Linear Separation of Toe Syndactyly With Preserved Subcutaneous Vascular Network Skin Grafts.

BACKGROUND: Conventional surgery of toe syndactyly is accomplished using a zig-zag incision and full thickness or split thickness skin grafting. However, this method has an aesthetic problem with a prominent scar on the dorsal side of the foot. The aim of the article was to investigate the efficacy of a cosmetically conscious operation for syndactylies performed using a single technique. METHODS: A retrospective analysis was performed involving 66 patients with 88 syndactylic toes who presented to the department of plastic and reconstructive surgery at 1 of 3 major institutions during a 16-year period (1998-2014). Syndactylies were classified according to the affected interdigital spaces (IDSs). All cases were treated using the same method: linear skin incisions to divide the digits, triangular flaps on the dorsal and plantar sides of the foot, and preserved subcutaneous vascular network skin grafts from the medial inframalleolar region. RESULTS: The most frequent type of toe syndactyly was IDS-2 (union of the second/third toes), which accounted for 59.1% (39/66) of the cases. The rates of familial anomalies were significantly different between bilateral IDS-2 (4/13) and unilateral IDS-2 (0/24) (P < 0.05, Fisher exact test). There were no severe postoperative complications, including scar contracture or keloid formation. All patients obtained sufficiently deep IDSs, and none required repeat surgery. CONCLUSIONS: A straight incision minimizes the scar observed from the visible side, and preserved subcutaneous vascular network skin grafts prevent postoperative contracture of linear scar.

Long-term results of orbicularis oris muscle reconstruction in primary cleft lip repair using the "basket-weave method".

The basket-weave method is an orbicularis oris muscle reconstruction method used in primary unilateral cleft lip repair. We compared the long-term results of the basket-weave method with those of a conventional method. For primary unilateral cleft lip repair, we compared the long-term results of 7 cases in which the orbicularis oris muscle was reconstructed by use of the basket-weave method, and of 7 cases in which the reconstruction was performed by use of the conventional method. The average postoperative follow-up period was 12 years and 7 months for the basket-weave method, and 11 years and 9 months for the conventional method. Using photographs of the front and elevation angle views, we evaluated the results as good if the philtrum ridge was formed on the fissure side and was almost symmetrical in height; as fair if the philtrum ridge was lower than the normal side; and as poor if the philtrum ridge had disappeared. For the basket-weave method, the results were good in 6 cases (85.7%), fair in 1 case (14.3%), and poor in 0 cases. For the conventional method, the results were good in 2 cases (28.6%), fair in 4 cases (57.1%), and poor in 1 case (14.3%). A significant difference was found between the 2 groups (Mann-Whitney U test, P = 0.0417). The philtrum ridge shape could be reconstructed by use of the basket-weave method, which gave better results in the long-term than did the conventional method for orbicularis oris muscle reconstruction in primary unilateral cleft lip repair.

Presurgical orthopedic treatment ameliorates postoperative nasal deformity after cheiloplasty.

We assessed the postoperative nasal form and symmetry after presurgical use of a Hotz plate (HP) in patients with unilateral complete cleft lip and palate (UCLP). The subjects were 28 infants with UCLP who underwent cheiloplasty. Of these subjects, 14 underwent presurgical orthopedic treatment using the HP (HP group) and 14 underwent the same treatment without the HP (non-HP group). Photographic records were obtained 1 and 6 months after cheiloplasty, and the results of anthropometric analysis were compared between the 2 groups. The nasal inclination and the ratio of the width of the nasal base to the total width of the nose were significantly improved in the HP group compared with the non-HP group at both postoperative time points. Thus, presurgical use of the HP significantly improved the nasal asymmetry and deformity in UCLP patients after primary cheiloplasty and nasal repair.

Staged repair of pentalogy of Cantrell with ectopia cordis and ventricular septal defect.

Pentalogy of Cantrell is a rare congenital anomaly characterized by a combination of severe defects in the middle of the chest and abdomen including intracardiac defects. Survival rate after cardiac surgery is extremely low. We present a successful staged complete repair of an omphalocele, a ventricular septal defect and a sternal defect in a case of pentalogy of Cantrell.

Presurgical nasoalveolar molding orthopedic treatment improves the outcome of primary cheiloplasty of unilateral complete cleft lip and palate, as assessed by naris morphology and cleft gap.

We evaluated the effects of presurgical nasoalveolar molding (NAM) with an orthopedic appliance and compared them with a passive orthopedic method (Hotz plate, HP), focusing on the naris morphology and width of the alveolar and palate cleft gap. The subjects were 28 unilateral complete cleft lip and palate patients treated with primary cheiloplasty at Tsukuba University Hospital from 2004 to 2011. Thirteen patients were treated preoperatively with NAM (NAM group), and 15 with HP (HP group). The surgical outcome was assessed according to left-right naris symmetry, as measured by the area ratio, perimeter ratio, aspect a/u ratio (aspect ratio of the affected side/aspect ratio of the unaffected side), and Hausdorff distance. In addition, the alveolar and palate cleft width was measured at the times of orthopedic plate setting and primary cheiloplasty. The aspect ratio was significantly smaller in the NAM group than in the HP group before the operation. In both groups, the aspect ratio, perimeter ratio, and Hausdorff distance were significantly smaller after the operation than before. The width of the alveolar and palate cleft gap was significantly narrowed in the NAM group, and the cleft gap at the initiation of NAM correlated significantly with the Hausdorff distance after cheiloplasty. We found that NAM improved the form of the naris after primary cheiloplasty and decreased the palate cleft gap more effectively than HP and that the width of the palate cleft gap was correlated with the surgical outcome of the naris.

Nasal molding prevents relapse of nasal deformity after primary rhinoplasty in patients with unilateral complete cleft lip: An outcomes-based comparative study of palatal plate alone versus nasoalveolar molding.

OBJECTIVES: In recent years, many studies have reported that the presurgical nasoalveolar molding method improves the nose morphology; however, the reason for its effectiveness after surgery has never been understood. We evaluated the effect of nasoalveolar molding by comparing it with a passive orthopedic method without a nasal stent and focusing on the nostril morphology after primary cheiloplasty using various measurement methods. We then analyzed the essential factors. MATERIALS AND METHODS: The patients involved were 31 infants with unilateral complete cleft lip and palate treated with primary cheiloplasty at the University of Tsukuba Hospital from 2004 to 2011. Of the 31 infants, 16 received nasoalveolar molding treatment and 15 received passive orthopedic treatment as controls. Photographic facial measurements were performed for all patients immediately and 7 months after primary cheiloplasty. The esthetics of the nostrils were assessed according to the left-right nostril symmetry, as measured by the Hausdorff distance, area ratio, perimeter ratio, and aspect a/u (the aspect ratio of the affected side)/(the aspect ratio of the unaffected side) ratio. In addition, the inclination of the nasal ridge was assessed using anthropometric measurements (Grc-Grn∠midline and midline∠columellar axis). RESULTS: The area ratio, perimeter ratio, and Grc-Grn∠midline were significantly greater in the nasoalveolar molding group immediately after surgery (p = 0.00062, 0.016, and 0.048, respectively) than in the control group. However, the Hausdorff distance and aspect a/u ratio were more favorable (p = 0.0018 and 0.0039, respectively) in the nasoalveolar molding group after 7 months. CONCLUSIONS: The results of our study suggested that using nasoalveolar molding as a presurgical orthopedic treatment could improve the shape of the nasal cartilage with surgeon's corrections.

FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion.

Mutations in the fused in sarcoma (FUS, also known as translated in liposarcoma) gene have been recently discovered to be associated with familial amyotrophic lateral sclerosis (FALS) in African, European and American populations. In a Japanese family with FALS, we found the R521C FUS mutation, which has been reported to be found in various ethnic backgrounds. The family history revealed 23 patients with FALS among 46 family members, suggesting a 100% penetrance rate. They developed muscle weakness at an average age of 35.3 years, followed by dysarthria, dysphagia, spasticity and muscle atrophy. The average age of death was 37.2 years. Neuropathological examination of the index case revealed remarkable atrophy of the brainstem tegmentum characterized by cytoplasmic basophilic inclusion bodies in the neurons of the brainstem. We screened 40 FALS families in Japan and found 4 mutations (S513P, K510E, R514S, H517P) in exon 14 and 15 of FUS. Even in Asian races, FALS with FUS mutations may have the common characteristics of early onset, rapid progress and high penetrance rate, although in patients with the S513P mutation it was late-onset. Degeneration in multiple systems and cytoplasmic basophilic inclusion bodies were found in the autopsied cases.

Rhythmic pupillary oscillation in Creutzfeldt-Jakob disease associated with the Glu/Lys mutation of prion protein codon 200.

We report two Creutzfeldt-Jakob disease (CJD) patients with rhythmic pupillary and palpebral oscillation who had a mutation of prion protein codon 200 that resulted in the substitution of lysine for glutamate (Glu/Lys). Alternating dilation and constriction of the pupils combined with elevation and descent of the eyelids occurred in correspondence with periodic sharp wave complexes (PSWCs) on the electroencephalogram and with myoclonus of the head, face, and extremities. The onset of pupillary dilation and palpebral elevation coincided with the PSWCs. Initiation of these rhythmic pupillary and palpebral movements may depend on sympathetic activity, but the site of the generator is unclear. Such rhythmic pupillary and palpebral oscillation may be a feature of rapidly progressive CJD with predominant right hemispheric involvement.

Clinical and histopathological features of progressive-type familial amyloidotic polyneuropathy with TTR Lys54.

The purpose of this study was to evaluate the clinical and pathological features in patients with progressive-type familial amyloidotic polyneuropathy (FAP) using autopsy and biopsy specimens. A proband is a 33-year-old man with FAP type I who developed motor, sensory and autonomic impairments with neuropathy, heart failure, and anorexia. Genetic findings of transthyretin (TTR) revealed G to A transition in codon 54 causing a rare mutation of TTR Lys54. He died of pneumonia and severe cardiac failure 4 years after onset. Autopsy showed heavy amyloid deposition in the heart, peripheral nerves, thyroid, skin, fat tissue, prostate and testis, moderate in the sympathetic nerve trunk, vagal nerve, celiac plexus, pelvic plexus, bladder, gastrointestinal tract, tongue, pancreas, lung, pituitary, blood vessel, gall bladder, adrenals and muscles, and free in the central nervous system, liver, kidney and spleen. Sural nerve biopsy in a sibling confirmed TTR amyloidosis immunohistochemically. Electronmicroscopic findings of amyloid fibrils were similar to that of FAP Met30. Immunoelectronmicroscopic findings indicated the relationship between amyloid fibrils or non-fibrillar structure and collagen fibers. The distribution of amyloid deposition, heavy in the heart and lacking in the kidney, is a characteristic feature and reflected severity of FAP with TTR Lys54.

Exercise training enhances in vivo clearance of endotoxin and attenuates inflammatory responses by potentiating Kupffer cell phagocytosis.

The failure of Kupffer cells (KCs) to remove endotoxin is an important factor in the pathogenesis of non-alcoholic fatty liver disease (NAFLD). In this study, the effects of exercise training on KC function were studied in terms of in vivo endotoxin clearance and inflammatory responses. Mice were allocated into rest and exercise groups. KC bead phagocytic capacity and plasma steroid hormone levels were determined following exercise training. Endotoxin and inflammatory cytokine levels in plasma were determined over time following endotoxin injection. KC bead phagocytic capacity was potentiated and clearance of exogenously-injected endotoxin was increased in the exercise group. Inflammatory cytokine (TNF-α and IL-6) levels were lower in the exercise group. We found that only DHEA was increased in the plasma of the exercise group. In an in vitro experiment, the addition of DHEA to RAW264.7 cells increased bead phagocytic capacity and attenuated endotoxin-induced inflammatory responses. These results suggest that exercise training modulates in vivo endotoxin clearance and inflammatory responses in association with increased DHEA production. These exercise-induced changes in KC capacity may contribute to a slowing of disease progression in NAFLD patients.

Thyroid anaplastic carcinoma transformed from papillary carcinoma in extrathyroid area.

We report a 75-year-old male with anaplastic carcinoma in an extrathyroid area. Thyroid remained unchanged. The patient is alive without incident of tumor recurrence at 3.5 years after total resection and at 5 years after initial symptom. The tumor developed between the sternocleidomastoid muscle and common carotid artery, and was completely separated from the thyroid. The tumor location was consistent with a branchial cyst. The tumor consisted of two parts; an upper solid tumor and a deep cystic tumor. The former showed anaplastic carcinoma with osteoclast-like giant cells. The latter was consistent with thyroid papillary carcinoma. The center was intermingled with these two carcinomas. Anaplastic carcinoma cells were positive for vimentin and papillary carcinoma cells were positive for keratin, thyroglobulin, and thyroid transcription factor-1. These results remain insufficient to find any conclusions concerning the tumor nature; either ectopic thyroid carcinoma arising from a branchial cyst or occult thyroid carcinoma metastasis. This is rare case in which thyroid anaplastic carcinoma transformed from papillary carcinoma in an extrathyroid area.

Modification of the Nuss Procedure: The Single-incision Technique.

SUMMARY: The Nuss procedure is a prevalent minimally invasive surgery for pectus excavatum. Although the Nuss procedure has the advantage of leaving less obtrusive scars, the standard technique requires at least 3 skin incisions to insert several instruments. We experienced 7 cases of the modified Nuss procedure using a single incision during a 7-year period. To facilitate passing of the bar, a traction guide was created according to our unique method. There was no need for a bar stabilizer, and no severe intraoperative complications occurred. All patients exhibited satisfactory short-term results; however, 1 patient suffered from bar rotation and required repeat surgery for fixation. Two patients underwent bar removal via the same single incision without any difficulties.

Laser therapy treatment of phacomatosis pigmentovascularis type II: two case reports.

INTRODUCTION: Phacomatosis pigmentovascularis is a rare congenital condition characterized by vascular malformation associated with extensive pigmented nevi. Even though it forms a large, prominent skin lesion, therapy for phacomatosis pigmentovascularis is rarely discussed. To the best of our knowledge, this is the first report of phacomatosis pigmentovascularis type II treated with combined laser therapy using Q-switched alexandrite and long-pulsed dye lasers. CASE PRESENTATIONS: In the first of two cases reported here, a 2-week-old Japanese baby girl was given a diagnosis of phacomatosis pigmentovascularis type II and Klippel-Trénaunay syndrome because of port-wine stains, cutis marmorata telangiectatica congenita, and aberrant Mongolian spots over her trunk and limbs. After five laser therapy sessions under general anesthesia, her aberrant Mongolian spots and port-wine stains have improved. But interestingly, the cutis marmorata telangiectatica congenita on the patient's back has improved without laser therapy.In the second case, a 4-month-old Japanese baby boy was referred to us because of port-wine stains, cutis marmorata telangiectatica congenita, and aberrant Mongolian spots over his face, trunk and limbs. Phacomatosis pigmentovascularis type II was diagnosed and laser therapy was started. After three laser therapy sessions under general anesthesia, the aberrant Mongolian spots and port-wine stains have improved. The cutis marmorata telangiectatica congenita on the baby's back, buttocks, and arms has faded somewhat without laser therapy. CONCLUSIONS: Combined laser therapy improved the phacomatosis pigmentovascularis skin lesions, but was not effective for the cutis marmorata telangiectatica congenita with hemiatrophy. Cutis marmorata telangiectatica congenita without atrophy can be expected to improve on its own. Our results will assist physicians considering how best to treat patients with phacomatosis pigmentovascularis.

A novel method for evaluating postsurgical results of unilateral cleft lip and palate with the use of Hausdorff distance: presurgical orthopedic treatment improves nasal symmetry after primary cheiloplasty.

OBJECTIVE: To evaluate surgery results, we established a novel method to digitize nasal morphology with the use of Hausdorff distance and analyzed nose morphology after cheiloplasty. STUDY DESIGN: We evaluated the naris after primary cheiloplasty of 30 unilateral cleft lip and palate patients. Similarity between left and right sides was assessed by visual evaluation, area ratio, perimeter ratio, aspect a/u ratio, and Hausdorff distance. The postoperative naris morphology was also compared between 15 patients treated with a Hotz plate before surgery and 15 not treated. RESULTS: Significant correlation with visual evaluation was found for Hausdorff distance. For the groups with and without Hotz plate treatment, the visual evaluation was higher and Hausdorff distance significantly lower in the treated group. CONCLUSIONS: The morphologic measurement obtained using the Hausdorff distance was the closest to visual evaluation, and assessment using Hausdorff distance suggested that using a Hotz plate helps retain the symmetry of the nares after cheiloplasty.

Conjunctival squamous cell carcinoma of the orbit 40 years after enucleation.

Conjunctival squamous cell carcinoma developed in a 51-year-old man 40 years after he had enucleation of his left eye because of an explosion injury. He had worn several ocular prostheses for more than 40 years. Recently he had noticed an increasing sanguineous conjunctival discharge, a foreign body sensation, and swelling of his left lower eyelid. Incisional biopsies of an underlying conjunctival mass revealed squamous cell carcinoma. His work history did not involve exposure to radiation, chemicals, or the sun. The authors concluded that squamous cell carcinoma in this case was caused by chronic irritation as the result of long-standing use of a poorly fitting ocular prosthesis.

A new surgical method for treating lateral ray polydactyly with brachydactyly of the foot: lengthening the reconstructed fifth toe.

Lateral ray polydactyly is the most common anomaly of the foot. However, when both the fifth and sixth toes are markedly shorter than a normal toe, reconstruction of a normal-length fifth toe has not been performed, because the toe rarely serves a functional purpose. The authors devised a new method whereby the fifth toe could be lengthened using the excess toe as a composite flap. Fifteen patients (15 toes) underwent our procedures and were evaluated in this study. The stepladder island flap is used. The decision as to which toe to use as the flap was made preoperatively based on nail size, and its morphologic and radiographic conditions. Stepladder incision lines were designed on the dorsal and plantar sides of the duplication. Care was taken to preserve the neurovascular bundle with the subcutaneous pedicle and osteotomies were made at the middle phalanx of the flap and the distal or middle phalanx of the recipient in almost all cases. The elevated composite toe flap was advanced and the bone was fixed rigidly for 1-2 months after the operation. During our follow-up periods, the reconstructed toes grew normally, and good aesthetic results had been achieved apart from the bulkiness of the reconstructed toes. There was no deficiency in circulation or function for walking, running, or shoe-fitting in this series.