Nonsurgical Creation of an Auriculocephalic Sulcus in Children With Congenital Auricular Deformities.

Nonsurgical correction of congenital auricular deformity is known to be effective in newborns. In this study, the authors investigated factors influencing the outcome of nonsurgical or surgical correction of the auriculocephalic sulcus, an important auricular structure and one that is necessary for wearing glasses or a mask. A total of 80 ears (63 children) were splinted using a metallic paper clip and thermoplastic resin in our outpatient clinic between October 2010 and September 2019. The ears were divided into a group, in which the auriculocephalic sulcus was formed nonsurgically (n =5 6) and a group, in which surgery was needed (n = 24). The authors compared the clinical characteristics of the deformities, whether cryptotia affected the superior or inferior crus, and whether constricted ears were Tanzer group IIA or IIB between the two study groups by retrospective chart review. There was a significant correlation between the age, at which ear-molding treatment was initiated and outcome ( P < 0.001). The optimal cutoff value for age, before which ear-molding treatment should be initiated was 7 months. Inferior crus-type cryptotia was corrected adequately by splinting, but all Tanzer group IIB constricted ears needed surgical treatment. Earlier initiation of ear-molding treatment is recommended, preferably before 6 months of age. Nonsurgical treatment is an effective intervention for the creation of the auriculocephalic sulcus in ears with cryptotia and Tanzer group IIA constricted ears but cannot correct for an inadequate amount of skin over the auricular margin or a defect in the antihelix.

Postoperative complications associated with extubation strategies following palatoplasty: a single-center retrospective analysis.

PURPOSE: Palatoplasty carries a high risk of airway obstruction as a postoperative complication. Since 2007, the protocol in our hospital has been to leave an endotracheal tube in place after surgery while the patient is moved to the pediatric intensive care unit. Extubation is then performed after achievement of hemostasis and recovery of consciousness. We compared the cases over the 5-year periods before and after the introduction of this revised postsurgical management plan to investigate its effect on postoperative complications. METHODS: This was a retrospective cohort study involving a single pediatric hospital. The subjects were 199 children aged 1-3 years, who underwent palatoplasty between January 2002 and July 2012. Changes in the incidence rates of postoperative complications were statistically examined. RESULTS: There were significantly more postoperative complications among the patients who were extubated in the operating room than among those extubated in the intensive care unit (operating room group, 22/94 cases; intensive care unit group, 10/105 cases; P < 0.01). Serious complications, such as hypoxemia and airway obstruction, also occurred more frequently in the operating room group. CONCLUSION: Extubation in an intensive care unit was possibly associated with a reduction in postoperative complications.

Congenital hypertrophy of multiple intrinsic muscles of the foot.

Congenital hypertrophy of a single intrinsic muscle of the foot is rare, and as far as we know, only six cases have been reported. We describe a case of congenital anomaly that showed hypertrophy of multiple intrinsic muscles of the foot; the affected muscles were all the intrinsic muscles of the foot except the extensor digitorum brevis or extensor hallucis. Other tissues such as adipose tissue, nervous tissue, or osseous tissue showed no abnormalities. To reduce the volume of the foot we removed parts of the enlarged muscles.

Combined free autologous auricular cartilage and fascia lata graft repair for a recurrent tracheoesophageal fistula.

Repair of recurrent tracheoesophageal fistula (TEF) after repair of congenital esophageal atresia continues to be a difficult problem. The most common re-operation for a recurrent TEF involves repair via a right thoracotomy and use of a flap as interposed tissue between the closure sites. Although several materials have been reported for recurrent fistula repair, natural pedicled flaps have been used in most previous reports. The harvesting of a pedicled flap can sometimes be difficult when the patient has had multiple surgeries. In this report, the successful use of a combined free autologous auricular cartilage and free fascia lata graft repair for a complex patient with multiple recurrent TEFs is described. We believe that our technique should be considered as an approach for patients with recurrent TEF.

Thoracoabdominoplasty with umbilicoplasty for Cantrell's syndrome.

Cantrell et al. described a syndrome with five anomalies characterised by defects of the abdominal wall, lower sternum, anterior diaphragm, diaphragmatic pericardium, and heart. Because most of the children who survived could not have the thoracoabdominal wall adequately reconstructed at the initial operation, ventral herniation is often the result and they have to live with the danger of direct trauma to the unprotected heart. It therefore becomes important protect the unguarded heart while improving the appearance of the thoracoabdominal region. The reconstruction of the lower sternum has rarely been reported. We describe four such patients, three of whom had a thoracoabdominoplasty and umbilicoplasty using autologous rib cartilage and rectus muscles. All these children had solidly reconstructed sternums, and their abdominal appearances are excellent.

[Breast deformities caused by the operation for congenital heart disease].

Breast deformities are seen as one of the late postoperative complications in thoracotomy, but there are very few reports in the literature. We investigated causes and treatments in 5 patients who have consulted to our department after operations for congenital heart disease between April 1989 and March 2011. The injured breast bud in the cardiac operation resulted in hypoplastic deformities, and deformities became apparent during puberty with breast growth. These patients required release of the scar contracture to lead to normal breast development, and also have to been treated chest deformity. After stopping at breast growth in adolescence, some augmentations were necessary if bilateral asymmetry had been remained. Breast deformities are very important issue in the viewpoints of patients' quality of life (QOL), though not relating directly to vital prognosis. We'd like to introduce these complications and some choices about breast reconstruction to thoracic surgeons.

Free fascia lata repair for a second recurrent congenital diaphragmatic hernia.

Repair of recurrent congenital diaphragmatic hernia (CDH) continues to be a difficult problem. Although several materials have been used to repair recurrent CDH, the ideal material has yet to be established. We report the successful use of an autologous free fascia lata graft to repair the diaphragm following a second recurrence of CHD. The fascia lata is one of the strongest fascia in the body and is easy to obtain without extremity functional loss even in children. This procedure is regarded as effective for the repair of recurrent CDH.

Abdominoplasty of cloacal exstrophy: application of reconstructive methods to recurrent and primary cases.

BACKGROUND: Surgical repair of cloacal exstrophy is still challenging. At birth, patients undergo bladder closure, colostomy, and osteotomy of the pubic bone (if necessary, when the interpubic range is wide and cannot be brought together with the hands). This abdominal defect is closed primarily by urologists or pediatric surgeons, if possible, but the patient may experience a relapse of bladder exstrophy and an abdominal defect. Abdominal reconstruction was performed for a series of recurrent and primary (preventive) cases. METHODS: Abdominal wall reconstruction was performed using the rectus abdominis and external oblique muscle fascia flaps, and reinforcement of the bilateral rectus abdominis muscles in 2 cases of cloacal exstrophy patients. One was a recurrent case treated at 7 months old, and the other was done primarily at 2 days after birth. RESULTS: The closure of the abdominal wall was successful and no relapse of bladder exstrophy or abdominal defect has occurred. These patients are now undergoing rehabilitation. CONCLUSION: Cloacal exstrophy usually has many serious complications. The abdominal-wall defect is often large and accompanied by a wide detachment of the pubic bone. Even if the simple closure of abdominal wall is possible at birth, it is usually insufficient. Abdominal-wall repair must be done thoroughly, soon after birth, and plastic surgery techniques should be used.

Ortho-phthalaldehyde-induced skin mucous membrane damage from inadequate washing.

Because body fluids and blood have a tendency to adhere to transesophageal echo devices, a high level of sterilisation is required when cleaning them. Ortho-phthalaldehyde (OPA) has been widely used in Japan since being approved as a high-level sterilant. The authors report a patient with widespread, severe skin and mucous membrane damage of the lip, tongue, pharynx and oesophagus areas that was attributed to inadequate washing after the sterilisation of a transesophageal echo device with OPA. This patient experienced sequelae, which did not improve after more than 1 year of continuous treatment. When using medical devices sterilised with OPA, the use of a probe cover, when applicable, is recommended and complete washing prior to use is required.

Respiratory management of Pierre Robin sequence using nasopharyngeal airway with Kirschner wire.

BACKGROUND: The Pierre Robin sequence (PRS) is a relatively rare symptom complex characterised by glossoptosis, micrognathia and respiratory obstruction. The initial problem that children with PRS face is obstructive dyspnoea, which can result in death without appropriate respiratory management. We designed and used a modified airway with a Kirschner wire (K-airway) in children with PRS who suffered from dyspnoea that did not improve with conservative treatment. METHODS: The subjects were four children diagnosed with PRS at the Department of Plastic Surgery, Shizuoka Children's Hospital, from February 2007 to December 2008. Since dyspnoea was not improved by conservative treatment, a phi0.8-mm Kirschner wire was set inside a nasopharyngeal airway bent in a form to lift the root of the tongue in order to prevent glossoptosis. The respiratory condition was evaluated with a test for sleep apnoea. RESULTS: Successful improvement in dyspnoea with the K-airway was noted in all cases. In Case 1, the subject was discharged from hospital without using the K-airway (92 days of age). In Case 2, the subject was discharged from hospital using the airway only at nighttime (122 days of age). CONCLUSIONS: This method is safe because it is less invasive, and its effects can be easily evaluated, suggesting that it is a good method to try prior to surgical treatment.

Video recording study of infants undergoing primary cheiloplasty: are arm restraints really needed?

OBJECTIVE: Arm restraints are traditionally used during the perioperative period for cleft surgery to prevent the affected infant from damaging the wound, but the benefits of this standard practice have been controversial. To investigate whether the use of arm restraints provides any benefit to the patient, a video recording study of infants undergoing primary cheiloplasty was conducted. DESIGN: Analysis of video recordings of infants undergoing cheiloplasty. SETTING: Shizuoka Children's Hospital, Shizuoka, Japan. PATIENTS AND METHODS: Eight patients who underwent primary cheiloplasty were recorded for 24 hours on the day before the operation, the day of the operation, and the fourth postoperative day. All recordings were examined by the first author, and the frequency and manner of lip touching were evaluated. RESULTS: Although the frequency of touching varied considerably from 2 to 136 times per 24 hours, it was not statistically different among the 3 recording days (Friedman test, p > .05). All infants touched their lips softly and never attempted to manipulate or scratch their wound. None of the patients pinched the stitches or adhesive tape on the wound with their hands. CONCLUSIONS: Although the examined infants touched their lips, they never touched them in a manner that would be harmful to the wound. We concluded that arm restraints are unnecessary after primary cheiloplasty if the procedure is performed before the age of 3 or 4 months.

Cleft palate in Kabuki syndrome: a report of six cases.

Kabuki syndrome is a syndrome of rare congenital anomalies that was named after its characteristic appearance, a face resembling that of an actor in a Kabuki play. Although cleft palate is a feature that is sometimes observed in patients with Kabuki syndrome, there are few clinical reports of cleft palate associated with Kabuki syndrome. This report presents six cases of Kabuki syndrome with cleft palate and reviews their clinical features. Our results suggest that (1) patients with cleft palate in Kabuki syndrome tend to fail in acquiring normal velopharyngeal function and (2) submucous cleft palate might be more common in patients with Kabuki syndrome than previously was reported.

Calcifying aponeurotic fibroma of the knee: A case report with radiological findings.

Calcifying aponeurotic fibroma is a rare type of benign tumor that occurs most commonly in the distal extremities of young children. Due to its infiltrative growth, it has a high tendency of recurrence. Although the clinicopathological features of over 100 cases of this rare disease have been reported, its clinical and radiological features have yet to be described in detail. We present a case of calcifying aponeurotic fibroma of the knee from birth with radiological images, that demonstrate the peculiar features of this uncommon benign tumor and discuss its clinicopathological features based on computed tomography and magnetic resonance images.

Subcranial facial bipartition osteotomy with glabellar reverse V-shaped and temporal approaches instead of the bicoronal approach.

Patients with Apert syndrome show hypertelorism and midfacial hypoplasia, and their features are significantly improved through facial bipartition surgery. In addition, because patients with Apert syndrome demonstrate cranial deformity as well as other deformities, they require multiple surgical interventions throughout their development. We present herein a girl with Apert syndrome for whom subcranial facial bipartition was performed. We could not use the coronal approach because she had a terribly cicatricial scalp and wide calvarial defects caused by previous cranial surgeries carried out at another institution. Therefore, we used the glabellar reverse V-shaped approach and temporal approaches in place of the standard approach. She enjoyed a complication-free intraoperative and postoperative course, and left the hospital on postoperative day 10. Her midfacial segment was repositioned 6 mm anteriorly and 12 mm inferiorly on the cephalometric measurement. Her interpupillary distance was altered from 83 mm preoperatively to 76 mm postoperatively. The reverse V-shaped glabellar approach permits more relaxation of the nasal skin and nasal augmentation that is more reliable, although an inconspicuous scar remains in the prominent area. The temporal approach is also useful, enabling easy zygomatic arch osteotomy and secure pterygo-maxillary separation for pterygo-maxillary separation through an oral approach, allowing chiseling toward the skull base.

Two cases of Tessier no. 4 cleft with anophthalmia.

OBJECTIVE: Tessier no. 4 cleft is a very rare craniofacial anomaly, and the primary surgical procedure has not been definitely standardized. The cheek advancement flap, technique, which was first reported by Van der Meulen (1985), has produced cosmetically favorable results. In this report, two cases with Tessier no. 4 cleft, which were treated with the cheek advancement flap technique, are presented. DESIGN AND PATIENTS: The first case was unilateral and the second was bilateral, and both were accompanied with anophthalmia on the cleft side. At the primary operation, dissection of the nasal soft tissue, medial canthopexy, and reconstruction of the lower eyelid and conjunctival fornix were performed. Cleft lip was repaired according to the rotation-advancement procedure. RESULTS AND CONCLUSIONS: Postoperative appearances were acceptable in both cases, because the resulting scars were made along the esthetic facial units. However, the soft tissue deficiency of the lower eyelid was prominent. Furthermore, in the unilateral case, caudal displacement of the palpebral fissure and cephalic deviation of the nasal ala was recognized.

Evaluation of the donor site in patients who underwent reconstruction with a free radial forearm flap.

A major disadvantage of free radial forearm flaps is the conspicuous donor site. However, there have been few studies on donor scars. The authors evaluated the donor site in patients who underwent oral-floor reconstruction with a free radial forearm flap. The subjects were 23 patients (19 males and four females) who underwent reconstruction with a free radial forearm flap following resection of a malignant oral tumor, and were followed for 1 year or longer. The fasciocutaneous flap collection site was closed by full-thickness skin graft (FTSG) from the groin with tie-over dressing. All grafts took perfectly. At the scar at the donor site, five items (pigmentation, scar width, depression, wrist mobility, and sensory abnormalities) were evaluated. Depression and pigmentation were often observed, but patient dissatisfaction was slight. While their main postoperative concern was the oral reconstruction site, after about 1 year, the donor site became more important to patients. However, the results were good. A 100 percent take of the FTSG at the donor site should produce good results. Surgeons should pay adequate attention not only to the outcome at the reconstruction site, but also to the closure of the donor site.

Epidermolysis bullosa simplex associated with pyloric atresia is a novel clinical subtype caused by mutations in the plectin gene (PLEC1).

Epidermolysis bullosa (EB) is an inherited mechano-bullous disorder of the skin, and is divided into three major categories: EB simplex (EBS), dystrophic EB, and junctional EB (JEB). Mutations in the plectin gene (PLEC1) cause EBS associated with muscular dystrophy, whereas JEB associated with pyloric atresia (PA) results from mutations in the alpha6 and beta4 integrin genes. In this study, we examined three EB patients associated with PA from two distinct families. Electron microscopy detected blister formation within the basal keratinocytes leading to the diagnosis of EBS. Surprisingly, immunohistochemical studies using monoclonal antibodies to a range of basement membrane proteins showed that the expression of plectin was absent or markedly attenuated. Sequence analysis demonstrated four novel PLEC1 mutations. One proband was a compound heterozygote for a nonsense mutation of Q305X and a splice-site mutation of 1344G-->A. An exon-trapping experiment suggested that the splice-site mutation induced aberrant splicing of the gene. The second proband harbored a heterozygous maternal nonsense mutation, Q2538X and homozygous nonsense mutations R1189X. Analysis of the intragenic polymorphisms of PLEC1 suggested that R1189X mutations were due to paternal segmental uniparental isodisomy. These results indicate that PLEC1 is a possible causative gene in this clinical subtype, EBS associated with PA. Furthermore, two patients out of our three cases died in infancy. In terms of clinical prognosis, this novel subtype is the lethal variant in the EBS category.

Usefulness of palatal mucoperiosteal grafts for artificial eye socket contracture.

The authors performed palatal mucoperiosteal grafting for contracture of the artificial eye socket in 4 patients. Mucoperiosteal grafts were collected from the paramedian area of the hard palate. After release of contracture, the grafts were sutured with absorbable thread to the defective areas on the conjunctival side of the artificial eye socket after release of contracture. All patients showed mucoperiosteal graft survival without problems, no recurrence of contracture, and good courses of artificial eye wear. The mucoperiosteal donor areas showed closed healing after 3 to 4 weeks. Palatal mucoperiosteal grafts can be collected en bloc and are relatively rigid, which allows the simultaneous reconstruction of the conjunctival side and supportive tissue of the eyelid. Although the size of graft collection is limited, grafts with adequate size for partial reconstruction can be collected. Mucoperiosteal grafts are a good reconstruction material for contracture of the artificial eye socket.

Cephalometric analysis in submucous cleft palate: comparison of cephalometric data obtained from submucous cleft palate patients with velopharyngeal competence and incompetence.

OBJECTIVE: The purpose of this study was to investigate the relationship between craniofacial and nasopharyngeal morphology and velopharyngeal function in submucous cleft palate. DESIGN AND PATIENTS: Fifty-two lateral cephalometric radiographs of 46 submucous cleft palate (SMCP) patients with velopharyngeal competence (24 patients) and incompetence (22 patients) at 4 and 7 years of age were studied. The patients had not received any surgical or orthodontic treatment prior to cephalography being performed. RESULTS: Significant differences were found between cephalometric variables (N-Ba, N-S-Ba angle) in children with velopharyngeal competence and incompetence. However, the results of our study showed that cephalometric data alone are not useful for predicting velopharyngeal function and can not serve as an absolute prognostic indicator. CONCLUSION;There are many factors that can influence velopharyngeal function in SMCP patients. Cephalometric data did not demonstrate a strong relationship to velopharyngeal function.