Safe and sustainable: the extracranial approach toward frontoethmoidal meningoencephalocele repair.

OBJECTIVE Although rare, frontoethmoidal meningoencephaloceles continue to pose a challenge to neurosurgeons and plastic reconstructive surgeons. Especially when faced with limited infrastructure and resources, establishing reliable and safe surgical techniques is of paramount importance. The authors present a case series in order to evaluate a previously proposed concise approach for meningoencephalocele repair, with a focus on sustainability of internationally driven surgical efforts. METHODS Between 2001 and 2016, a total of 246 patients with frontoethmoidal meningoencephaloceles were treated using a 1-stage extracranial approach by a single surgeon in the Department of Neurosurgery of the Yangon General Hospital in Yangon, Myanmar, initially assisted by European surgeons. Outcomes and complications were evaluated. RESULTS A total of 246 patients (138 male and 108 female) were treated. Their ages ranged from 75 days to 32 years (median 8 years). The duration of follow-up ranged between 4 weeks and 16 years (median 4 months). Eighteen patients (7.3%) showed signs of increased intracranial pressure postoperatively, and early CSF rhinorrhea was observed in 27 patients (11%), with 5 (2%) of them requiring operative dural repair. In 8 patients, a decompressive lumbar puncture was performed. There were 8 postoperative deaths (3.3%) due to meningitis. In 15 patients (6.1%), recurrent herniation of brain tissue was observed; this herniation led to blindness in 1 case. The remaining patients all showed good to very good aesthetic and functional results. CONCLUSIONS A minimally invasive, purely extracranial approach to frontoethmoidal meningoencephalocele repair may serve well, especially in middle- and low-income countries. This case series points out how the frequently critiqued lack of sustainability in the field of humanitarian surgical missions, as well as the often-cited missing aftercare and dependence on foreign supporters, can be circumvented by meticulous training of local surgeons.

De novo mutations in SMCHD1 cause Bosma arhinia microphthalmia syndrome and abrogate nasal development.

Bosma arhinia microphthalmia syndrome (BAMS) is an extremely rare and striking condition characterized by complete absence of the nose with or without ocular defects. We report here that missense mutations in the epigenetic regulator SMCHD1 mapping to the extended ATPase domain of the encoded protein cause BAMS in all 14 cases studied. All mutations were de novo where parental DNA was available. Biochemical tests and in vivo assays in Xenopus laevis embryos suggest that these mutations may behave as gain-of-function alleles. This finding is in contrast to the loss-of-function mutations in SMCHD1 that have been associated with facioscapulohumeral muscular dystrophy (FSHD) type 2. Our results establish SMCHD1 as a key player in nasal development and provide biochemical insight into its enzymatic function that may be exploited for development of therapeutics for FSHD.

Heinrich von Pfalzpaint, Pioneer of Arm Flap Nasal Reconstruction in 1460, More Than a Century Before Tagliacozzi.

Heinrich von Pfalzpaint (circa 1415-1465) was a Bavarian military surgeon of the Teutonic Order who treated more than 4000 casualties during the siege of Marienberg Fortress (1454-1457). In 1460, he reported "How to create a new nose if it has been chopped off and the dogs have eaten it" in his treatise on wound care Bündt-Ertznei. He used opium-soaked sponges for anesthesia, described the surgical extraction of bullets and cleft lip repair. Pfalzpaint would have been the first author to describe nasal reconstruction in Europe if his treatise had not been lost. Only 5 copies of his manuscript existed. One was rediscovered and printed in 1868. Pfalzpaint's technique for nasal reconstruction was performed in 2 stages using an undelayed skin flap from the upper arm, which was sutured to the nasal defect and the arm was bandaged to the head. After 8 to 10 days, he divided the pedicle; inset the flap; and fashioned the nasal dorsum, alae, and columella. Tagliacozzi described arm flap nasal reconstruction more than a century later in 1597. He used delayed skin flaps, with at least 6 operative stages over 4 months. Pfalzpaint was ahead of his time regarding his knowledge of wounds, insistence on surgical cleanliness, and his technically easier arm flap rhinoplasty, compared with Tagliacozzi. Pfalzpaint, who is rarely referenced in the literature, should be remembered as a great pioneer of reconstructive surgery in Europe.

An outcome analysis and long-term viability of cryopreserved cultured epidermal allografts: assessment of the conservation of transplantable human skin allografts.

PURPOSE: To assess the viability of cultured epithelium and preserved by freezing for periods varying from one month to one year. METHODS: Samples of cultured epithelium were incubated in cryoprotectant medium (Group A), packed in aluminum envelopes and packed in polystyrene boxes. The boxes were subjected to a temperature of-70 ºC. After freezing for a period of time ranging from one to 12 months, cultured epithelial samples were assessed for their viability by vital staining (Trypan blue) and metabolic analysis based on glucose consumption and lactate production. Samples of not frozen cultured epithelium (Group B) were also tested for viability and the results obtained were used as comparison parameter for the variation of viability. RESULTS: Statistical analysis between the group A and B indicate that the mean age of the donors (p=0.51) and the culture time (p=1.18) showed no statistical difference. In 30 days we obtained 37% of the original viability of cultured epithelium, 25% at six months and one year, less than 15%. This trend was confirmed statistically with a reduction of approximately 1.8% of the original viability epithelium cultured every 30 days of storage. In the analysis by lactate production, similar results were observed. In the analysis by the glucose consumption results were not significant. The viability indices show statistically significant difference between the group A and B (p<0.0001). CONCLUSIONS: Although cryopreserved cultured epithelium showed significant reduction of viability, all samples remained viable. It was also found that the viability of cryopreserved cultured epithelial decreased as a function of storage time.

An outcome analysis and long-term viability of cryopreserved cultured epidermal allografts: assessment of the conservation of transplantable human skin allografts

PURPOSE: To assess the viability of cultured epithelium and preserved by freezing for periods varying from one month to one year. METHODS: Samples of cultured epithelium were incubated in cryoprotectant medium (Group A), packed in aluminum envelopes and packed in polystyrene boxes. The boxes were subjected to a temperature of-70ºC. After freezing for a period of time ranging from one to 12 months, cultured epithelial samples were assessed for their viability by vital staining (Trypan blue) and metabolic analysis based on glucose consumption and lactate production. Samples of not frozen cultured epithelium (Group B) were also tested for viability and the results obtained were used as comparison parameter for the variation of viability. RESULTS: Statistical analysis between the group A and B indicate that the mean age of the donors (p=0.51) and the culture time (p=1.18) showed no statistical difference. In 30 days we obtained 37% of the original viability of cultured epithelium, 25% at six months and one year, less than 15%. This trend was confirmed statistically with a reduction of approximately 1.8% of the original viability epithelium cultured every 30 days of storage. In the analysis by lactate production, similar results were observed. In the analysis by the glucose consumption results were not significant. The viability indices show statistically significant difference between the group A and B (p<0.0001). CONCLUSIONS: Although cryopreserved cultured epithelium showed significant reduction of viability, all samples remained viable. It was also found that the viability of cryopreserved cultured epithelial decreased as a function of storage time.

Congenital arhinia: molecular-genetic analysis of five patients.

Congenital arhinia, complete absence of the nose, is an extremely rare anomaly with unknown cause. To our knowledge, a total of 36 cases have been reported, but there has been no molecular-genetic study on this anomaly. We encountered a sporadic case of congenital arhinia associated with a de novo chromosomal translocation, t(3;12)(q13.2;p11.2). This led us to analyze the patient by BAC-based FISH for translocation breakpoints and whole-genome array CGH for other possible deletions/duplications in the genome. We found in this patient an approximately 19 Mb deletion spanning from 3q11.2 to 3q13.31 but no disruption of any gene(s) at the other breakpoint, 12p11.2. As the deleted segment at 3q was a strong candidate region containing the putative arhinia gene, we also performed the array CGH in four other arhinia patients with normal karyotypes, as well as mutation analysis of two genes, COL8A1 and CPOX, selected among hundreds of genes located to the deleted region, because they are expressed during early stages of human craniofacial development. However, in the four patients, there were no copy number aberrations in the region examined or no mutations in the two genes. Although our study failed to identify the putative arhinia gene, the data may become a clue to unravel the underlying mechanism of arhinia.

Assessment of splanchnic perfusion by gastric tonometry in patients with acute hypovolemic burn shock.

OBJECTIVE: To compare the changes in pHi and intramucosal-arterial CO(2)-gap with invasive haemodynamic and global perfusion measurements during hypovolemic burn shock and to evaluate the sensitivity of these parameters as an early predictor of mortality in patients with extensive burns. DESIGN: Prospective, controlled, clinical study. SETTING: An eight-bed intensive burn care unit in a university-affiliated hospital. PATIENTS: Fifty severely burned patients with TBSA burned >25% BSA. METHODS: During the first 48h after burn, gastric intramucosal CO(2) was measured every 8h using automated air tonometry. pHi and intramucosal-arterial CO(2)-gap were calculated. Simultaneously invasive haemodynamic data were registered by the transpulmonary thermodilution technique, using the mean of triplicate injections. The intramucosal-arterial CO(2)-gradient and pHi were compared with haemodynamic and global perfusion data by regression analysis. Mean pHi and CO(2)-gap values at 8 and 24h after injury were compared between survivors and non-survivors to evaluate the prognostic significance of this parameter. RESULTS: Regression analyses revealed no or a negligible correlation between intramucosal and haemodynamic or perfusion data, even during the critical low flow-high resistance phase of resuscitation. Mean pHi and PCO(2)-gap at 8 and 24h did not differ significantly between survivors and non-survivors. CONCLUSION: Gastric tonometry is a poor indicator of splanchnic perfusion in patients with burn shock, even when all precautions are taken to prevent methodological errors. The intramucosal-arterial PCO(2)-gap and pHi do not distinguish survivors from non-survivors. Therefore, gastric tonometry does not seem to improve the ability to anticipate and avert regional anaerobic metabolism during burn shock and its routine use in these patients cannot be recommended.

Computer imaging and surgical reality in aesthetic rhinoplasty.

BACKGROUND: Computer imaging enables a surgeon to simulate the postoperative result in a quite artistic and natural manner. Many plastic surgeons, however, are reluctant to use this sophisticated new tool for various reasons, such as the cost of the equipment, the learning process, the extra time involved, and the potential medicolegal implications. The aim of this study was to add experience to the value of computer imaging for rhinoplasty. METHODS: One hundred twenty patients with corrective rhinoplasty were followed from 1 to 5 years postoperatively. The authors and the patients compared the "virtual" with the surgical results on a scale from 1 to 4, where 1 = identical, 2 = similar, 3 = approximate, and 4 = poor. RESULTS: Approximately 70 percent of the surgical results were rated identical or similar by the authors versus 80 percent by the patients, 25 percent versus 14 percent were rated approximate, and only 5 percent versus 2.5 percent were rated as poor. The outcome of the matches between the simulation and the postoperative results correlates favorably with the authors' experiences during the precomputer era. The patients who were satisfied with the outcome in general were not critical about the accuracy of the match. So far, the authors have not experienced medicolegal problems. CONCLUSIONS: The authors have been using computer imaging for more than 5 years, with growing enthusiasm; it has become a valuable tool with which to communicate potential results to the patient during consultation and planning of the procedure. The extra time necessary is well invested. The available software programs are affordable and easy to handle, even for nonexperts.

Reproducibility of transpulmonary thermodilution measurements in patients with burn shock and hypothermia.

Thermal noise, including hypothermia and short-term variations in body temperature, has been reported to influence the accuracy and reproducibility of thermodilution measurements. This variation might theoretically limit the usefulness of this technique in patients with acute burns. We therefore sought to determine the reproducibility of hemodynamic parameters derived from arterial thermal dilution in patients with acute burns and hypothermia. This was a prospective, clinical study of 50 mechanically ventilated patients with burns involving more than 25% of the body surface area that were treated at an eight-bed intensive care burn unit in a university-affiliated hospital. A total of 750 arterial thermodilution measurements were analyzed using the COLD system. Triplicate measurements of the intrathoracic blood volume, cardiac output, total blood volume, and extravascular lung water were performed at regular intervals during the first 48 hours after the thermal injury. Reproducibility was assessed by the coefficient of variation of the triplicate measurements. The correlation of variation was less than 10% at all measurement times for cardiac output, intrathoracic blood volume, and total blood volume. For the extravascular lung water, the coefficient of variation ranged from 9.5% to 12.9%. A maximum of 12.9% was found at 48 hours after burn. No correlation was found between body core temperature and the reproducibility of intrathoracic blood volume index (r = 0.145), cardiac index (r = 0.217), or extravascular lung water index (r = 0.167). The parameters derived from arterial thermodilution show a clinically sufficient reproducibility in patients with acute burns associated with thermal instability.

Effects of aesthetic abdominoplasty on abdominal wall perfusion: a quantitative evaluation.

Abdominoplasty procedures involve a high risk of early complications, including hematomas, seromas, necrosis, and wound-healing problems. Their rationale is evident from the vascular anatomy of the abdominal wall, as traditional abdominoplasty includes a division of the main perforating vessels. No studies exist to quantitatively assess the consequences of abdominoplasty on the perfusion of the random pattern abdominal flap. To address this issue and quantify the influence of classic abdominoplasty on the perfusion of the abdominal skin, the authors performed a prospective clinical trial including 15 low-risk patients undergoing abdominoplasty for aesthetic purposes. Perfusion of the abdominal flap was measured intraoperatively using the technique of dynamic laser-fluorescence-videoangiography. In the region between the umbilicus and the transverse scar (zone 1), the increment of fluorescence (the slope of the intensity curve during inflow of the indocyanine green) was recorded and compared with the intensity curve of normal tissue that was not involved in surgery (thoracic wall). The results of the intraoperative indocyanine green perfusography showed a significant impairment of the vascular supply of zone 1 in all patients. The mean perfusion index in this region was 17.2 percent (range, 5 to 32 percent) of the perfusion of the surrounding skin that was not involved in surgery. The complication rate was 33 percent (five patients) and included two cases of hematoma and three cases of scar dehiscence with skin and/or fat necrosis. These data indicate that conventional abdominoplasty including extended undermining and division of the superficial and the deep arterial systems causes profound devascularization of the abdominal flap. This might explain the high incidence of complications following this procedure.

Effect of crystalloid resuscitation and inhalation injury on extravascular lung water: clinical implications.

STUDY OBJECTIVE: Arterial thermal dilution with an integrated fiberoptic monitoring system (COLD Z-021; Pulsion Medical Systems; Munich, Germany) allows measurement of extravascular lung water (EVLW) and pulmonary permeability index (PPI). The aim of this study was to evaluate the widespread clinical assumption that early respiratory failure following burn and inhalation injury is due to interstitial fluid accumulation in the lung. DESIGN: Clinical, prospective study. SETTING: ICU of a university referral center of burn care. PATIENTS: Thirty-five severely burned adults (> 20% of body surface area). INTERVENTIONS: Resuscitation therapy was guided by the results of hemodynamic monitoring using the intrathoracic blood volume (ITBV) as a cardiac preload indicator. The resuscitation goals included a normalization of preload (ITBV > 850 mL/m(2)) and cardiac index (> 3.5 L/min/m(2)) within 24 h after ICU admission. Fluid loading was implemented to reach these goals. MEASUREMENTS AND RESULTS: One hundred forty lung water measurements were performed at 0 h, 12 h, 24 h, and 48 h after admission to the ICU. Significant elevation of EVLW and PPI was found in three measurements (2%) at 48 h after ICU admission, and was in one patient associated with inhalation injury. EVLW and PPI were not significantly different between patients with and without inhalation injury. No correlation was found between resuscitation volume and EVLW (r(2) = 0.02) or between the alveolar-arterial oxygen pressure difference and EVLW (r(2) = 0.017). Chest radiograph abnormalities were found in 2 of 22 patients with inhalation injury; these were not associated with increased values of EVLW. CONCLUSION: Early fluid accumulation in the lung in burned patients is very uncommon, even in the presence of inhalation injury. There is no evidence that thermal injury causes an increase in pulmonary capillary membrane permeability.