Asteria Albert

Long-Term Follow-Up of Nineteen Cystic Lymphangiomas Treated With Fibrin Sealant

BACKGROUND Surgical exeresis is regarded as the first choice treatment for cystic lymphangioma. Surgery may be extremely complex, giving rise to complications. Several therapeutic methods have been described to avoid the complications derived from the conventional surgical approach. The idea of treating lymphangioma by means of suction and injection of fibrin sealant (Tissucol), is an alternative to surgery. METHODS The authors present 19 cases of cystic lymphangioma, treated with fibrin sealant injected into the lesion, during the 1991 to 1997 period. Two of the patients had been treated surgically and experienced recurrence of the tumor previous to treatment. In the other 17 cases, puncture was the only therapy applied. One patient required 3 punctures, another 6 patients required 2 punctures, and only 1 of them, after 2 unsuccessful punctures, was treated with surgical resection. The rest of the 10 cases subsided after the first puncture. Follow-up ranged between 3 and 72 months, with a mean of 40 months. RESULTS The ultrasonographic (US) follow-up showed a complete remission in 17 patients treated with puncture. One patient remained with a small intermittent tumor, the appearance of which is related to catarrhal processes, and another patient rejected further puncture after the second one. No complications appeared. CONCLUSION These results support the fact that the puncture, aspiration, followed by injection of Tissucol, is a choice in the surgical treatment of cystic lymphangioma.

Upper lip fistulas: three new cases.

OBJECTIVE We present three new cases of congenital upper lip fistula. Two of them were located in the philtrum midline, one of which was associated to a double maxillary frenulum, a medial lip cleft, and a medial cleft of the primitive palate. The other was located in the left side of the vermilion. All three patients had clear fluid discharge through the fistulous orifice without pain. Two of them had a history of recurrent swelling of the philtrum area. CONCLUSIONS A simple surgical excision is the treatment of choice in these cases, in which the anatomy is preserved; this fact is more consistent with a completed but aberrant development than with focal dysgenesis.

Antiangiogenic treatment as a pre-operative management of alveolar soft-part sarcoma†

Alveolar soft‐part sarcoma (ASPS) is a rare tumor. Cure is based solely on radical surgery. The general prognosis is poor. The tongue is an unusual site in adults, but not in children. Tumor removal can cause a severe impact on quality of life, even if reconstruction is possible. ASPS is a highly vascularized tumor and antiangiogenic therapy may have a role. We describe the use of the antiangiogenic combination bevacizumab and celecoxib in the preoperative management of a patient with an ASPS of the tongue. Pediatr Blood Cancer 2011; 57: 1071–1073.

Morphology and mucosal biochemistry of gastroschisis intestine in urine-free amniotic fluid.

BACKGROUND/PURPOSE The aim of this study was to determine the role of urine exposure in gastroschisis on the pathologic and biochemical aspects. METHODS The intestines of fetal rabbits with gastroschisis (group G), gastroschisis and urethral ligation (group GL), and normal controls (group C) were studied by measuring weigh and length, intestinal diameter and wall thickness, and thickness of each intestinal layer. Number and length of villi and villi edema were evaluated. Total protein and DNA were measured in intestinal homogenate. Lactase activity and alkaline phosphatase activity were analyzed in isolated microvilli membranes. RESULTS Intestinal length, diameter, and wall thickness were significantly different in groups G and GL compared with C but not between groups G and GL. The same was true for the thickness of the internal muscular and serosa, villi length, and villi edema. Serosal reaction was milder in group GL than in group G, and absent in C. Total protein, lactase activity, and alkaline phosphatase activity were also significantly different in G and GL compared with C but not between G and GL. CONCLUSIONS Urine in amniotic fluid causes an increased serosal reaction but does not account for the mucosal enzyme dysfunction.

Effect of the environment on fetal skin wound healing

Skin wound healing has been shown to be a different process in the fetus than in the adult animal. Some of these differences have been attributed to the unique fetal environment (i.e., amniotic fluid). The aim of the present study is to compare fetal skin healing in intraamniotic and extraamniotic wounds. A fetal rabbit model has been used in which three types of skin wounds were induced on 23-day-old fetuses in contact with either amniotic fluid or maternal peritoneal fluid. The wounds consisted of a sutured skin incision, a nonsutured incision, and an electrocautery burn. Seven days later all wounds were examined mechanically (scar resistance), biochemically (collagen and noncollagen protein concentration), and histologically. Biochemical and growth factor studies of both environments, the amniotic and the peritoneal fluids, were also conducted. The results showed excellent healing by first intention and absence of healing by second intention in both environments, greater scar resistance in the intraperitoneally positioned fetal wounds, and a higher concentration of insulin-like growth factor-1 (IGF-1) in the peritoneal fluid than in the amniotic fluid. It can be concluded that fetal skin wounds in contact with peritoneal fluid show the same healing pattern as in the natural fetal environment (amniotic fluid). The higher concentration of IGF-1 in the peritoneal fluid suggests that this growth factor, through its relationship with the growth hormone, plays a role in increasing the scar resistance of fetal skin wounds in contact with maternal peritoneal fluid.

Wound healing in the fetal period: The resistance of the scar to rupture

Wound healing in the fetal period is fundamentally different from that of the adult. In order to better understand this difference, we have studied wound healing in three types of wounds which are the most common in surgical practice, and have paid special attention to tensile strength in the scar. A sutured wound, a nonsutured wound, and an electrocautery burn were performed on a group of 30 rabbit fetuses with a gestational age of 23 days. Seven days later, the resulting scars were examined using histological, mechanical, and biochemical studies. The results were compared with those obtained in a group of 30 newborn rabbits and in a group of 30 adult rabbits who had had the same type of wounds performed on them. The body weight of operated fetuses was smaller than their control siblings. The sutured wound in the fetuses showed a more perfect macroscopic repair than in older subjects. However, the nonsutured wounds and the burns of the fetal group did not close. In the fetuses, the tensile strength of the sutured wound was 20% of the tensile strength of healthy skin. A similar relationship occurred in the group of adult rabbits. In the sutured wound of the fetal group, the synthesis of collagen was significantly less than in the adult group. The ratio between hydroxyproline (HYP) concentration in the sutured wound and HYP concentration in healthy skin was also significantly less in the fetal group than in the adult group.(ABSTRACT TRUNCATED AT 250 WORDS)

Lung Tissue Blood Perfusion Changes Induced by in utero Tracheal Occlusion in a Rabbit Model of Congenital Diaphragmatic Hernia

Objective: To analyze the impact of in utero tracheal occlusion (TO) on lung tissue blood perfusion, as measured by fractional moving blood volume (FMBV) and conventional spectral Doppler, in a rabbit model of congenital diaphragmatic hernia (CDH). Methods: In 50 fetal rabbits, a left CDH was surgically created at 23 days of gestational age (GA). At 28 days of GA, the surviving CDH fetuses were randomly assigned to undergo either TO (CDH+TO group) or a sham operation (CDH group). Twenty littermates, which were not operated on, served as internal normal controls. At 30 days of GA, lung perfusion estimated by FMBV and spectral Doppler of the proximal intrapulmonary artery were evaluated in the right lung during cesarean section. Doppler waveform analysis included the pulsatility index (PI), peak early diastolic reverse flow and peak systolic velocity. Results: Eleven CDH fetuses, 9 CDH+TO and 20 controls were suitable for the study. CDH fetuses showed a significantly higher PI [8.0 (SD 1.8) vs. 5.22 (SD 1.1), p < 0.001] and lower FMBV [13.5% (SD 4.6) vs. 23.0% (SD 2.1), p < 0.001] than the controls. In contrast, CDH+TO fetuses had a significantly lower PI [5.8 (SD 2.3) vs. 8.0 (SD 1.8), p = 0.015] and higher FMBV [27.6% (SD 7.1) vs. 13.5% (SD 4.6), p < 0.001] than CDH fetuses, with values similar to the controls. Peak early diastolic reverse flow and peak systolic velocity showed nonsignificant differences among the study groups. The lung to body weight ratio at necropsy correlated positively with lung FMBV (r = 0.60, p < 0.001) and negatively with the pulmonary artery PI (r = –0.48, p < 0.01). Conclusion: Tracheal occlusion is consistently associated with increased lung tissue perfusion and decreased intrapulmonary impedance in a rabbit model of CDH.

Use of angioembolization as an effective technique for the management of pediatric solid tumors

PURPOSE In oncology practice, angioembolization has been reported for tumor reduction before surgery, treatment of life-threatening conditions, and for palliative care. Nevertheless, the overall experience with angioembolization for the treatment of tumors is limited. We report our experience in 7 nonvascular solid pediatric tumors. MATERIALS AND METHODS A retrospective review was carried out of medical records from pediatric patients (0-18 years) with solid nonvascular tumors who underwent angioembolization in the last 5 years at our institution. RESULTS Seven patients underwent embolization: 2 neuroblastomas, 1 metastatic paraganglioma, 1 hepatoblastoma, 1 myofibroblastic tumor, 1 osteosarcoma, and 1 undifferentiated sarcoma. The reason for angioembolization was preparation for surgery (3), treatment of a life-threatening event (1), or palliative care (3). Each case is presented and discussed. The outcome was subsequent complete surgical resection in 3 cases, tumor vanished in 1 case, symptom control was achieved in 1, and the other 2 patients improved their survival and quality of life, however, died of disease progression. CONCLUSIONS Tumor angioembolization may enter the treatment algorithm for selected patients who have to face difficult or unwarranted surgical procedures or have diseases where conventional therapies have failed.

Congenital epigastric evisceration: a case report

A rare case of abdominal wall defect at the epigastric midline is presented. This newborn boy had only the greater omentum eviscerated, and no other abnormalities could be detected. This case does not seem to relate to the abdominal wall defects reported so far in the literature. This singular case contributes to enlarge the spectrum of congenital defects of the abdominal wall.

A standard set of outcome measures for the comprehensive appraisal of cleft care

Care of the patient with cleft lip and/or palate remains complex. Prior attempts at aggregating data to study the effectiveness of specific interventions or overall treatment protocols have been hindered by a lack of data standards. There exists a critical need to better define the outcomes- particularly those that matter most to patients and their families-and to standardize the methods by which these outcomes will be measured. This report summarizes the recommendations of an international, multidisciplinary working group with regard to which outcomes a typical cleft team could track, how those outcomes could be measured and recorded, and what strategies may be employed to sustainably implement a system for prospective data collection. It is only by agreeing on a common, standard set of outcome measures for the comprehensive appraisal of cleft care that intercenter comparisons can become possible. This is important for quality-improvement endeavors, comparative effectiveness research, and value-based health-care reform.