Comparative study of internal device vs. external device in Le Fort III distraction for syndromic craniosynostosis.

BACKGROUND: Le Fort III distraction for syndromic craniosynostosis is performed using internal or external devices. We compared the results of both devices. PATIENTS AND METHODS: We retrospectively evaluated 60 patients with syndromic craniosynostosis treated with Le Fort III distraction (internal or external device) between 2001 and 2021. We verified demographic data, surgery-related data, and complications using medical records. For each of the two devices, we compared the various factors associated with the device to each other. RESULTS: External deviceThirty-two patients with syndromic craniosynostosis were included. The mean age at surgery was 11.7 years, and the mean elongation length was 20.0mm. Class-III occlusion reoccurred in 11 patients and was significantly associated with age at surgery. Seven complications (device problems and others) were noted. Cranial pin slippage was significantly related to the elongation length.Internal deviceTwenty-eight patients with syndromic craniosynostosis were included. The mean age at surgery was 10.4 years, and the mean elongation length was 18.7mm. There were 15 complications, including device problems, zygomatic-maxillary fractures, and infections. Elongation length was significantly related to these complications. Class-III occlusion reoccurred in nine patients and was significantly related to age at surgery. CONCLUSION: This study found that complications are significantly more likely to occur in internal devices than in external devices, especially device infection. Our findings identified several factors that may assist surgeons in selecting between external and internal devices. The relationship between the amount of extension and device-related problems found in this study will be beneficial for solving these problems.

Long-Term Clinical Results of Two-Stage Total Ear Reconstruction of Microtia Using Autologous Cell-Engineered Chondrocytes.

BACKGROUND: Microtia repair requires a large volume of reconstruction material.In pediatric patients, the collectable volume of autologous cartilage is limited, and the impact of surgical invasion and donor-site morbidity can be particularly severe. The authors developed a new treatment method using cultured autologous human auricular chondrocytes that provides a sufficiently large volume of reconstruction material. METHODS: Approximately 1 cm2 of auricular cartilage was collected from the affected site. Chondrocytes were isolated and cultured with autologous serum to accelerate cell proliferation. The cells were subcultured and formed a gel-form mass without a scaffold. In our two-stage implantation, the cultured chondrocytes were first injected into the patient's lower abdomen, where the cells grew into a large, newly generated cartilage in 6 months. Thereafter, this cartilage was sculpted into an ear framework and subcutaneously reimplanted into the new ear location. Clinical outcomes were assessed over a long-term follow-up. RESULTS: Eight patients underwent surgery using cultured autologous auricular chondrocytes from 2002 to 2008. The patients' ages ranged from 6 to 10 years. The follow-up period ranged from 11 to 18 years. None of the patients experienced absorption of cultured chondrocytes after the second stage. Complications included one case of absorption and one case of allergic reaction in the first stage. CONCLUSIONS: The authors' patients represent the first successful cases of regenerative surgery for microtia using cultured chondrocytes. No malignant transformation, change in size, deformation, or other abnormalities were observed during the long-term follow-up, demonstrating the safety of cultured cartilage. No major complications occurred. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Factors Affecting Optic Nerve Damage in Le Fort III Osteotomy: A Retrospective Study.

The causes of visual impairment following Le Fort osteotomy for syndromic craniosynostosis have not been completely elucidated. The authors investigated the potential causes and means of prevention of optic nerve damage, with particular emphasis on intraoperative blood transfusion volume and operating time. This retrospective study evaluated patients who underwent Le Fort III osteotomy for syndromic craniosynostosis between 2000 and 2020. Data on pupillary reflex, pupil size, operating time, blood transfusion, age at time of surgery, sex, and syndrome type were obtained from medical records. Univariate analysis and multivariate analysis with the level of statistical significance set at P <0.05. For the 86 patients included, the mean values of operating time, amount of blood transfusion based on body weight, amount of blood transfusion per body weight per hour, and age were 6.0 hours (range: 3.5-12.3 h), 30.5 mL/kg (range: 0-322 mL/kg), 5.14 mL/kg/h (range: 0-35.7 mL/kg/h), and 10.0 years (range: 4-38 y), respectively. Crouzon, Apert, and Pfeiffer syndromes were observed in 49, 29, and 8 patients, respectively. Abnormal pupillary findings were observed in 27 patients of whom 25 showed no abnormalities in subsequent visual function and 2 developed blindness. Abnormal pupillary findings correlated with the amount of blood transfused per body weight ( P =0.0082) and amount of blood transfused per body weight per hour ( P =0.0052). As demonstrated in this study, increased intraoperative bleeding and amount of blood transfused were associated with optic nerve damage, particularly during acute bleeding. Prompt inspection of the pupils following surgery is therefore warranted.

Complications Due to Cranial Distraction for Craniosynostosis.

BACKGROUND: Complications of cranial distraction techniques can arise perioperatively. This study assessed long-term clinical outcomes following cranial distraction for craniosynostosis. We analyzed factors involved in those complications. METHODS: We retrospectively assessed outcomes from 120cases treated with cranial distraction for craniosynostosis between 1997 and 2019. Age at surgery, type of craniosynostosis, length of advancement, and complications were reviewed. We analyzed cases in which clinical characteristics and medical data appeared to increase the risk of complications. RESULTS: Of the 120 patients (65 males, 55 females), 79 had syndromic craniosynostosis and 41 had nonsyndromic craniosynostosis. Type of craniosynostosis was scaphocephaly in 17 patients, trigonocephaly in 4, plagiocephaly in 15, brachycephaly in 57, oxycephaly in 14, cloverleaf in 10, and others in 3. Mean age at surgery was 18.6 months (range, 3-525 months). Mean total length of advancement was 32.3 mm (range, 5.5-62.0 mm). No deaths were encountered. Complications included cerebrospinal fluid leaks in 5 patients, epidural abscess in 1, local infections in 33, device problems in 20, erosions and/or ulcers in 23 and decubitus ulcers in 8.Comparisons of complications and factors: Local infection showed no significant correlation with type of craniosynostosis, nor with the total length of advancement. Plate displacement showed significant relationships with total length of advancement, or with age at surgery (P < 0.01). CONCLUSION: These results suggested that local infection occurred independent of the type of craniosynostosis. Plate displacement and ulcers appear to occur more easily among patients with longer lengths of advancement or with surgery at a younger age, due to the weakness of the cranial bones.

Novel Suturectomy Using Absorbable Plates in Early Surgery for Craniosynostosis.

BACKGROUND: In craniosynostosis patients under 3 months of age, suturectomy is a valuable early treatment improving their outcomes. However, conventional suturectomy might not be in severe patients. The efficacy of our developed suturectomy using absorbable plates was examined. METHODS: Our method was indicated for craniosynostosis patients under 3 months old who had severe intracranial hypertension, scaphocephaly, plagiocephaly, or trigonocephaly between September 2011 and March 2018. All patients underwent suturectomy, and the bone edges on both sides of the cuts were covered with absorbable plates. Evaluation was conducted with 3-dimensional computed tomography and photographs, and cephalic index, distance from dorsum sellae to forehead on computed tomography were analyzed. RESULTS: Twenty-one of the 25 patients were evaluated. The preoperative cranial shapes were 4 brachycephaly, 6 scaphocephaly, 5 oxycephaly, 2 clover-leaf deformity, and 4 plagiocephaly. There were 9 syndromic and 12 nonsyndromic patients. The mean age at the time of surgery was 52.3 days (7-89), and the mean follow-up period was 3.5 years (1-8).The cephalic index and cranial definition improved in 18 patients. The secondary surgery was not required in four syndromic and none of the 12 nonsyndromic patients. There were no major complications. CONCLUSION: Placement of absorbable plates was able to prevent bone formation during the early postoperative period, and yet also promote bone formation after plate absorption. The authors believe syndromic craniosynostosis patients with severe deformities and nonsyndromic ones with scaphocephaly or plagiocephaly, successfully avoided secondary surgeries. This approach is less invasive for craniosynostosis and is expected to be highly effective.

Rare Median Cleft-Lip Repair With Novel Surgical Procedures Using Bilateral Cleft Margin Flaps.

Reports of median cleft lip with nasal anomalies are few, and descriptions of concomitant surgical repair of a median cleft lip and bifid nose deformity are similarly rare. These surgical repair techniques mainly involve the use of normal neighboring structures. Reconstruction of the columella and nasal base is especially important. A new surgical procedure was examined using bilateral cleft margin flaps for patients with a rare median cleft lip. Two patients with a rare median cleft lip were indicated for the procedure. One patient was 3 months old at surgery, and the other patient was 6 months old. These patients underwent single-stage reconstruction of the lip and nose. The new surgical procedure is characterized by the reconstruction of the columella, which involves creating a supportive structure using a cleft margin flap; subsequently, an anterior covering is created using a hinge flap from the nasal tip. Upper lip formation is performed by repair of the orbicularis oris muscle and rotation advancement of a cutaneous and mucosal flap from the lateral segments. The postoperative follow-up period was 4 to 5 years. The postoperative appearance was satisfactory in both patients. Furthermore, no significant complications were observed postoperatively. In conclusion, the surgical procedure with bilateral cleft margin flaps described in this report allows for successful reconstruction of a median cleft lip. This procedure is effectively tissue saving and results in satisfactory outcomes, which are maintained over a long term.

Long-term functional and aesthetic evaluation of using a single rhomboid-shaped fascial strip for severe congenital unilateral blepharoptosis.

PURPOSE: Surgery for congenital blepharoptosis is often performed at approximately 4-5 years of age. A long-term follow-up is important during facial growth. We performed frontal suspension by using a single rhomboid-shaped autogenous fascial strip. We conducted a long-term evaluation of patients with severe congenital unilateral blepharoptosis. PATIENTS AND METHODS: A total of 334 patients with congenital blepharoptosis underwent surgery between 1994 and 2006. Of these, 192 patients had unilateral blepharoptosis with levator function less than 3 mm. A retrospective analysis was conducted on those patients who could be followed up postoperatively for at least 10 years and had accurate clinical photographs available. Visual acuity, palpebral fissure height (PFH), and marginal reflex distance (MRD) were evaluated from the photographs to compare both eyes. PFH and MRD were assessed as ratio. Morphological evaluation was based on patient and family comments. Statistical analysis included the t-test. RESULTS: Participants comprised 95 patients (63 male and 32 female; 43 left and 52 right). Visual acuity showed improvements. Although the postoperative MRD ratio of the affected eye was significantly improved, no clinically significant bilateral difference was seen in postoperative PFH, compared with those in the unaffected side. The surgical results were as follows: 62 excellent, 14 good, 12 fair, 3 poor, and 4 other cases in our criteria. No major complications occurred. Satisfactory result could be seen in 76 of the 95 cases. CONCLUSION: The evaluation results show that our surgical procedure appears useful for patients with unilateral congenital ptosis. Moreover, blepharoptosis surgery is useful for improving visual acuity. In this paper, we report this surgical procedure and discuss its long-term results.

Our Surgical Repair for Macrostomia with Two Novel Small Triangular Flaps: Long-Term Follow-Up and Results.

The surgical methods for repairing congenital macrostomia should be carefully selected from both functional and aesthetic points of view. The surgical correction of macrostomia results in normal orbicularis oris function and symmetry of the repaired lip. In this article, the authors report their novel technique and the results obtained, assessed retrospectively. Twelve primary cases of macrostomia underwent surgical repair between 1996 and 2013. The age at surgery ranged from 3 to 78 months. The authors' operative procedure uses two small triangular flaps with a superficial layer of orbicularis muscle to preserve the dynamic action of muscle fibers attached to the skin of the triangular flaps, with suturing across the orbicularis muscle bundles. The optimal length of a medial small triangular flap is 1.5 to 2.0 mm, and the length of a lateral flap is 5 to 8 mm. Evaluation was based on the degree of symmetry of the lips as a ratio of the affected side to the unaffected side, and the scar characteristics. The follow-up period in this retrospective study ranged from 3 to 21 years. The same method was applied to all 12 cases in the reconstruction of a natural appearance and dynamic function of the oral commissure, and the results over several years showed satisfactory outcomes. The authors' technique is very useful in the primary surgery for macrostomia, as the dynamic action of muscle fibers is preserved, if the muscle is not dissected from the skin in the cleft margin that includes the two medial small triangular flaps. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Ankylosis of temporomandibular joints after mandibular distraction osteogenesis in patients with Nager syndrome: Report of two cases and literature review.

Nager syndrome, also known as Nager acrofacial dysostosis, was first described by Nager and de Reynier in 1948. The patients commonly present with micrognathia, and a preventive tracheostomy is necessary when there are symptoms of upper airway obstruction. Mandibular distraction osteogenesis is considered as an effective procedure, which not only improves micrognathia but also minimizes the chances of tracheostomy. However, mandibular distraction osteogenesis has some complications such as relapse, teeth injury, infection, and injury of the temporomandibular joints (TMJs). In this study, the author reported two patients with Nager syndrome who suffered from ankylosis of TMJs after mandibular distraction osteogenesis. In addition, a comprehensive literature review of post-distraction ankylosis of TMJs in patients with Nager syndrome was performed. Few studies demonstrated the condition of TMJs after mandibular distraction osteogenesis, and three studies were identified from the review. One study reported ankylosis of bilateral coronoid processes, in which coronoidectomies were necessary. Another study reported the use of prostheses to replace the ankylosed joints in a patient who had undergone many surgeries of the joints, such as gap arthroplasties, reconstructions with costochondral grafts, etc. One other study raised the concept of unloading the condyles during the mandibular distraction to prevent subsequent ankylosis. It seems that multiple factors are related to the ankylosis of TMJs after mandibular distraction osteogenesis in patients with Nager syndrome. Prevention of post-distraction ankylosis of the joints is important because the treatment is difficult and not always effective. We should conduct more studies about protection of the joints during mandibular distraction in the future.

Dynamic eye socket reconstruction after extensive resection of midfacial malignancies: preliminary results using temporalis transfer.

The functional and esthetic results of reconstructive surgery after extended total maxillectomy or extended orbital exenteration greatly depend on the quality of the orbital reconstruction. We developed dynamic eye socket reconstruction using temporalis transfer to achieve good orbital reconstruction, and examined the usefulness of our technique. Five patients (three men and two women, aged 44-72 years) who underwent extensive resection of midfacial malignancies were treated with dynamic eye socket reconstruction using temporalis transfer. In most cases, eye socket reconstruction was performed approximately 1 year after the initial surgery, and temporalis transfer was used after maturation of the eye socket. The follow-up period ranged from 16 to 120 months (average 63.8 months). Movement of the upper and lower eyelids was achieved in all cases, and definite creases at the lateral canthus were observed in two patients. A good shape in the reconstructed medial and lateral canthal areas was maintained in all patients. Our reconstruction technique is extremely effective in creating natural creases ('crow's feet') at the lateral canthus during smiling, enabling movement of the upper and lower eyelids, and maintaining a sharp palpebral morphology.

A hybrid therapy for Buerger's disease using distal bypass and a free temporoparietal fascial flap: a case report.

Buerger's disease presents a difficult challenge, with its uncertain etiology and lack of a standardized therapy, and is often refractory to treatment. In the present report, we have described the case of a 43-year-old male with Buerger's disease, a refractory ulcer on his right great toe, and severe pain at rest. We obtained favorable results using a hybrid therapy that combined distal bypass with a free temporoparietal fascial flap. Postoperatively, the flap healed, and the patient's pain resolved. After more than 3 years of follow-up, he had had no recurrence of the foot ulceration, and the patency of the bypass graft had been confirmed using Doppler flowmetry. We believe that the hybrid therapy we devised for the present patient (ie, combined distal bypass with a free temporoparietal fascial flap) is an effective treatment of Buerger's disease with pedal ulceration. The therapeutic strategies for this condition should not adhere to a single treatment modality; rather, they should combine available treatment modalities according to each individual patient's condition.

Dynamic eye socket reconstruction after extended total maxillectomy using temporalis transfer.

The functional and cosmetic results of the reconstructive surgery after extended total maxillectomy greatly depend on the quality of the orbital reconstruction. In order to achieve good orbital reconstruction, we developed the dynamic eye socket reconstruction using temporalis transfer. In this report, I will present the details of the technique, including tips and innovations for dynamic eye socket reconstruction.Two patients (2 males, aged 70 and 72 years old) who underwent extensive resection of midfacial tumours were treated with dynamic eye socket reconstruction using temporalis transfer. The follow up period was 16 and 102 months. No acute complications were observed. The movements of the upper/lower eyelids including crow's feet were observed and a good shape in the reconstructed medial/lateral canthal area was maintained in all patients.This procedure provides both the eyelids with movement and also a good shape in the reconstructed medial/lateral canthal region. Furthermore, it contributes to achieving satisfactory functional and cosmetic results in the orbital reconstruction.

Psycho-oncological aspect of surgery in palliative care: our satisfactory experience using a V-Y island flap.

CONCLUSIONS: Our reconstruction method using a V-Y island flap was minimally invasive and yielded a satisfactory esthetic result without impairing the patient's postoperative quality of life (QOL). OBJECTIVE: Malignant skin tumors of the head and neck often affect the patient's appearance and QOL. Therefore, surgery for terminally staged patients with malignant skin tumors may be a treatment of choice for surgeons in a palliative care team to improve and sustain the patient's QOL. METHODS: We describe our experience of surgical treatment of a large primary malignant skin tumor in the head performed as part of palliative care in a 38-year-old woman in the terminal stage. We developed a V-Y island flap that did not result in postoperative alopecia. RESULTS: After surgery, the patient did not have alopecia and her hair hid the scar. The patient and her family were greatly satisfied with the result. Although the surgery did not extend her life, it alleviated her depressed condition caused by the uncomfortable primary tumor.

Intense pulsed light therapy for aberrant Mongolian spots.

BACKGROUND: Aberrant Mongolian spots (AMS) distal to the lumbosacral region are thought to be more likely to persist than typical sacral Mongolian spots. So far, Q-switched ruby laser (QSRL) has been the treatment of choice for AMS. Intense pulsed light (IPL) is obtained from flashlamp devices that emit wavelengths between 515 and 1200 nm. IPL has documented efficacy for the treatment of irregular pigmentation, telangiectasia, rough skin texture, rhytids, hair removal, and vascular lesions, with several filters being available that can be used to block shorter wavelengths from the skin. As far as we could determine, there have been no clinical and histological studies on the treatment of AMS with IPL. Accordingly, the aim of this study was to assess the clinical and histological efficacy of IPL for AMS. METHODS: Seven patients (4 males and 3 females) presenting from September 2008 to July 2009 were assessed. Their mean age was 2.0 years, ranging from 0 to 7 years. The IPL device used in this study was a Natulight (Lumenis Ltd., Tokyo, Japan). Photographs were taken of all patients with a high-resolution digital camera at baseline and 6 months after treatment. Skin biopsy specimens were taken from 1 patient (case 4) before, immediately after, and 6 months after treatment. RESULTS: According to the 7 family members of the patients, the outcome of IPL was graded as follows: excellent improvement in 1 (14%), good improvement in 4 (57%), and slight improvement in 2 (29%). All families would have liked to continue IPL treatment. Evaluation of the effect of treatment by a physician was less favorable, with excellent improvement in 1 (14%), good improvement in 2 (29%), and slight improvement in 4 (57%). Histopathologic examination of the pigmented region revealed the typical features of a Mongolian spot in the hematoxylin-eosin stained section. Immediately after IPL, there were no changes in the dermis. At 6 months after treatment, however, the number of melanocytes in the middle and upper dermis was obviously decreased. CONCLUSIONS: IPL is an effective method for the treatment of AMS.