Management Challenges in an Infant With Pentalogy of Cantrell, Giant Anterior Encephalocele, and Craniofacial Anomalies: A Case Report

Pentalogy of Cantrell is a rare syndrome consisting of midline abnormalities involving the heart, sternum, abdominal wall, and the anterior and pericardial diaphragm. This combination of defects places patients at particular perioperative risk and requires individualized management during anesthetic care. The following report documents the management of a patient with pentalogy of Cantrell, whose condition was further complicated by severe midline craniofacial abnormalities, including large anterior encephalocele, deficient mandible, tethered tongue, and cleft palate. The case offers insight into the complexity of care in this unique patient population.
Source: A&A Case Reports - Category: Anesthesiology Tags: Case Reports Source Type: research

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Condition:   Intravenous Anesthesia Interventions:   Drug: Fentanyl´╝îRemifentanil;   Drug: Ketamine,Dexmedetomidine Sponsor:   Shanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
ConclusionsThis study describes a modified method for whistling deformity reconstruction in uni- and bilateral clefts. The aesthetic results are based on a reconstruction of the subcutaneous muscle layers and the creation of a symmetrical lip contour and prolabium using transposition flaps from the lateral side of the cleft. The great advantage is the uncomplicated performance under local anesthesia, even for all children over 10 years, and the short operation time. Postoperative complications did not occur..
Source: Journal of Cranio Maxillofacial Surgery - Category: ENT & OMF Source Type: research
Abstract BACKGROUND: Malaria is a common problem throughout the world, particularly in sub-Saharan Africa, where 90% of all deaths in the world from malaria occur. While many studies on malaria are available in the medical literature, few publications have addressed the problems of managing malaria during surgery and anesthesia. At a newly opened hospital in Niger, we initiated further studies to evaluate our process of managing malaria when we had a number of problems in our first group of pediatric patients having elective cleft lip and palate repairs. Many patients had fevers during and soon after surgery and w...
Source: Anesthesia and Analgesia - Category: Anesthesiology Authors: Tags: Anesth Analg Source Type: research
Conclusions: Although adverse events following cleft palate surgery are rare, systemic disease remains the greatest predictor for readmission and reoperation. Neurologic and pulmonary diseases are the greatest systemic risk factors. Intraoperative adverse events requiring blood transfusion are the greatest surgical risk factor for post-surgical complications.
Source: Journal of Craniofacial Surgery - Category: Surgery Tags: Original Articles Source Type: research
Rationale: Insignifificant, asymmetrical or lack of labial tubercle often occurs after cleft lip surgery due to improper treatment of vermilion tissue. Especially in the cases of bilateral cleft lip, because of short front lip and insuffificient vermilion tissue, the median vermilion depression often occurs after surgery, forming a “whistling” deformity. The object of this study is to verify the outcomes of patients with median labial tubercle detects after treatment with bilateral vermilion musculomucosal sliding flflaps (VMSF). Patient concerns: Six patients with median labial tubercle defect after bilat...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
This study was approved by the Animal Experimental Ethics Committee of Southern Medical University, Guangzhou, China (2014-024). All experiments were performed under protocols to meet the requirements of the Association for Assessment and Accreditation of Laboratory Animal Care International and the Experimental Animal Center of Southern Medical University. Male cynomolgus monkeys (Macaca fascicularis) were used, preliminary power analysis being performed to get the proper sample size for three groups (G∗Power 3.1, Heinrich-Heine-Universität Düsseldorf, Germany; alpha = 0.05, power = 0.95; total N = 20 hem...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
Alexandra Mills†, Elizabeth Bearce†, Rachael Cella, Seung Woo Kim, Megan Selig, Sangmook Lee and Laura Anne Lowery* Biology Department, Boston College, Chestnut Hill, MA, United States Wolf-Hirschhorn Syndrome (WHS) is a human developmental disorder arising from a hemizygous perturbation, typically a microdeletion, on the short arm of chromosome four. In addition to pronounced intellectual disability, seizures, and delayed growth, WHS presents with a characteristic facial dysmorphism and varying prevalence of microcephaly, micrognathia, cartilage malformation in the ear and nose, and facial asymmetr...
Source: Frontiers in Physiology - Category: Physiology Source Type: research
We present the case of a female patient with an isolated Tessier 30 facial cleft affecting the tongue, lower lip, and mandibular symphysis with ankyloglossia. This was reconstructed with a template-guided resorbable "U"-shaped plate at 10 months of age. The procedure was carried out in one stage, which avoided the need for a repeat general anesthetic for the patient. We had a successful outcome with normal dental eruption and we believe such an approach could be considered as a relevant treatment modality for future cases. PMID: 30971098 [PubMed - as supplied by publisher]
Source: The Cleft Palate-Craniofacial Journal - Category: ENT & OMF Authors: Tags: Cleft Palate Craniofac J Source Type: research
CONCLUSIONS:: Linear anthropometric measurements taken from 3D surfaces generated from a single stereophotograph correlate closely with direct anthropometric measures. This improves workflow and applicability of anthropometric studies to our youngest patients. PMID: 30955356 [PubMed - as supplied by publisher]
Source: The Cleft Palate-Craniofacial Journal - Category: ENT & OMF Authors: Tags: Cleft Palate Craniofac J Source Type: research
Abstract Wide cleft palates (>15-mm gap) present a number of challenges to the surgeon tasked with their repair ( Bardach, 1999 ). Eliminating the need for secondary surgery due to fistula formation can reduce additional anesthetic and scarring risks and optimize early speech development. Greater palatine foraminal osteotomy is a useful surgical adjunct that allows additional medial movement of oral mucoperiosteal flaps to aid in tension-free closure of the oral layer. We use a technique similar to that described by Seibert in 1995 with a few modifications. Closure of the nasal layer in these wide clefts can be...
Source: The Cleft Palate-Craniofacial Journal - Category: ENT & OMF Authors: Tags: Cleft Palate Craniofac J Source Type: research
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