition, 4 assessors used visual analog scale (VAS) to assess the aesthetic satisfaction.RESULTS:Seven hundred fifty-two patients with primary diagnosis of cleft lip and/or palate underwent surgical correction at "S. Chiara" Hospital, 432 (57.45%) male and 320 (42.55%) female. The most common cleft types in our study were incomplete cleft palate (152 patients) and left unilateral complete cleft lip and palate (152 patients). Associated syndromes were found in 46 patients (6.12%). Cleft lip was repaired using a modified Tennison-Randall technique when the defect was unilateral, whereas a modified Mulliken technique was used for bilateral cleft lip. Cleft palate was repaired using the Bardach technique or Von Langenbeck technique at 5 to 6 months of age. Cleft lip and palate was repaired in several surgical steps. In total, complications were reported in 81 of 752 patients (14.16%). Average fathers' satisfaction score assessed using CEP was 4.5 (lip), 4.8 (nose), 4.7 (teeth), 4.8 (bite), 4.2 (breathing), 4.6 (profile). Average mothers' satisfaction score assessed using CEP was 4.3 (lip), 4.6 (nose), 4.4 (teeth), 4.5 (bite), 4.1 (breathing), 4.4 (profile). Average level of aesthetic satisfaction, assessed using VAS, was 8.7 (fathers), 8.1 (mothers), 7.9 (lay person), and 8.0 (senior cleft surgeon).CONCLUSION:The multidisciplinary management of children with oral cleft determinated good results, in terms of satisfaction and aesthetic appearance. From our experience, periosteoplasty and lip-adhesion are key surgical techniques.

AIMMale gynecomastia (MG), the most frequent mammary anomaly in human males, is a clinical disease occurring mainly in adolescence and old age. The aim of this study was therefore to analyze 126 consecutive cases of mixed gynecomastia, in order to assess the incidence of early and late postoperative complications and to evaluate the aesthetic results and the quality of life after surgery.METHODSFrom January 1st, 2000, to December 31st, 2006 a total of 126 cases of MG were performed by the Plastic Surgery Units of Siena and Pisa. Patients' average age was 28 years, 111 patients (88%) presented bilateral MG, and 15 (11.9%) had monolateral MG. The prevalent surgical approach was adenomammectomy with periareolar inferior or inverted "Omega" incision, other technique included circumareolar or vertical scar incision and liposuction. Before and one year after surgery, all patients were given a questionnaires to evaluate the motivations leading to the request of a treatment ,the degree of satisfaction related to the result and the improvement of the quality of life.RESULTSOverall complication rate was 17.72% All patients reported an improvement in their quality of life with an average score of satisfaction of 8.2/10.CONCLUSIONPatients' degree of satisfaction was high, surgery, in fact, has contributed in all cases to improve their quality of life. On the basis of the short operating time and of the few sequele, we suggest to the patients affected by gynecomastia to undergo surgery always and as soon as possible. A separated analysis of the data obtained by the two University Centres show that they overlap in respect to the sample, the employed technique and results.

A 24-year-old woman was admitted to our department with a loss of substance of the volar aspect of her right forearm. Surgical debridement and subsequent coverage with skin graft were required. Her symptoms had started 10 months earlier when she had broken her right wrist. Right forearm cellulitis had been diagnosed 2 weeks after the gypsum was removed and treated in several hospitals under different specialties. Because her condition had gradually worsened, she was referred to our unit.

High tension and tissue dislocation caused by wide maxillary clefts could prevent an optimal aesthetic and functional outcome in primary cheilognathoplasty. Many surgical techniques or conservative (orthodontic and orthopaedic) devices have been proposed for the initial management of infants with complete cleft lip and palate to achieve a tension-free repair of the lip. Adhesion converts a wide unilateral complete cleft into a lesser deformity anatomically similar to an incomplete cleft lip. This study aimed to measure the effect of lip adhesion on the width of maxillary clefts. Between 2000 and 2007 lip adhesion was used on 49 consecutive infants affected by complete unilateral (n = 35) and bilateral (n = 14) cleft lip and palate. The indication for lip adhesion was the presence of a wide alveolar cleft (gap > 7 mm) with severely malpositioned maxillary segments. Lip adhesion was done at about 48 days, followed by definitive cheilognathoplasty at 98 days. Photographs and impressions were obtained before any operation. The width of the maxillary clefts was reduced by 60% in unilateral clefts and 61% in bilateral clefts. By converting a complete wide cleft lip to an incomplete cleft in both unilateral and bilateral clefts, adhesion of the lip achieved a better position and stabilised the arch in a symmetrical platform that eased both definitive closure of the defect in the lip and the restoration of the maxillary gap by periosteoplasty during definitive cheilognathoplasty.

Sir: A 35-year-old white woman with a history of penicillin allergy underwent bilateral subpectoral augmentation mammaplasty in March of 2008. Intramuscular (Roche Pharmaceuticals, Basel, Switzerland) (1 vial/24 hours for 2 days) and oral ceftriaxone (500 mg/24 hours for the other 5 days) was administered after the intervention. Thirty-five days after she was discharged to home, the patient was referred to us because a 0.5-cm region of inframammary fold overlying the device broke down and the implant became exposed. She was afebrile and without signs of infection. Thus, we débrided all compromised tissue, opting for simple closure of the wound because sufficient viable soft tissue remained. Although oral antibiotics were administered, unfortunately, 10 days later, the patient presented with fever (40°C) and erythema on the left side, purulent material drainage, and left implant exposure. Pus culture grew Staphylococcus aureus. The abscess was drained (Fig. 1) and the patient placed on intravenous teicoplanin (Targocid; Sanofi-Aventis, Bridgewater, N.J.), 400 mg/24 hours for 5 days. Because the patient desired an attempt at implant salvage, refusing device explantation, she was started on saline, povidone-iodine (Betadine; Purdue Pharma, Stamford, Conn.), and antibiotic (Targocid) irrigations of the submuscular pocket three times per week. In addition, she was treated with 10 cycles of hyperbaric oxygen therapy (2 to 3 atm for 90 minutes each) and oral antibiotics. The patient was readmitted at the end of May by our unit and underwent capsulotomy, implant exchange, and definitive pocket closure with a closed-suction drainage catheter. After the infectious disease unit was consulted, the patient was started on intravenous daptomycin (Cubicin; Cubist Pharmaceuticals, Lexington, Mass.), 350 mg/24 hours for 5 days. At 2 months, the patient was symptom-free, without signs of infection or capsular contracture, and achieved a satisfactory result (Fig. 2).Fig. 1.: Drainage of the abscess through the small exposed region.Fig. 2.: Final cosmetic outcome after left breast implant salvage.Among the potential complications associated with the use of breast prostheses are the risks of periimplant infection and device extrusion, with an infection rate following breast augmentation ranging from 1 to 2 percent.1,2 Traditional recommendations for these problems dictate antibiotic treatment alone and/or device removal, with delayed replacement of the implant. Few reports have described successful techniques for salvage of an infected breast tissue device or salvage of an exposed but not infected implant, whereas no case exists reporting successful management for salvage of an infected and exposed breast implant after cosmetic augmentation. Yii and Khoo3 proposed a combination of capsulectomy and continuous irrigation with saline and intermittent antibiotic instillation to salvage infected expanders in breast reconstruction. Spear and colleagues4 developed treatment guidelines for implant infections, threatened device exposure, and actual device exposure. They submitted patients with severe implant infection and actual exposure (both reconstructive mammoplasties) to device removal posing a 0 percent salvage rate (without a real attempt at salvage). Chun and Schulman5 described the successful salvage of nine consecutive severely infected breast prostheses after mastectomy reconstruction, adopting a technique of immediate intravenous antibiotics followed by early device exchange and a long course of postoperative antibiotics. Salvage of an infected and exposed breast cosmetic implant must achieve two main goals: resolution of the infection and maintenance of the aesthetic outcomes, principally by avoiding device explantation. The described approach provides a means of achieving these objectives and was successful in our patient. DISCLOSURE There are no financial conflicts or interests to report in association with the contents of this article. Gian Luca Gatti, M.D. Davide Lazzeri, M.D. Marco Stabile, M.D. Gianfranco Romeo, M.D. Alessandro Massei, M.D. Plastic and Reconstructive Surgery and Burn Center Unit; Hospital of Pisa; Pisa, Italy

Background: Since antiquity, many attempts have been carried out and a number of theories proposed to explain the process that leads to bone regeneration. Through manifold experiments, several authors tried to understand and subsequently to control the physiological events that enable bone healing. Between the 18th and 19th centuries something changed. Although the scientific world was initially skeptical, a new idea emerged in the field of bone surgery. It principally concerned the necessity to perform amputations and resections leaving intact the periosteum to obtain new bone formation. Materials and Methods: With this article we emphasize the contribution of many authors in the development of knowledge about the osteogenic properties of the periosteum. In particular we focus on the experiences of unknown Italian authors Michele Troja and Bernardino Larghi, consider well-recognized scientific personalities such as Leopold Ollier and Bernhard von Langenbeck, and reach a milestone of plastic surgery with Tord Skoog and his description of periosteoplasty. Conclusion: Various surgical approaches have been proposed to provide optimal care for patients with cleft lip and palate disorders. Among several treatment options, periosteoplasty is one of the choices to correct maxillary clefts. Highlighting difficulties and successes of many authors in demonstrating osteogenic properties of periosteum, this paper describes how periosteoplasty performed in maxillary cleft restoration capitalizes on what has been discovered during a 250-year-long history.

OBJECTIVE:Poland syndrome is a rare congenital anomaly characterized by complete or partial agenesis of the pectoralis major muscle variably associated with other thoracic malformations, upper limb malformations, or both. More than 20 patients with dextrocardia and left-sided Poland syndrome have been previously described. The association between these 2 rare anomalies suggests a causal relationship, but the etiopathogenetic mechanism has not been clarified yet. We studied the clinical correlation between these 2 anomalies, and we tried to elucidate whether dextrocardia or Poland syndrome comes first.METHODS:This is a multicentric multidisciplinary study conducted over the last 5 years. We identified 122 patients with Poland syndrome, and we investigated heart position through different imaging techniques. Logistic regression statistical analyses were carried out.RESULTS:We observed dextrocardia in 14 (11.5%) patients, which was never associated with situs inversus. All of them presented with left-sided Poland syndrome and partial agenesis of 2 or more ribs. Conversely, all patients with Poland syndrome with partial agenesis of 2 or more ribs presented with dextrocardia, whereas dextrocardia was never associated with partial agenesis of a single rib. Three patients with dextrocardia presented with simple congenital heart defects.CONCLUSIONS:These findings suggest that mechanical factors during embryonic life could explain the strong association between left-sided Poland syndrome and dextrocardia. According to this hypothesis, partial agenesis of 2 or more ribs is needed to displace the heart toward the right side. The peculiar features of dextrocardia when associated with Poland syndrome (neither associated with situs inversus nor complex intracardiac anomalies) support our hypothesis.

Most of our knowledge of the effects of aging on the hematopoietic system comes from studies in animal models. In this study, to explore potential effects of aging on human hematopoietic stem and progenitor cells (HSPCs), we evaluated CD34+ cells derived from young (<35 years) and old (>60 years) adult bone marrow with respect to phenotype and in vitro function. We observed an increased frequency of phenotypically defined stem and progenitor cells with age, but no distinct differences with respect to in vitro functional capacity. Given that regeneration of peripheral blood counts can serve as a functional readout of HSPCs, we compared various peripheral blood parameters between younger patients (≤50 years; n = 64) and older patients (≥60 years; n = 55) after autologous stem cell transplantation. Patient age did not affect the number of apheresis cycles or the amount of CD34+ cells harvested. Parameters for short-term regeneration did not differ significantly between the younger and older patients; however, complete recovery of all 3 blood lineages at 1 year after transplantation was strongly affected by advanced age, occurring in only 29% of the older patients, compared with 56% of the younger patients (P = .009). Collectively, these data suggest that aging has only limited effects on CD34+ HSPCs under steady-state conditions, but can be important under consitions of chemotoxic and replicative stress.

OBJECTIVE:This study analyzed histological and histochemical features of specimens of the orbicularis oris muscle, and palatal and pharyngeal muscles biopsied during surgery from 33 patients affected by cleft lip and palate.DESIGN:Three groups were studied: 20 patients affected by cleft palate with or without cleft lip (at the time of primary palatoplasty), seven by cleft lip with or without cleft palate (primary lip closure), and six by cleft lip with or without cleft palate (secondary cheiloplasty). Muscle sections were stained with hematoxylin-eosin, modified Gomori trichrome, ATPase reaction at pH 9.4, and NADH-TR. Analyzed parameters included organization, muscle fiber size and type, nuclear changes, presence of ragged-red fibers, degree of fibrosis, and presence of inflammatory infiltrate.RESULTS:In all patients who underwent primary palatoplasty and lip closure we noted dystrophic-like alterations of orbicularis oris and palatopharyngeal muscles, such as variability of fiber size, fiber disorganization, and increased fibrosis. The same alterations were found in adult patients submitted to secondary cheiloplasty, notwithstanding surgical repair. Furthermore, in all groups neither neurogenic atrophy nor ragged-red fibers or inflammatory infiltrate were detected.CONCLUSIONS:Muscle damage is a constant event in this deformity, and it could play an important role in its etiopathogenesis. Muscular biopsy during cheiloplasty and palatoplasty could offer useful information about muscle condition and possible functional recovery in cleft lip and palate patients.