Refractory CSF leakage following untethering surgery performed 10 months after birth for enlarging terminal myelocystocele associated with OEIS complex.

Background: Terminal myelocystocele (TMC) is an occult spinal dysraphism characterized by cystic dilatation of the terminal spinal cord in the shape of a trumpet (myelocystocele) filled with cerebrospinal fluid (CSF), which herniates into the extraspinal subcutaneous region. The extraspinal CSF-filled portion of the TMC, consisting of the myelocystocele and the surrounding subarachnoid space, may progressively enlarge, leading to neurological deterioration, and early untethering surgery is recommended. Case Description: We report a case of a patient with TMC associated with OEIS complex consisting of omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I), and spinal deformity (S). The untethering surgery for TMC had to be deferred until 10 months after birth because of the delayed healing of the giant omphalocele and the respiration instability due to hypoplastic thorax and increased intra-abdominal pressure. The TMC, predominantly the surrounding subarachnoid space, enlarged during the waiting period, resulting in the expansion of the caudal part of the dural sac. Although untethering surgery for the TMC was uneventfully performed with conventional duraplasty, postoperative CSF leakage occurred, and it took three surgical interventions to repair it. External CSF drainage, reduction of the size of the caudal part of the dural sac and use of gluteus muscle flaps and collagen matrix worked together for the CSF leakage. Conclusion: Preoperative enlargement of the TMC, together with the surrounding subarachnoid space, can cause the refractory CSF leakage after untethering surgery because the expanded dural sac possibly increases its own tensile strength and impedes healing of the duraplasty. Early untethering surgery is recommended after recovery from the life-threatening conditions associated with OEIS complex.

Presurgical naso-alveolar molding paired with cheiloplasty to treat median cleft lip deformity in holoprosencephaly.

We report median cleft lip in an infant girl with lobar-typed holoprosencephaly who underwent presurgical naso-alveolar molding and subsequent cheiloplasty. At seven months postoperatively, we observed an upper lip with natural cupid-bow-shape formed with a nasal dome and two nostrils separated with reconstructed columella, which were maintained for eight years.

Rezoning Free Muscle-Sparing Transverse Rectus Abdominis Myocutaneous Flaps Based on Perforasome Groupings and a New Understanding of the Vascular Architecture of the Deep Inferior Epigastric Artery-Based Flaps.

BACKGROUND: We compare the vascular territory of free muscle-sparing transverse rectus abdominis myocutaneous (MS-TRAM) flaps, deep inferior epigastric perforator (DIEP) flaps, and crossover anastomosis (CA) flaps using intraoperative ex vivo angiography. We also use ex vivo angiography to analyze the vascular architecture of the MS-TRAM flap. METHODS: Our study includes 84 lower abdominal free flaps: MS-TRAM, DIEP-1 (1 perforator), DIEP-2 (2 perforators), and CA. We compare the arterial perfusion area and vascular territory pattern in each group. We also analyze the vascular architecture in MS-TRAM flaps and determine the number and location of their dominant perforators and the direction of the axial arteries connecting them. RESULTS: The CA's arterial perfusion area is the largest, and the DIEP-1's, the smallest of our groups; there is no statistically significant difference between MS-TRAM and DIEP-2. In all groups, average arterial perfusion area in the vascular pedicle's ipsilateral side is larger than in its contralateral side. The MS-TRAM and DIEP-2 flaps have homologous perfusion patterns and the same arterial perfusion areas. The DIEP-1 perfusion pattern varies with perforator location. Ex vivo angiograms show the MS-TRAM flap's axial arteries heading laterally to be larger and longer than those heading medially. CONCLUSIONS: Two dominant perforators are preferable in DIEP flap breast reconstruction. Lateral perforators play a more important role in flap perfusion than do medial ones. Crossover anastomosis is an effective technology for increasing arterial perfusion areas. Our rezoning shows which areas are better for surgery and which have a high risk of complications-valuable information for a surgeon designing a flap for breast reconstruction.

Ultrasonography for classifying lymphatic sclerosis types and deciding optimal sites for lymphatic-venous anastomosis in patients with lymphoedema.

We have previously categorised of degeneration of the collecting lymphatic vessels into four types: normal, ectasis, contraction and sclerosis type (NECST classification). Herein, we evaluated the collecting lymphatic vessels in lymphoedema-affected limbs using ultrasonography. In step 1, we investigated 110 lymphatic vessels from 25 patients with lymphoedema, who underwent lymphatic-venous anastomosis (LVA) following preoperative ultrasonography. We classified the lymphatic vessels using the NECST classification during intraoperative microscopic observation. Post-operatively, we evaluated the preoperative ultrasonographic images and identified the lymphatic vessels. In step 2, we investigated 79 lymphatic vessels from 17 patients. We performed ultrasonography and detected the lymphatic vessels preoperatively and compared the results with the intraoperative findings. This study is not blinded. In step 1, normal-type lymphatic vessels were observed as spicular and flat hypo-echoic lesions on ultrasonography. Ectasis-type lymphatic vessels appeared as a rounded hypo-echoic region and coloured on Doppler imaging once in 20-30 s. Contraction-type lymphatic vessels appeared as a small hypo-echoic region in the centre of the hyper-echoic ellipse. Sclerosis-type lymphatic vessels appeared as a hyper-echoic ellipse without lumen, similar to fibrotic tissues. In step 2, of 79 lymphatic vessels found intraoperatively, 65 (82.3%) were detected on ultrasonography and 37 (46.8%) were accurately diagnosed according to the NECST classification criteria preoperatively. All lymphatic vessels detected on ultrasonography were found intraoperatively. Collecting lymphatic vessels could be observed by ultrasonography in lymphoedema-affected limbs. Depending on the degree of collecting lymphatic vessel sclerosis-corresponding to the NECST classification-various findings such as spicular, rounded, hyper-echoic and similar to these were presented. Moreover, we can decide optimal sites for LVA preoperatively.

Site Specific Evaluation of Lymphatic Vessel Sclerosis in Lower Limb Lymphedema Patients.

BACKGROUND: Histological changes in the collecting lymphatics in patients with lymphedema are classified as Normal type, Ectasis type, Contraction type, and Sclerosis type (NECST) classification. In this study, we investigated the condition of the lymphatic vessels in different sites of the legs. PATIENTS AND METHODS: We prospectively investigated the lymphatic vessels of patients with lymphedema who underwent lymphaticovenous anastomosis (LVA) from August 8, 2014 to August 4, 2015 based on the NECST classification. Lymphedema was diagnosed using lymphoscintigraphy, indocyanine green (ICG) lymphography, and the International Society of Lymphology (ISL) Classification. The affected limbs were divided into four sites: proximal thigh (Site 1), distal thigh (Site 2), proximal crus (Site 3), and distal crus (Site 4). RESULTS: A total of 109 patients (205 limbs and 1028 lymphatics) were included in this study. Of the 109 patients, there were 100 women and 9 men with an average age of 61 years. The ratio of Ectasis type vessels increased toward the distal end of the limb with the highest occurrence rate being 54% at Site 4. As ISL stage, ICG stage, and lymphoscintigraphy stage advanced, so too did the ratio of Sclerosis type. In secondary lymphedema patients with lymphedema, the ratio of Ectasis type was more predominant in the distal end of the limb, whereas this tendency was not observed in primary lymphedema patients. CONCLUSIONS: Sclerotic lymphatics are more predominantly found in the proximal limb whereas nonsclerotic vessels are more often found toward the distal end. These findings help lymphatic surgeon determine incision sites.

Multi-site lymphatic venous anastomosis using echography to detect suitable subcutaneous vein in severe lymphedema patients.

BACKGROUND: The method of lymphatic venous anastomosis (LVA), including its indications or preoperative examinations, has not been established. The purpose of this study is to reveal the possible application of preoperative echography in surgical LVA outcome. METHODS: We performed a retrospective case-control study on patients with lower limb lymphedema who underwent LVA between August 15, 2013 and August 15, 2014. As a preoperative examination, we used venous echography to identify subcutaneous veins in the echo group, while we only used Accuvein visualizing system in the control group. The operation time, number of anastomoses, and limb circumference were compared between the two groups. RESULTS: Seventeen patients (34 limbs) were included in the echo group, and 21 patients (42 limbs) were included in the control group. The average follow-up period was 11.9 (6-16) and 12.4 (6-27) months, respectively. The average operation time in the echo group was 258.6 min, and that in the control group was 216.5 min. The average number of anastomoses was 9.8 and 7.0 in the echo and control group, respectively. The average time per anastomosis was 27.4 and 32.6 min, respectively. The diameter of the vein had a tendency to be larger in the echo group than in the control group. In 5.8% of the echo group, we observed a circumference increase, compared with 23.8% in the control group. CONCLUSIONS: Preoperative venous echography allowed surgeons to increase the number of anastomoses performed within the operating time, resulting in improvement of surgical outcomes.

The neck burn scar contracture: a concept of effective treatment.

A neck scar contracture can severely and negatively affect the function of mastication, phonic, or breathing and result in neck pain and issues with esthetics. The best way is of course to avoid such contracture by means of non-surgical treatment such as use of a growth factor. The basic fibroblastic growth factor is clinically well proven in decreasing scar formation and improving healing. There are numerous reconstructive methods for neck contracture, especially when the lesions are relatively limited in part of the neck. However, a very severe and full circumferential scar contracture requires extensive reconstruction. The thin groin flap is one of the answers and well matches with the tissue texture and maintains the flexibility. Even with extensive burns and delayed reconstructions due to resuscitation first, the groin area is well preserved and can be safely harvested by dual vasculature systems of the superficial circumflex iliac artery and superficial epigastric artery, which warrant more reliability compared to the perforator flaps in this area. More demanding and stringent forms of the neck burn scar contracture are the sequelae of radiation. A radiation burn or radiation injury can be progressing and hard to heal. Adipose-derived stem cells can reverse the scar contracture as the surrounding tissue is softened and can accelerate wound healing. In this review, different types of neck burn scar contracture and reconstructive methods are summarized, including innovative use of bFGF and ADSCs in the management of difficult wound healing and scar contracture.

Osteosarcoma of the patella mimicking giant cell tumor: imaging features with histopathological correlation.

Patellar tumors represent an uncommon etiology of anterior knee pain and their diagnosis is often delayed. We present an unusual case of conventional osteosarcoma arising in the patella of a 47-year-old man. The patient presented with a 1-year history of increasing anterior knee pain and swelling. Plain radiographs revealed a multi-locular lytic lesion in the inferolateral side of the patella. Computed tomography scans demonstrated an intraosseous lytic lesion with cortical thinning/breakthrough anteriorly. On magnetic resonance imaging, the lesion exhibited low signal intensity on T1-weighted images and heterogeneous high signal intensity on T2-weighted images. Fluid-fluid levels were also observed on T2-weighted images. Contrast-enhanced fat-suppressed T1-weighted images demonstrated strong enhancement of the lesion. These imaging features were suggestive of a benign condition; however, the diagnosis of osteosarcoma was confirmed by histopathology. After neoadjuvant chemotherapy, a wide resection with a free anterolateral thigh flap was performed. The patient subsequently underwent adjuvant chemotherapy and had no evidence of local recurrence or distant metastasis six months after surgery. Our case highlights the difficulty in the diagnosis of patellar osteosarcoma and the importance of performing a biopsy before definitive treatment.