Airway Obstruction during Sleep due to Diaphragm Pacing Precludes Decannulation in Young Children with CCHS.

Children with congenital central hypoventilation syndrome (CCHS) have a PHOX2B mutation-induced control of breathing deficit necessitating artificial ventilation as life support. A subset of CCHS families seek phrenic nerve-diaphragm pacing (DP) during sleep with the goal of tracheal decannulation. Published data regarding DP during sleep as life support in the decannulated child with CCHS and related airway dynamics in young children are limited. We report a series of 3 children, ages 3.3-4.3 years, who underwent decannulation. Sleep endoscopy performed during DP revealed varied (oropharynx, supraglottic, glottic, etc.) levels of complete airway obstruction despite modification of pacer settings. Real-time analysis of end tidal CO2 and SpO2 confirmed inadequate gas exchange. Because the families declined re-tracheostomy, all 3 patients rely on noninvasive mask ventilation as a means of life support while asleep. These results emphasize the need for extreme caution in proceeding with tracheal decannulation in young children with CCHS who expect to use DP during sleep as life support. Parents and patients should anticipate that they will depend on noninvasive mask ventilation (rather than DP) during sleep after undergoing decannulation. This information may improve management and guide expectations regarding potential decannulation in young paced children with CCHS.

Perioperative anesthetic management of children with congenital central hypoventilation syndrome and rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation undergoing thoracoscopic phrenic nerve-diaphragm pacemaker implantation.

BACKGROUND: Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation are rare neurocristopathies characterized by autonomic dysregulation including bradyarrhythmias, abnormal temperature control, and most significantly, abnormal control of breathing leading to tracheostomy and ventilator dependence as life support. Surgical advancements have made phrenic nerve-diaphragm pacemakers available, to eliminate the tether to a mechanical ventilator for 12-15 hours each day. The thoracoscopic approach to implantation has allowed for a less invasive approach which may have implications for pain control and recovery time. However, thoracoscopic implantation of these devices presents several challenges to the anesthesiologist in these complex ventilator-dependent patients, including, but not limited to, sequential lung isolation, prevention of hypothermia, and management of arrhythmias. Postoperative challenges may also include strategies to treat hemodynamic instability, managing the ventilator following lung derecruitment, and providing adequate pain control. AIMS: We aimed to describe the anesthetic management of Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation patients undergoing thoracoscopic phrenic nerve-diaphragm pacemaker implantation and the nature and incidence of perioperative complications. METHODS: A retrospective chart review was performed of 14 children with Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation undergoing phrenic nerve-diaphragm pacemaker implantation at a single academic pediatric hospital between 2009 and 2017. Demographic information, intraoperative management, and perioperative complications were analyzed from patient records. RESULTS: Twelve of 14 patients (86%) underwent an inhalational induction via tracheostomy. Lung isolation was achieved via fiberoptic guidance of a single lumen endotracheal tube sequentially into the right or left mainstem bronchi for 12 patients (86%). Double lumen endotracheal tubes were utilized in two patients (7%) and bronchial blockers in two patients (7%) for lung isolation. Anesthesia was maintained using a balanced technique of volatile agents (sevoflurane/isoflurane) and opioids (fentanyl). Bradyarrhythmias developed in six patients (43%) during surgery, 5 (36%) responded to anticholinergics and one patient (7%) required backup cardiac pacing using a previously implanted bipolar cardiac pacemaker. Intraoperative hypothermia (<35.5°C) was present in five patients (36%) despite the use of warming devices. Hypercarbia (>50 mm Hg) during lung isolation was present in eight patients (57%) and hemoglobin desaturation (<90%) in four patients (29%). Postoperatively, oxygen desaturation was a common complication with nine patients (64%) requiring supplemental oxygen administration via mechanical ventilator or manual bag ventilation. Opioids via patient-controlled analgesia devices (12 patients, 86%) or intermittent injection (two patients, 14%) were administered to all patients for postoperative pain control. Phrenic nerve-diaphragm pacemaker placement was successful thoracoscopically in all patients with no perioperative mortality. CONCLUSION: The main anesthetic challenges in patients with Congenital Central Hypoventilation Syndrome and Rapid-Onset Obesity with Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation include hemodynamic instability, the propensity to develop hypothermia, hypercarbia/hypoxemia, and the need to perform bilateral sequential lung isolation requisite to the thoracoscopic implantation technique. Most anesthetic agents can be used safely in these patients; however, adequate knowledge of the susceptibility to complications, coupled with adequate preparation and understanding of the innate disease characteristics, are necessary to treat anticipated complications.

Accessory hemi diaphragm.

BACKGROUND/PURPOSE: Accessory hemi diaphragm (AHD) is an uncommon condition. Its clinical features, diagnosis and management have not been clearly defined. METHODS: We reviewed the world literature and added a case of our own. RESULTS: There are 40 proven cases in the literature. The lesion occurs almost exclusively in the right hemithorax. Twenty seven cases presented with cardiovascular and/or respiratory symptoms while 3 had symptoms pertaining to other systems. In the remaining 10 patients the diagnosis was either made accidentally or information pertaining to their presentation is missing. Abnormalities of pulmonary and systemic blood vessels often accompany the abnormality. The diagnosis is usually suspected because of an abnormal chest radiograph and confirmed by a variety of tests. CONCLUSIONS: If the AHD prevents proper aeration of the lung it should be excised. If vascular abnormalities coexist they should be treated along with the AHD. Asymptomatic patients may be observed after confirming the diagnosis.

Validation of measures from a thoracoscopic esophageal atresia/tracheoesophageal fistula repair simulator.

PURPOSE: A validated high fidelity simulation model would provide a safe environment to teach thoracoscopic EA/TEF repair to novices. The study purpose was to evaluate validity evidence for performance measures on an EA/TEF simulator. METHODS: IRB-exempt data were collected from 12 self-reported "novice" and 8 "experienced" pediatric surgeons. Participants evaluated the EA/TEF repair simulator using survey ratings that were analyzed for test content validity evidence. Additionally, deidentified operative performances were videotaped and independently rated by two surgeons using the Objective Structured Assessment for Technical Skills (OSATS) instrument. Novice and experienced OSATS were compared with p<.05 significant. RESULTS: Participants had high overall simulator ratings. Internal structure was supported by high interitem consistency (α=.95 and .96) and interrater agreement (ICC) [.52, .84] for OSATS ratings. Experienced surgeons performed at a significantly higher level than novices for all five primary and two supplemental OSATS items (p<.05). CONCLUSION: Favorable participant ratings indicate the simulator is relevant to clinical practice and valuable as a learning tool. Further, performance ratings can discriminate experienced and novice performances of EA/TEF repair. These findings support the use of the simulator for performance assessment, representing the first validated measures from a simulator intended for pediatric surgical training.

Incidental GIST after appendectomy in a pediatric patient: a first instance and review of pediatric patients with CD117 confirmed GISTs.

A 7-year-old boy underwent uncomplicated laparoscopic appendectomy for acute appendicitis. Incidentally, he was found to have a spindle cell tumor with CD117 immunopositivity, consistent with gastrointestinal stromal tumor (GIST) in the appendix. Although commonly reported in adults, pediatric GISTs are rare gastrointestinal malignancies that occur in only 1.4-2.7% of children and adolescents. Due to the paucity of reports, data are insufficient to adequately characterize tumor behavior, recurrence, and survival. We present the first case of pediatric GIST in the appendix. In addition, a review of the literature for CD117 confirmed pediatric GISTs was conducted to summarize its clinical features and current treatment options.

Fate of the uterus in 46XX cloacal exstrophy patients.

PURPOSE: Genetically female cloacal exstrophy (46XX CE) patients develop complications later in life due to their abnormal uterine anatomy, resulting in various invasive gynecologic procedures. Furthermore, they have difficulty becoming pregnant, and if they do conceive, they are unlikely to carry the pregnancy to term. We performed this review to determine the rate of gynecological complications, the fate of the uterus, and the rate of pregnancy in 46XX cloacal exstrophy patients. METHODS: All charts for 46XX CE patients treated by us were reviewed following IRB approval. Patient age at last follow-up, surgical management of the uterus, uterine complications, and pregnancies were recorded. RESULTS: The charts of all 16 of our 46XX CE patients who survived past the neonatal period were reviewed. Two patients underwent hemi-hysterectomy (HH): 1 for an atretic hemi-uterus at birth, another for abnormal uterine insertion at 3 years. A third patient initially had HH for hydrometrocolpos leading to ureteral and colonic obstruction at 14 years but she required a completion hysterectomy a year later. Four patients underwent total hysterectomy (TH) at the outset: 2 neonates for a diminutive uterus with extreme disparity in the halves, another for uterine prolapse at 1 month of age, and a fourth for hematometrocolpos at 16 years of age. Six patients reached adulthood without requiring gynecologic intervention; one of these six is now being managed at another institution. Two patients are prepubertal and one was lost to follow-up. The only patient in the series who became pregnant miscarried at 11 weeks gestation. CONCLUSION: Out of 13 post-pubertal patients 6 have retained the entire uterus and another 2 had a hemi-hysterectomy. One patient who became pregnant miscarried at 11 weeks gestation. We believe it is appropriate to avoid ablative genital surgery as far as possible and for these patients to become pregnant after detailed discussion with physicians experienced in the care of 46XX CE patients.

Current diagnosis and management of urachal remnants.

PURPOSE: The purposes of this study are to review our experience with patients who were found to have urachal remnants. We discuss their diagnosis and management and we also evaluate post-operative complications in our cohort of patients with this entity. METHODS: We performed a retrospective review of all patients diagnosed with a urachal remnant (UR) between January 2006 and December 2010. Patient variables included age, gender, presenting symptoms and signs, diagnostic modality, and type of urachal remnant. Operative management, post-operative complications, and non-operative follow-up were abstracted. RESULTS: We identified 103 pediatric patients with either a urachal cyst (n = 38), urachal diverticulum (n = 13), urachal sinus (n = 11), patent urachus (n = 21), or a non-specific atretic urachal remnant (n = 20). Seventy-eight patients had symptomatic URs. Thirty-four symptomatic patients underwent surgical excision of the UR and 44 symptomatic patients were observed. Eighteen URs were incidentally diagnosed in asymptomatic patients, none of whom underwent surgical excision. In 7 patients symptoms could not be determined from the records. No patients with non-specific atretic remnants were operated upon. Nineteen of the observed patients were reimaged subsequently. In fifteen (78.9%) of these patients, the URs had resolved spontaneously. None of the patients who were observed required subsequent excision of the UR. Post-operative complications occurred in 14.7% of patients in whom URs were excised. CONCLUSION: Review of our patients diagnosed with URs indicates there is a subset of patients in whom spontaneous resolution could be expected. Complications are not uncommon after surgical excision.

Severe menorrhagia, unilateral ovarian mass, elevated inhibin levels, and severe hypothyroidism: an unusual presentation of Van Wyk and Grumbach syndrome.

A 10 year old female presented with a 3 week history of persistent vaginal bleeding, lower abdominal pain, and generalized fatigue. Further work-up determined that she had severe autoimmune hypothyroidism, precocious puberty, and a large left cystic ovarian mass, consistent with Van Wyk and Grumbach syndrome. In addition, the patient had elevated inhibin A and B levels, which may be elevated in granulosa cell tumor. The patient was managed with thyroid hormone replacement therapy and careful observation. Complete resolution of the ovarian mass and interval normalization of the inhibin levels were achieved. In females with severe primary hypothyroidism, ovarian masses, and elevated tumor markers, operative resection may be avoided with appropriate thyroid replacement therapy and close observation.

Utility of the computed tomography severity index (Balthazar score) in children with acute pancreatitis.

BACKGROUND: Previous studies in children with acute pancreatitis have demonstrated that clinical scoring systems such as the Ranson, modified Glasgow, and pediatric acute pancreatitis scores are of value in predicting severity of the disease. The aim of this study was to determine the predictive value of the computed tomography severity index (CTSI or Balthazar score) in pediatric patients. METHODS: All children (≤ 18 years) admitted to our institution with acute pancreatitis from 2000 through 2009 were reviewed. Contrast-enhanced computed tomographic (CT) images at presentation were retrospectively reviewed by 2 pediatric radiologists. Peripancreatic fluid and the extent of necrosis were assessed to determine the CTSI. The predictive value of the CTSI was calculated and compared with clinical scoring systems. RESULTS: Of 211 children with acute pancreatitis, 64 underwent contrast-enhanced CT at presentation. The median age was 12.3 years. Etiology of pancreatitis was idiopathic (35.9%), gallstone (17.2%), medication-induced (20.3%), posttransplant (9.4%), traumatic (6.3%), structural (1.6%), and other (9.4%). The sensitivity, specificity, positive predictive value, and negative predictive value of the CTSI (using a cutoff score of 4+) were 81%, 76%, 62%, and 90%, respectively, which compared favorably to the results of the pediatric acute pancreatitis (53%, 72%, 41%, 80%), Ranson (71%, 87%, 67%, 89%), and modified Glasgow (71%, 87%, 67%, 89%) scores. CONCLUSION: The CTSI is superior to clinical scoring systems for identifying children with acute pancreatitis at heightened risk for developing serious complications.

Pyloric duplication with communication to the pancreas in a neonate simulating hypertrophic pyloric stenosis.

An 11-day-old neonate presents with worsening nonbilious emesis and radiographic findings suggestive of pyloric stenosis. Using a laparoscopic approach, a slightly asymmetric and hypertrophied pylorus was visualized. The unusual age of presentation and appearance of the pylorus led to the decision to perform an open exploration for suspicion of a pyloric duplication. An incision of the pyloric muscle exposed and confirmed a duplication cyst. Further exposure revealed accessory pancreatic tissue communicating from the cyst to the main body of the pancreas. The duplication cyst and accessory pancreas were resected, and the patient had an uneventful recovery. Pyloric duplication is a rare cause of congenital extrinsic obstruction in the newborn. Even rarer is a communication of the duplication cyst with the pancreas. Resection of the duplication cyst and the aberrant pancreatic tissue and duct is recommended.

Acute pancreatitis in children: spectrum of disease and predictors of severity.

BACKGROUND: The aim of this study was to describe the spectrum of disease in children with acute pancreatitis and assess predictors of severity. METHODS: Children (≤ 18 years) admitted to a single institution with acute pancreatitis from 2000 to 2009 were included. The accuracy of the Ranson, modified Glasgow, and pediatric acute pancreatitis severity (PAPS) scoring systems for predicting major complications was assessed. RESULTS: The etiology of pancreatitis in these 211 children was idiopathic (31.3%), medication-induced (19.9%), gallstones (11.8%), trauma (7.6%), transplantation (7.6%), structural (5.2%), and hemolytic-uremic syndrome (3.3%). Fifty-six patients (26.5%) developed severe complications. Using the cutoff thresholds in the PAPS scoring system, only admission white blood cell count more than 18,500/µL (odds ratio [OR], 3.1; P = .010), trough calcium less than 8.3 mg/dL (OR, 3.0; P = .019), and blood urea nitrogen rise greater than 5 mg/dL (OR, 4.1; P = .004) were independent predictors of severe outcome in a logistic regression model. The sensitivity (51.8%, 51.8%, 48.2%) and negative predictive value (83.2%, 83.5%, 80.5%) of the Ranson, modified Glasgow, and PAPS scores were, respectively, insufficient to guide clinical decision making. CONCLUSION: Commonly used scoring systems have limited ability to predict disease severity in children and adolescents with acute pancreatitis. Careful and repeated evaluations are essential in managing these patients who may develop major complications without early signs.

Laparoscopic repair of traumatic abdominal wall hernia from handlebar injury.

A 14-year-old boy was seen at an outside hospital after falling over the handlebar of his bicycle and was discharged home. He was subsequently seen in our emergency department with complaints of persistent abdominal pain. A computed tomography scan of the abdomen revealed disruption of the muscles of the upper right abdominal wall containing the hepatic flexure of the colon, with a small amount of intraperitoneal free fluid noted. The patient underwent laparoscopic exploration using 3 ports (2-5 mm and 1-12 mm) and 2 separate stab incisions. The traumatic abdominal wall hernia was repaired with interrupted sutures placed with an ENDO CLOSE (Covidien, Mansfield, MA) device, and a mesenteric defect in the colon was approximated with intracorporeal sutures. The trocar sites were sutured closed. The patient recovered well and was discharged home. Follow-up examination revealed no abdominal wall defect and resolution of his symptoms. Laparoscopic repair of a traumatic abdominal wall defect and exploratory laparoscopy after trauma is feasible and safe in the pediatric patient. It should be considered as an alternative approach with potentially less morbidity than an exploratory laparotomy for handlebar injuries in a stable patient.

Total thyroidectomy for benign disease in the pediatric patient--feasible and safe.

PURPOSE: Total thyroidectomy (TT) is a safe and efficacious treatment of malignant thyroid disease in children. The role of TT in benign thyroid disease is less well-defined. The goal of this study was to compare the safety of TT performed for benign and malignant disease. METHODS: The medical records of 31 patients undergoing TT from January 2000 to June 2007 at a single center were reviewed. The benign cohort totaled 15 patients consisting of 12 with Graves' disease, 2 with hyperthyroidism, and 1 with large and symptomatic multinodular goiter. The malignant cohort totaled 16 patients consisting of 9 with malignant disease, 4 with a nodule and history of cancer or radiation exposure, and 3 with RET proto-oncogene mutations. RESULTS: The most common complication was transient hypocalcemia observed in 7 (46%) of 15 patients with benign disease and 9 (56%) of 16 patients with malignancy (P = .72). Permanent hypocalcemia, defined as need for calcium supplement 6 months postprocedure, was observed in 1 patient with benign disease (6.67%) and 1 patient with malignancy (6.25%; P = 1.0). A single parathyroid gland was reimplanted in 2 patients with malignancy and 2 patients with benign disease (P = 1.0). One case of keloid scar was noted, and no cases of recurrent laryngeal nerve palsy, nerve paralysis, tracheal injury, tracheostomy, or wound infection were encountered in either cohort. There were no cases of relapse hyperthyroidism in the benign cohort. CONCLUSIONS: Similar rates of postoperative complications can be expected with TT for benign thyroid disease as compared to TT for malignant disease. Total thyroidectomy is a safe treatment option for benign thyroid disease in children.

Presentation and management of late-onset duodenomegaly in a teenager with chronic obstruction from malrotation.

Anomalies of intestinal rotation typically present in infancy and early childhood. The diagnosis in older children and adults may be vague and delayed. We discuss a case and management of a patient with late-onset duodenomegaly with chronic obstruction from malrotation.

Previous portal hypertension surgery negatively affects results of mesenteric to left portal vein bypass.

PURPOSE: The mesenteric to left portal vein bypass (MLPVB) has been successfully used to treat extrahepatic portal vein obstruction (EHPVO) in children. We examined the effect of failed prior surgical or radiological procedures intended to treat complications of portal hypertension on the success rate of subsequent MLPVB surgery. METHODS: Sixty-two patients younger than 18 years with EHPVO underwent MLPVB between 1997 and 2006. Children were divided into 3 groups: those with no prior surgery related to portal hypertension, those with prior portosystemic shunts, and those with either splenectomy or mesenteric vascular embolization procedures. The effect of prior procedures on the patency rate of the MLPVB was then examined. RESULTS: Of 62 children, 11 (17.7%) had significant procedures to treat symptoms of portal hypertension: 6 had at least 1 portosystemic shunt attempt, 3 had isolated splenectomy, and 2 had embolization of the splenic artery or coronary and peripancreatic varices. Patients with previous portal hypertension surgery were significantly older and larger than those with no surgery. Patients with no prior interventions had a significantly higher MLPVB patency rate (88.2%, 45/51) than those with no prior interventions (63.6%, 7/11). Prior splenectomy alone was not found to adversely affect MLPVB. Patients with prior embolization procedures or unsuccessful shunts had significantly poorer successful outcomes (0% and 66.7%) than those with no prior interventions (88.2%; P < .005). CONCLUSIONS: The results demonstrate that prior portosystemic shunts or mesenteric embolizations have a deleterious effect on outcome after MLPVB and should be avoided whenever possible. This study suggests that patients with symptomatic EHPVO should undergo MLPVB as a primary intervention rather than as a rescue procedure to optimize MLPVB patency.

Total gastrectomy: options for the restoration of gastrointestinal continuity.

Total gastric resection and the subsequent selection of the optimum procedure for reconstruction of the oesophageal-intestinal tract is a highly debated topic. Many different types of reconstruction have been proposed, but attempts to objectively validate the potential advantages of these techniques of have not produced any definitive conclusions. The main aim of reconstruction should be to maintain the nutritional status and quality of life of the patient, with the additional goal of achieving similar function to normal gut. In this review, we discuss the prospectively randomised studies of gastrointestinal reconstruction that were done between 1987 and 2002.

Superselective mesenteric embolization with microcoils in a porcine model.

PURPOSE: There is a lack of data regarding the degree of ischemic change that may occur in small and large bowel after superselective arterial embolotherapy with platinum microcoils. The purpose of this study was to gain a clearer understanding of the ischemic complications of superselective embolization of peripheral mesenteric arteries with microcoils by gross and histologic examination of postembolectomy bowel specimens at several time intervals in an adult porcine model. METHODS: Two-millimeter platinum microcoils with fibrils were superselectively deployed into six isolated mesenteric branch vessels in each of nine adult pigs. The animals were observed for two days (n = 3), one month (n = 3), or three months (n = 3) and then killed. At necropsy, intestines were examined for gross abnormalities. Sections of bowel containing microcoils were identified under fluoroscopy, resected, and evaluated histologically. RESULTS: A total of 54 microcoils were deployed into the distal arterial mesentery supplying the jejunum (n = 14), ileum (n = 26), and colon (n = 14) of nine adult pigs. Each animal received six microcoils. There were no clinical complications, and all pigs gained weight during their observation periods. Gross examination of the intestines did not reveal any evidence of acute or chronic ischemia. The coils were found in the distal arterial vasculature of the intestine. Histologic examination revealed mild superficial necrosis of villous tips in several samples; however, this finding is likely related to a fixation artifact rather than ischemic injury. There was no other histologic evidence suggestive of ischemic injury. CONCLUSIONS: Gross and histologic findings after superselective arterial embolotherapy demonstrated minimal changes in the bowel, and there were no significant clinical consequences to the animals. We conclude that transcatheter arterial embolotherapy in pigs is safe and may be applicable in the control of massive lower gastrointestinal hemorrhage in humans.