Clinical Experience of Treatment of Facial Malformations in Oto-Palato-Digital Syndrome: A Familial Patient.

Oto-palato-digital syndrome type 1 (OPD1) is an X-linked recessive disorder comprising characteristic facial appearances and skeletal alterations. The authors report OPD1 in a mother and her 2 sons who had multiple common congenital anomalies. Both of the brothers were born with mild hearing impairment, frontal bossing with prominent supraorbital ridges, downslanting palpebral fissures, dental malocclusion, and palatal clefts. They underwent a series of aesthetic surgeries for their facial malformations with good cosmetic results. The mother had a milder phenotype with less prominent craniofacial defects that did not require surgical correction. The older brother required a 2-jaw surgery whereas the younger brother required a surgically assisted rapid palatal expansion. In the second series of operations, both brothers underwent scraping of their prominent supraorbital ridge. They have been free of complications throughout their 16-year follow-up. The authors also screened the family for possible genetic etiologies and identified mutations in the causative gene of OPD1 on Xq28 in all 3 patients. In conclusion, the authors have identified 3 patients with OPD1, performed surgical treatments on the affected brothers and have obtained good reconstructive results. There are no reports involving OPD1 patients who have received good surgical treatment. With careful examination and identification of patients with characteristic facies and skeletal abnormalities, it is our opinion that the authors can help more patients with this disease through surgical management.

Photosensitizer Effects of Artificial Red Cells on Dye Laser Irradiation in an Animal Model Assuming Port-Wine Stain Treatment.

BACKGROUND: The complete removal of port-wine stains has remained challenging. Based on the principle of treating port-wine stains with a dye laser, intravenous injection of artificial red cells (hemoglobin vesicles) immediately before laser treatment might improve the clinical outcome of the therapy. The hemoglobin vesicle injection increases the hemoglobin concentration in microvessels. Photons of dye laser are absorbed by the mixture of red blood cells and this newly added hemoglobin, potentially producing more heat and photocoagulation and, ultimately, necrosis of the endothelial cells effectively. METHODS: To confirm the performance of hemoglobin vesicles as a photosensitizer, the authors compared the absorbance of hemoglobin vesicles and human blood against 595-nm wavelength and the temperature increases that occur following dye laser irradiation. Furthermore, the authors investigated the microvessel transformation induced by the hemoglobin vesicle intravenous injection. Finally, the authors investigated the effect of the hemoglobin vesicle on the vascular destruction of dye laser irradiation with chicken wattle. RESULTS: Results show that hemoglobin vesicles have the same absorbance as that of human blood. They produce the same level of heat as human blood after laser irradiation. The hemoglobin vesicle intravenous injection caused dilatation of microvessels in animal models. The dye laser with hemoglobin vesicle can destroy the vessel wall effectively in animal models. CONCLUSIONS: Hemoglobin vesicles can function as photosensitizers to destroy the vessel wall. A possible mechanism of pulsed dye laser-resistant port-wine stains is that overly small vessels do not contain sufficient red blood cells. They are therefore poor absorbers/heaters for these lasers. Hemoglobin vesicle combination therapy will improve clinical outcomes of dye laser treatment against such lesions only.

Urethroplasty Using Diverticular Tissue for Hypospadias.

CASE: The patient was a 3-year-old with hypospadias. Urethroplasty was performed as a two-stage procedure, but a severe urethral stricture occurred due to a postoperative infection and the patient was virtually in urinary retention. An emergency cystostomy was performed, and thereafter urethral reconstruction was performed. A large urethral diverticulum had formed in the scrotum. The inner wall of the diverticulum was found to be completely epithelialized and well vascularized. Therefore, it was elevated as a skin flap and a neourethra was reconstructed up to the glans region using the diverticular tissue. OUTCOME: Postoperative complications were not observed, and micturition was satisfactory. CONCLUSION: A urethral diverticulum is one of the often encountered complications of hypospadias surgery. However, there are only a few literature reports related to the treatment of urethral diverticulum. In addition, there are almost no reports on reconstructions using urethral diverticular tissue. In this report, we describe such a treatment experience.

Halo-Type Distraction Device Used to Treat a Severe Midface Depression Fracture.

The deep layers of the nasoethmoid region comprise a thin-walled lacrimal bone, a paper-thin ethmoid plate, and ethmoidal cells, forming an extremely fragile and easily crushed structure. In a nasoethmoid complex fracture where the support structure itself is destroyed, epithesis and immobilization are difficult and can lead to residual saddle nose deformities. Therefore, bone grafts are often necessary at a later date. Recently, the authors occasionally see reports of nasoethmoid complex fractures that are treated with Halo distraction devices. Advantages of this device are that it applies constant traction to maintain the shape of the structures until the ruptured nasoethmoid bone and mucosa are repaired, thus minimizing relapse. There is no need for rigid fixation with a plate, no limitations on how much distraction is possible, and no major skin incisions are required for the approach. The authors treated a 30-year-old man who suffered a severe and widespread depressed facial deformation due to a nasoethmoid fracture that included a midface comminuted fracture using a Halo-type distraction device. A gentle traction was maintained on the nasoethmoid bone and part of the maxilla pulling it forward, and resulted in an extremely good outcome both esthetically and functionally. This method is believed to be extremely useful and effective, requiring only minimally invasive surgery for comminuted midface fractures involving a nasoethmoid fracture with a depressed frontal process of the maxilla. Below, the authors provide a detailed description of their experience with this device.

Regional Oxygen Saturation Index: A Novel Criterion for Free Flap Assessment Using Tissue Oximetry.

BACKGROUND: Tissue oximetry is a useful tool for postoperative free flap monitoring. Reported criterion values have been accurate; however, there are various complicated author-dependent criteria, and sometimes it is too late for flap salvage. The authors offer a new, simple criterion for faster and accurate detection of perfusion problems. METHODS: Intraoperatively, perfusion areas of various free perforator flaps were assessed by both indocyanine green angiography and regional oxygen saturation. The elevated flap was categorized into the early-stained area, the delayed-stained area, and the no-stained area by indocyanine green angiography. The regional oxygen saturation index (regional oxygen saturation on the flap on the control nondissected portion) of each area was calculated. Postoperative continuous flap monitoring was conducted, recording the value of the regional oxygen saturation index at the early-stained area. The blood glucose measurement index was also recorded periodically. RESULTS: In 60 cases of perforator-based free flaps, intraoperative indocyanine green areas were significantly correlated with the values of regional oxygen saturation index. The postoperative regional oxygen saturation index showed very stable values in various types of perforator flaps, provided that no vascular problem occurred, and it never went below 0.75. When vascular problems occurred, the regional oxygen saturation index dropped below 0.75 in all three cases before the blood glucose measurement index and the absolute value of regional oxygen saturation dropped below the criterion value. CONCLUSION: The regional oxygen saturation index may be a simple and fast criterion for detecting vascular problems following free flap reconstruction compared with existing criteria. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, II.

Hyperpigmentation after surgery for a deep dermal burn of the dorsum of the hand: partial-thickness debridement followed by medium split-thickness skin grafting vs full-thickness debridement followed by thick split-thickness skin grafting.

BACKGROUND: Early excision and skin grafting are commonly used to treat deep dermal burns (DDBs) of the dorsum of the hand. Partial-thickness debridement (PTD) is one of the most commonly used procedures for the excision of burned tissue of the dorsum of the hand. In contrast, full-thickness debridement (FTD) has also been reported. However, it is unclear whether PTD or FTD is better. METHODS: In this hospital-based retrospective study, we compared the outcomes of PTD followed by a medium split-thickness skin graft (STSG) with FTD followed by a thick STSG to treat a DDB of the dorsum of the hand in Japanese patients. To evaluate postoperative pigmentation of the skin graft, quantitative analyses were performed using the red, green, and blue (RGB) and the hue, saturation, and brightness (HSB) color spaces. We have organized the manuscript in a manner compliant with the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement. RESULTS: Data from 11 patients were analyzed. Six hands (five patients) received grafts in the PTD group and eight hands (six patients) received grafts in the FTD group. Graft take was significantly better in the FTD group (median 98 %, interquartile range 95-99) than in the PTD group (median 90 %, interquartile range 85-90) (P < 0.01). Quantitative skin color analyses in both the RGB and HSB color spaces showed that postoperative grafted skin was significantly darker than the adjacent control area in the PTD group, but not in the FTD group. CONCLUSIONS: There is a possibility that FTD followed by a thick STSG is an option that can reduce the risk of hyperpigmentation after surgery for DDB of the dorsum of the hand in Japanese patients. Further investigation is needed to clarify whether the FTD or the thick STSG or both are the factor for the control of hyperpigmentation.

Early Detection of Lymphatic Disorder and Treatment for Lymphedema following Breast Cancer.

BACKGROUND: Upper extremity edema following surgical treatment for breast cancer does not always result in chronic breast cancer-related lymphedema. Changes in the findings of indocyanine green lymphography and upper extremity volume following breast cancer surgery were observed prospectively to understand the early changes in lymphatic function and to establish a new strategy for early diagnosis and treatment of breast cancer-related lymphedema. METHODS: Lymphatic function for 196 consecutive breasts was examined using indocyanine green lymphography and bilateral upper extremity volume before surgery and 1, 3, 6, 9, and 12 months after surgery. When a "stardust," "diffuse," or "no flow" pattern was observed, patients were diagnosed with lymphatic disorder and subsequently underwent conservative treatments such as compression sleeve therapy. RESULT: In 35 patients, lymphatic disorder was observed after a mean of 5.2 ± 3.0 months after surgery. In 21 of these patients, no significant limb volume change was observed. In 14 patients, lymphatic disorder and volume change appeared simultaneously. In 11 of 35 patients, lymphatic function improved later, and compression therapy was discontinued. Lymph node dissection, radiation therapy to axillary lymph node, and the use of docetaxel chemotherapy were significant risk factors for lymphatic disorder. No patients experienced cellulitis during the study period. CONCLUSIONS: Lymphatic function disorder could be detected before volume changes. By early intervention with conservative treatments, lymphatic function improved in just under one-third of cases. Indocyanine green lymphography for high-risk patients may be useful for detecting lymphatic disorder early, thereby increasing the chance for disease cure. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Human adipocytes from the subcutaneous superficial layer have greater adipogenic potential and lower PPAR-γ DNA methylation levels than deep layer adipocytes.

Human subcutaneous fat tissue consists of two layers, superficial adipose tissue (SAT) and deep adipose tissue (DAT). Some recent reports suggest that a disproportionate accumulation of DAT is related to obesity-associated metabolic complications. However, the differences in adipocyte function between SAT and DAT are unclear. To clarify the differences in human adipocyte characteristics between SAT and DAT, human ceiling culture-derived proliferative adipocytes (ccdPAs) were primary cultured from SAT and DAT of three lean female patients. Differences in adipogenic differentiation potential and sensitivity to exogenous adipogenic factors were examined. Epigenetic modification of the CpG island DNA methylation levels of genes related to adipogenesis was measured. In histological analyses, the mean adipocyte size in SAT was significantly larger than that in DAT (8,741 ± 416 vs. 7,732 ± 213 µm(2), P < 0.05). Primary cultured adipocytes from SAT showed significantly greater adipogenesis than did those of DAT. Sensitivity to partial adipogenic stimulation was significantly different between ccdPAs of SAT and DAT. Peroxisome proliferator-activated receptor-γ (PPAR-γ) protein expression and leptin protein secretion from ccdPAs were significantly higher in SAT than DAT. DNA methylation levels of PPAR-γ were significantly lower in ccdPAs of SAT than DAT. Adipocyte size was larger in SAT than DAT in vivo. This is consistent with the findings of an in vitro study that, compared with ccdPAs in DAT, ccdPAs in SAT have higher adipogenic potential and lower DNA methylation levels of PPAR-γ.

Combination Treatment of Artificial Dermis and Basic Fibroblast Growth Factor for Skin Defects: A Histopathological Examination.

UNLABELLED: This study examined a combination of artificial dermis and basic fibroblast growth factor (bFGF) to treat skin defects in clinical cases, and it histopathologically examined the effects on the conditions of recipient beds. MATERIALS AND METHODS: The subjects were 11 patients with skin defects from burn ulcers or traumatic ulcers. In each subject, debridement was performed and subsequently artificial dermis was applied to the defect. The bFGF was used on 1 side (combination therapy) of the artificial dermis and not used on the other side (artificial dermis monotherapy). A histopathological examination was performed on the granulation tissue collected from the recipient bed. The authors also measured skin hardness 6 months after the skin graft. RESULTS: Histologically, the combination therapy site had more extensive capillary angiogenesis than the monotherapy site. The combination therapy site also had capillary walls consisting of thick, large endothelial cells; fibroblast proliferation and activation; and more severe infiltration of inflammatory cells. Skin hardness after the graft was also much softer in the combination therapy. CONCLUSION: The results suggest the usefulness of this combination therapy in the preparation of skin graft beds to improve skin hardness after skin grafts in clinical cases.

An anatomical study of the pathophysiology of carotid cavernous sinus fistula associated with Le Fort III osteotomy.

UNLABELLED: Carotid-cavernous sinus fistula (CCF) is a rare complication occurring after the Le Fort osteotomy. We aimed to elucidate the cause of CCF in the Le Fort osteotomy. METHODS: Eleven fresh cadavers were used. After craniotomy, a Le Fort III bipartition osteotomy was then performed. On the left side, both the lateral wall of the maxilla and the pterygomaxillary junction (PMJ) were divided, in addition to the conventional osteotomy line. On the right side, those parts were kept intact. After the osteotomy, a tensiometer was fixed to the skull base. The sensor was linked with the wall of the carotid artery in the cavernous portion. A down-fracture was then performed initially from the left, followed by the right. Tensile force data of both sides were recorded. RESULTS: In all cases, a higher tensile load was observed on the right side compared to the left side. In right side, two skull base fractures complexed with high pterygoid process fractures, and nine pure high fractures of the pterygoid process were identified. CONCLUSIONS: During down-fracture, the wall of the carotid artery experiences significant tensile load. The tensile load on the arterial wall may collapse the fine branches of the carotid artery in the cavernous portion.

An anatomical and histological study of the vascularized iliotibial tract graft.

BACKGROUND: An examination of the vascular anatomy of the iliotibial tract (IT) has not been previously reported. Because a flap resists infection better than an avascular graft, a vascularized IT graft is useful for reconstructive surgeries pertaining to infected wounds or wounds in contact with artificial material. The purpose of this study was to examine the vascular anatomy of the IT. MATERIALS AND METHODS: The study sample consisted of 39 limbs of freshly frozen cadavers. The study was divided into three parts. The ascending and transverse branches of the lateral circumflex femoral artery (LCFA) of all cadavers were injected with latex. Distance from the tensor fasciae latae muscle and the most distal point at which the vessel on the IT was stained by latex was recorded. A microscopic observation was performed for these limbs. The deep femoral artery (DFA) or superior lateral genicular artery (SLGA) was also observed. RESULTS: The length of the IT fed by the LCFA was 162.3 ± 36.2 mm. The IT vascularity was located between the layered structure of the fascia and there was a vascular source for the IT within 1 mm above the IT by optical microscopy. The vascularity derived from the DFA or SLGA was not confirmed in any specimens. CONCLUSIONS: Blood supply of the IT was derived from the LCFA and a vascularized IT graft could be elevated in length to approximately 16 cm. This knowledge may be useful for improving the safety of surgery when transferring an IT flap. © 2015 Wiley Periodicals, Inc. Microsurgery 36:325-329, 2016.

Reconstruction using a divided latissimus dorsi muscle flap after conventional posterolateral thoracotomy and the effectiveness of indocyanine green-fluorescence angiography to assess intraoperative blood flow.

PURPOSE: In most general thoracic operations performed via standard posterolateral thoracotomy, such as for descending aortic aneurysms and lung cancer, the latissimus dorsi (LD) muscle is divided. However, division of the LD can hamper reconstructive surgery because the initial operation creates unstable blood flow to the divided LD. We conducted this study to assess blood flow in a divided distal LD muscle flap using intraoperative indocyanine green-fluorescence angiography (ICG-FA) with the Hyper Eye Medical System(®) (Mizuho Medical Co., Ltd., Tokyo, Japan). METHODS: The subjects were 11 patients who underwent posterolateral thoracotomy with reconstructive surgery using a divided distal LD and other peripheral muscle flaps. Intraoperative ICG-FA was conducted to assess blood flow to the LD. RESULTS: Intraoperative ICG-FA revealed that at least two intercostal perforators from the sixth to the tenth intercostal spaces were preserved as feeding vessels to the divided distal LD. There were no major complications associated with inadequate blood flow to the muscle flaps. CONCLUSION: Intraoperative ICG-FA proved extremely useful for assessing altered blood flow of the divided LD and for selecting preserved intercostal perforators.

Appropriate indication of fronto-orbital advancement by distraction osteogenesis in syndromic craniosynostosis: Beyond the conventional technique.

Currently the distraction technique could be very useful for posterior expansion in syndromic craniosynostosis. Even for fronto-orbital advancement (FOA), it is thought that distraction has the definitive advantage over the conventional technique. The authors describe the appropriate indication of distraction for FOA in our series of craniosynostosis patients. Since 1998, FOA by distraction has been used for 61 cases of simple and syndromic craniosynostosis. Among these, seven severe cases were extracted with a conspicuous craniofacial deformity with a multiple honeycomb appearance in 3DCT views. Early surgery for FOA by distraction to the extent possible was performed and a retrospective study was conducted. Seven children were identified. All of them exhibited a severe craniofacial deformity. Pfeiffer syndrome in 5 children and clover-leaf skull in two unidentified children were diagnosed. Mean age at surgery for FOA by distraction ranged 10-18 months. Mean operative time: 3-3.5 h. Blood loss: 230-320 mL. The mean advancement by distraction: 20-27 mm. We conclude FOA by distraction when a large amount of advancement is required for early severe cases as our series can be definitely one of the most appropriate candidates beyond the conventional technique, despite disadvantages.

Low-rise scar deep inferior epigastric artery perforator flap for breast reconstruction.

BACKGROUND: To achieve an unnoticeable postoperative scar in patients with little abdominal skin laxity for breast reconstruction by deep inferior epigastric artery perforator (DIEP) flap, we devised a new design called the low-rise scar DIEP flap; the skin paddle of this flap is located lower with a smaller vertical width, and more adipose tissue is elevated to obtain enough volume. The purpose of this report is to evaluate the utility of the low-rise scar DIEP flap compared with that of the conventionally designed flap. METHODS: Twelve patients who underwent low-rise scar DIEP flaps (study group) and 11 patients who underwent conventionally-designed DIEP flaps (control group) were included in the present study. The distance from the umbilicus to horizontal scar was divided by the patient's height. The length of the scar was divided by the abdominal circumference. These ratios were compared between groups. RESULTS: All flaps survived completely and no recipient site complication was observed, except for one case in the control group with small-range fat necrosis. No donor site complication was observed in either group. The distance ratio was significantly larger in study group (<0.01, 0.049 ± 0.004, and 0.028 ± 0.005, respectively). The length ratio was significantly smaller in the study group <0.01, 0.42 ± 0.02 and 0.36 ± 0.02, respectively). The weight of the flap used for reconstruction was 338.8 ± 127.7 g in the study group and 320.5 ± 63.0 g in the control group CONCLUSIONS: A low-rise scar DIEP flap leaves a lower and shorter postoperative scar.

Low-dose radiation pretreatment improves survival of human ceiling culture-derived proliferative adipocytes (ccdPAs) under hypoxia via HIF-1 alpha and MMP-2 induction.

Poor survival is a major problem of adipocyte transplantation. We previously reported that VEGF and MMPs secreted from transplanted adipocytes are essential for angiogenesis and adipogenesis. Pretreatment with low-dose (5 Gy) radiation (LDR) increased VEGF, MMP-2, and HIF-1 alpha mRNA expression in human ceiling culture-derived proliferative adipocytes (hccdPAs). Gene expression after LDR differed between adipose-derived stem cells (hASCs) and hccdPAs. Pretreatment with LDR improved the survival of hccdPAs under hypoxia, which is inevitable in the early stages after transplantation. Upregulation of VEGF and MMP-2 after LDR in hccdPAs is mediated by HIF-1 alpha expression. Our results suggest that pretreatment with LDR may improve adipocyte graft survival in a clinical setting through upregulation of VEGF and MMP-2 via HIF-1 alpha.