Assessment of airway abnormalities in patients with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals.

BACKGROUND: Children with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals (TOF/MAPCAs) are at risk for post-operative respiratory complications after undergoing unifocalisation surgery. Thus, we assessed and further defined the incidence of airway abnormalities in our series of over 500 children with TOF/MAPCAs as determined by direct laryngoscopy, chest computed tomography (CT), and/or bronchoscopy. METHODS: The medical records of all patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery from March, 2002 to June, 2018 were reviewed. Anaesthesia records, peri-operative bronchoscopy, and/or chest CT reports were reviewed to assess for diagnoses of abnormal or difficult airway. Associations between chromosomal anomalies and airway abnormalities - difficult anaesthetic airway, bronchoscopy, and/or CT findings - were defined. RESULTS: Of the 564 patients with TOF/MAPCAs who underwent unifocalisation or pulmonary artery reconstruction surgery at our institution, 211 (37%) had a documented chromosome 22q11 microdeletion and 28 (5%) had a difficult airway/intubation reported at the time of surgery. Chest CT and/or peri-operative bronchoscopy were performed in 234 (41%) of these patients. Abnormalities related to malacia or compression were common. In total 35 patients had both CT and bronchoscopy within 3 months of each other, with concordant findings in 32 (91%) and partially concordant findings in the other 3. CONCLUSION: This is the largest series of detailed airway findings (direct laryngoscopy, CT, and bronchoscopy) in TOF/MAPCAS patients. Although these findings are specific to an at-risk population for airway abnormalities, they support the utility of CT and /or bronchoscopy in detecting airway abnormalities in patients with TOF/MAPCAs.

Midterm Echocardiographic Assessment of Right Ventricular Function After Midline Unifocalization.

BACKGROUND: Patients with an open ventricular septal defect (VSD) after repair of pulmonary atresia (PA), VSD, and major aortopulmonary collaterals (MAPCAs) are the most vulnerable subgroup. We analyzed the impact of concomitant versus delayed VSD closure on survival and intermediate-term right ventricular (RV) function. METHODS: Between October 1996 and February 2017, 96 patients underwent a pulmonary flow study-aided repair of PA/VSD/MAPCAs. For patients who underwent either concomitant or delayed intracardiac repair, echocardiographic RV systolic function was retrospectively calculated to assess (1) RV fractional area change (RVFAC) and (2) two-dimensional RV longitudinal strain (RVLS) of the free wall of the right ventricle. QLAB cardiac analysis software version 10.3 (Philips Medical Systems, Andover, MA) was used for analysis. RESULTS: A total of 64 patients underwent concomitant VSD closure at the time of unifocalization, and 16 patients underwent delayed VSD closure at a median of 2.3 years (range: 3 days to 7.4 years). At a median follow-up of 8.1 years (range: 0.1 to 19.5 years) for the concomitant repair group versus 7.4 years (range: 0.01 to 15.3 years) for the delayed repair group, no differences in RVFAC and RVLS were observed (RVFAC: 41.0% ± 6.2% versus 41.2% ± 7.6%, p = 0.91; RVLS: -18.7 ± 4.3 versus -18.9 ± 4.0, p = 0.87). CONCLUSIONS: Patients (83%) with PA/VSD/MAPCAs underwent complete repair at intermediate-term follow-up with preserved RV function. Delayed VSD closure was accomplished in 50% of the patients initially deemed unsuitable for repair. Delayed VSD closure did not affect survival and did not portend impaired RV systolic function.

Primary Repair in Patients With Unilateral Complete Cleft of Lip and Primary Palate: Assessment of Outcomes.

OBJECTIVE: Asymmetry of median facial structures is a major concern for patients with unilateral cleft lip and palate, and the principal goal of its treatment is to restore symmetry. Unilateral complete cleft of lip and primary palate (UCCLPP) is an uncommon subgroup and rarely reported. Patients with UCCLPP have protruding and deviating premaxilla, rendering a primary repair difficult. This study evaluated consecutive patients with UCCLPP and presented their treatment outcome. METHODS: We assessed 36 patients with UCCLPP and collected clinical information. Surgical repair was performed at 3 months of age by using a modified rotation-advancement method and primary nasal reconstruction. A single surgeon performed all surgical procedures. A postoperative nasal stent was used for 6 months. Follow-up standardized photographs were collected. Landmarks were identified, and nose and lip dimensions measured. The ratios of the corresponding parameters (cleft vs noncleft side) were obtained for evaluating nose and lip symmetry. RESULTS: No surgical complication was noted in any patient. The overall lip and nose outcome after the primary repair were adequate, and all ratios were close to 1. The nostril width was slightly wider on the cleft side, but the alar width and height were acceptable. The lip heights were balanced between the 2 sides. Patients who received presurgical nasoalveolar molding did not show more favorable lip and nose dimensions, except for the medial philtral height. CONCLUSION: Even in the presence of protruding and deviating premaxilla, our patients with UCCLPP obtained adequate lip and nose outcome after undergoing primary lip repair and nasal reconstruction.

A standardised investigative strategy prior to revisional oesophageal surgery in children: High incidence of unexpected findings.

BACKGROUND/PURPOSE: Revisional oesophageal reconstructive surgery carries uncommon and unusual risks related to previous surgery. To provide maximum anatomical detail and facilitate successful outcome, we report a standardised pre-operative investigative strategy for all such patients. METHODS: Prospective 8-month cohort study following the introduction of this strategy. All patients underwent high resolution thoracic contrast CT scan and micro-laryngo-bronchoscopy by a paediatric ENT surgeon in addition to upper gastrointestinal contrast study, oesophagoscopy, and echocardiogram. RESULTS: Seven children (median age 5.6 months [range 2.2-60]) completed the pathway. Four were referred with recurrence of a previously divided tracheo-oesophageal fistula (3 congenital, 1 acquired) and 3 (all with oesophagostomy) for oesophageal replacement for congenital isolated oesophageal atresia (OA, n=1) and failed repair of OA with distal TOF with wide gap (n=2). Overall, unanticipated findings were demonstrated in 6/7 children and comprised severe tracheomalacia and right main bronchus stenosis requiring aortopexy (n=1), vocal cord palsy (n=2), extensive mediastinal rotation (n=1), proximal tracheal diverticulum (n=1), severe subglottic stenosis requiring airway reconstruction (n=1), proximal tracheal diverticulum (n=1), right sided aortic arch (n=1) and left sided aortic arch (previously reported to be right sided, n=1). CONCLUSIONS: This standardised approach for this complex group of patients reveals a high incidence of unexpected anatomical and functional anomalies with significant surgical and possible medico-legal implications. We recommend these investigations during the pre-operative work-up prior to all revisional oesophageal surgery.

Volumetric and functional assessment using cardiac magnetic resonance imaging in young children exposed to acute pulmonary regurgitation: is pulmonary regurgitation just a long-term matter?

BACKGROUND: The early effect of pulmonary regurgitation (PR) on both ventricular volume and function has not been well established in children. We evaluated the early effect of PR on both ventricular volume and function in young children. METHODS: Among patients who underwent total repair of pulmonary atresia with ventricular septal defect between January 2007 and December 2008, cardiac magnetic resonance imaging (CMRI) was performed in 12 patients at a median interval of 15.6 months (6-22 months). Valveless right ventricular outflow tract (RVOT) reconstruction was performed in five patients (valveless group) and RVOT reconstruction using valved conduit in seven patients (valve group). Age and weight at operation, and the interval between the operation and CMRI were not different between the groups. RESULTS: We observed a higher pulmonary regurgitant fraction (p = 0.003), a higher right ventricular end-diastolic volume index (RVEDVI) (p = 0.003), a higher right ventricular end-systolic volume index (p = 0.003), a higher left ventricular end-diastolic volume index (p = 0.010), a higher left ventricular end-systolic volume index (p = 0.018), and a lower left ventricular ejection fraction (LVEF; p = 0.048) in the valveless group. Right ventricular ejection fraction (RVEF) was not different between two groups. The RVEDVI was negatively correlated with RVEF (rho = -0.601, p = 0.039) and LVEF (rho = -0.580, p = 0.048). CONCLUSIONS: Both ventricular volumes increased and left ventricular function was compromised, but right ventricular function was preserved early after the exposure to PR in children. Right ventricular volume was associated with both ventricular functions.

[Congenital abdominal wall defects--an analysis of prevalence and operative management by means of gastroschisis and omphalocele]. / Ventrale Bauchwanddefekte--Darstellung der Entwicklung in Prävalenz und operativem Vorgehen anhand von Gastroschisis und Omphalozele.

The management of congenital abdominal wall defects is one of the main characteristics of quality for a department of paediatric surgery. The results of treatment in the early years were the reason for a continuous improvement of procedures, operation strategies and the kinds of -material that had been used. During the last years there has been a great discussion about the presumed increase in the incidence of gastroschisis and the preterm delivery of such cases. On the basis of our own first results (2006-2009) and details from the literature, we survey these two topics.

Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome.

PURPOSE: We evaluated the accuracy of magnetic resonance imaging (MRI) in young women with primary amenorrhoea with suspected Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome (congenital absence of both vagina and uterus and presence of normal ovaries). MATERIALS AND METHODS: Fifty-eight women (age range 14-30 years, mean 20.9) with primary amenorrhea were studied with MRI performed with a 1.0-T superconducting magnet (Philips NT Intera). All patients were examined in the supine position using a phased-array coil (four channels). Turbo spin-echo T2-weighted images were acquired in the sagittal, axial and coronal planes with the following parameters: TR 4,750-6,686, TE 100-120, FOV 350-375, 4- to 5-mm sections with a 0.4- to 0.5-mm intersection gap and NSA 6. T1-weighted images were acquired in the axial and coronal planes (TR 470, TE 15, FOV 350, 4-mm sections with a 0.6-mm intersection gap, NSA 3). Two experienced radiologists evaluated all the examinations in consensus to assess the presence, position and morphology of vagina, uterus, ovaries and kidneys and any pelvic abnormalities. MRI results were judged on the basis of laparoscopic findings in 41 patients. RESULTS: MRKH syndrome was confirmed in 56 patients with 100% sensitivity and specificity. MRI identified bilateral Müllerian buds in 34/56 (61%) and unilateral in 10/56 (18%) patients. MRI sensitivity was 81.42%, and there was good agreement with laparoscopy (k=0.55) and full agreement in the identification of cavitation between MRI and intraoperative sonography. Both ovaries were visualised in 54 patients, with regular morphology in 46 (82.1%), polycystic in 10 (17.8%), pelvic in 47 (83.6%) and extrapelvic in eight (14.5%). We found associated abnormalities of the upper urinary tract in six patients (solitary kidney in four and ptosis in two). CONCLUSIONS: MRI is a useful diagnostic tool in the preoperative evaluation of MRKH syndrome and is less expensive and invasive than laparoscopy. Strong cooperation between radiologists and surgeons is highly recommended.

Perspectives on plastic surgery and global health.

Of the many factors affecting the health of the human race, those amenable to correction by plastic surgical intervention comprise a significant number. The interface between the global health community and the plastic surgery community historically has been quite diminutive, but this is changing with globalization. This overview provides a primer of global health for the plastic surgeon, and a discussion of the global burden of disease as it relates to plastic surgery. The article then briefly discusses the disparity between the global plastic surgery needs and the supply of expertise, and the difficulties presented by policy, finances, and implied societal preferences for care.

Approaches to neurodevelopmental assessment in congenital diaphragmatic hernia survivors.

BACKGROUND: Infants with congenital diaphragmatic hernia require complex surgical care and may have neurodevelopmental morbidity. We examined the performance of reports of motor functioning in 25 congenital diaphragmatic hernia survivors using the parent-completed Developmental Profile-II and a clinical evaluation by a neurodevelopmental pediatrician (MD) measured against the Bayley motor scale. METHODS: Bayley motor scores were dichotomized as normal or abnormal. Sensitivity and specificity were calculated for each test. RESULTS: The median age at assessment was 25 months. Bayley motor scores were abnormal in 77% of infants tested (10/13). The MD examinations detected motor problems in 92% (12/13). Sensitivity and specificity of the MD examination were 1.0 and 0.33, respectively. Developmental Profile-II physical scores were abnormal in 15% (2/13); sensitivity and specificity were 0.2 and 1.0, respectively. CONCLUSIONS: The high rate of abnormal motor findings in this study supports the need for ongoing screening and evaluation. The sensitivity of MD examinations was excellent, but hypotonia findings were not universally corroborated by the Bayley. Although specificity of parent-reported motor findings was high, parents underreported abnormal motor findings. Parental reports of neurodevelopmental problems should be heeded, and physicians should perform screening motor examinations. Bayley assessments may be warranted to determine the functional implications of observed abnormalities.

Controversies, genetics, diagnostic assessment, and outcomes relating to the heterotaxy syndrome.

How best to analyse and describe the features of the situation commonly known as "visceral heterotaxy" remains controversial. Much of the disagreement devolves on how to deal with the concept of isomerism. In the opinion of some, the concept of bilateral right-sidedness and bilateral left-sidedness, while useful in helping to remember which abnormalities are likely to occur in asplenia or polysplenia, should not be granted the status of a specific "situs", since there are numerous examples of exceptions to these patterns. On the other hand, those who favour the concept of isomerism point out that, when describing only the heart, and taking the structure of the atrial appendages as the starting point for analysis, basing this on the extent of the pectinate muscles relative to the atrioventricular junctions, then the only possible arrangements for the appendages are the usual one, its mirror-image, and the two situations in which appendages of comparable morphology are found on both sides of the heart, these being the arrangements of right or left isomerism. It is certainly the case that the arrangement of the organs is not always in harmony with the arrangement of the atrial appendages, but those circumstances, in which there is disharmony, can readily be described by paying specific attention to each series of organs. On this basis, in this review, we describe the approach to heterotaxy, and isomerism of the atrial appendages, in terms of the genetic background, the diagnosis, and outcomes after cardiac surgery. Attention is given to the various diagnostic modalities, including fetal and postnatal echocardiography, recent tomographic and magnetic resonance imaging techniques, and the time-honoured approach using angiography.

Pulmonary function assessment in an infant with Barnes syndrome: proactive evaluation for surgical intervention.

Our aim for this study was to report pulmonary mechanics in a neonate with a severe case of Barnes syndrome, a rare form of thoracolaryngopelvic dysplasia, and to use these data to guide ventilatory support and serve as a presurgical screening tool. A comprehensive pulmonary function evaluation was performed on a 36-day-old patient with Barnes syndrome who was being mechanically ventilated because of severe pulmonary distress secondary to thoracic dystrophy. The measurements consisted of respiratory volumes including functional residual capacity, ventilatory mechanics including compliance and resistance, and thoracoabdominal synchrony. Chest wall compliance was 64% below normal, and the thoracoabdominal motion was indicative of predominantly abdominal displacement during inspiratory breaths. The lungs were functioning at a low functional residual capacity, resulting in low lung compliance and increased pulmonary resistance. As a result of the evaluation, the patient was recommended for lateral thoracic expansion surgery and the ventilatory management was adjusted to focus on end-distending pressure support.

[Aortopulmonary window: clinical assessment and surgical results]. / Ventana aortopulmonar: valoración clínica y resultados quirúrgicos.

INTRODUCTION AND OBJECTIVES: Aortopulmonary septal defect is an uncommon congenital cardiac anomaly. To date, approximately 300 cases have been reported. We present our experience, emphasizing the importance of early correction to avoid irreversible pulmonary hypertension. PATIENTS AND METHOD: Between 1979 and 2000, seven patients underwent surgical repair of this heart defect in our hospital. Two had type I (proximal), 4 had type II (distal) and 1 had type III (complete). Complex associated cardiac anomalies were present in 4 cases: type A interruption of the aortic arch in 2 cases, hypoplastic aortic arch in 1 and transposition of great arteries with ventricular septal defect in 1. Four cases (57%) were diagnosed by echocardiography. In all patients diagnoses were confirmed by cardiac catheterization. Patient records were reviewed retrospectively, with special attention to clinical, echocardiographic and hemodynamic data as well as surgical characteristics. RESULTS: No intraoperative deaths occurred. The patient with associated transposition of great arteries died 22 days after surgery as a result of severe pulmonary hypertension. The remaining patients are asymptomatic without treatment after a mean follow-up period of 69 months. CONCLUSIONS: Even though aortopulmonary septal defect is a rare anomaly, it should be considered whenever the course of complex congenital heart disease includes early cardiac failure and pulmonary hypertension. Repair before 6 months will prevent irreversible damage of pulmonary vessels.

[The pathology of the peritoneo-vaginal process in the young males: clinical and therapeutical aspects in 160 cases]. / La pathologie du canal péritonéo-vaginal chez le garçon: aspects cliniques et thérapeutiques à propos de 160 cas.

The objective of this work was to describe the clinical and therapeutical aspects of pathology of the peritoneo-vaginal process. We have performed a retrospective study including 160 patients operated between January 1990 up to December 1996. Mean age at diagnosis was 8 years, ranged from 1 month to 13 years old. All patients were male. The abnormality was located in the right side in 60% of cases and was bilateral in 6.7% of cases. The main clinical features were scrotal mass (81%) and scrotal pain (13.46%). The diagnosis was made at birth only in 20% of cases. A maldescended testis was associated in 7.5% of cases. A groin incision have been used in 91.25% of patients. The average hospital stay after surgery was 1 day. Thus, the pathology of the peritoneo-vaginal process is common and apparently banal. Need for treatment through a groin incision owing to the possibility of associated maldescended testis.

The importance of detailed diagnostic assessment in a case of partial situs inversus.

Partial situs inversus is not necessarily symptomatic; however, recent experience with a child affected by this condition and polysplenia led us to reconsider the approach to such cases. The collection of as much pre operative information as possible is desirable in order to delineate a correct surgical approach. In particular, the presence of gastrointestinal anomalies, e.g., duodenal dilatation, should be assessed individually, since they are virtually always pathological and not necessarily related to the situs inversus. At laparotomy all of the intra-abdominal contents should be carefully assessed.

Neonatal intensive care as a locus for ethical decisions.

Children born with congenital anomalies are usually cared for in the neonatal intensive care unit (NICU). Although most of these children will have conditions amenable to surgical correction, many will have serious underlying disorders that will alter the approach to management of the secondary birth defects and the child. The decision as to whether to treat or withhold treatment from a child with congenital anomalies lies with the parents or legal guardians with guidance and counseling from the health and medical care givers. The ability to make a rational decision about whether or not to offer treatment depends upon the ability to make a correct diagnosis, understand the implications of the diagnosis, and to be able to communicate this information to the patient's family. This responsibility, in many centers, falls upon the shoulders of the clinical geneticist. There is a critical need for clear communication among care givers who comprise the management team and between the management team and the family. Major obstacles that can arise include lack of communication among care givers and the reluctance of family members to make decisions regarding withholding treatment despite a diagnosis of a condition with a grave prognosis. As our technology improves, our ability to save the lives of the smallest and sickest infants will increase; the greatest dilemma, however, which we will face will not be whether we can treat, but rather, whether we should treat certain conditions and how these decisions will be made.

Who decides? Patients, parents, or gatekeepers: pediatric decisions in the craniofacial setting.

Special ethical issues arise for the craniofacial team dealing with pediatric patients, which include competency, surrogacy, and the "best interests" standard. Medical decisions for children are made by surrogates, usually parents, who must use the "best interests" standard. The team's primary responsibility is to the child, not the parents. Children should participate as abilities allow, especially for elective procedures. Increasingly, cost considerations also influence medical decisions. The craniofacial team is often a de factor gatekeeper. Ethically responsible team behavior includes: weighing risks and benefits of proposed interventions; promoting discussion with families and patients to identify "best interests;" monitoring outcomes; and advocacy for craniofacial patients individually and at a policy level. Care guidelines and definitions of basic levels of care should be developed to assist teams with decision-making and advocacy efforts. Ethical analysis is part of both good patient care and good policy formation, and should be a part of regular team deliberations.

Factors influencing choice of procedure in transposition of the great arteries: a decision analysis approach.

Clinicians have a difficult choice between the arterial switch (Jatene et al.) and the atrial baffle operation (Mustard or Senning) for transposition of the great arteries. The surgical decision is essentially a determination of whether the long-term course after the arterial switch procedure (thought to be more favorable than after the atrial baffle procedure) plus elimination of presurgical attrition with the atrial baffle is substantial enough to offset the higher early mortality rate associated with the arterial switch. Decision analysis was undertaken to answer the following questions: 1) are there clinical circumstances under which published surgical results support a clear procedure of choice in transposition of the great arteries? and 2) what short- and long-term outcomes must be anticipated for the arterial switch to compare favorably with the published experience with the atrial baffle? A decision tree was constructed accounting for the major variables influencing mortality and morbidity in the surgical management of transposition of the great arteries. Presuming that the arterial switch has moderate advantages over the atrial baffle in terms of late morbidity and mortality for simple transposition of the great arteries at an institution with average results from the atrial baffle, the early mortality rate of the arterial switch must be less than 24% to recommend the switch operation. Assuming extremely good surgical results from the atrial baffle, an early mortality rate of the arterial switch less than 20% is required to recommend the switch procedure over the atrial baffle operation.(ABSTRACT TRUNCATED AT 250 WORDS)