Successful lung donation at the age of 6 weeks: Challenges and lessons learned.

A clinical case of successful procurement and transplantation of bilateral lungs from 6-week-old infant with sepsis secondary to bacterial meningitis is reported. Forty-one-day-old male infant (height 60 cm, weight 4 kg) died of cerebral edema secondary to Escherichia coli meningitis and bacteremia. Preretrieval assessment included the following: arterial gases; pO2 50.4 kPa (378 mm Hg), pCO2 4.9 kPa (37 mm Hg), on FiO2 100%, PEEP 5 cm H2 O. Fiberoptic bronchoscopy showed no secretions nor mucosal inflammation; CXR revealed clear lung fields and pleural spaces. Inspection revealed dense adhesions in pericardial cavity and purulent left hemithorax effusion (urgent Gram-stain came back as negative) but there was no consolidation in the lung. Good compliance of the lungs on inflation/deflation test was observed. The lungs were retrieved using the technique described. The recipient was a 4-month-old infant with alveolar capillary dysplasia and malaligned pulmonary veins. Implantation of the lungs was performed via bilateral thoracosternotomy on cardiopulmonary bypass, cooling to 30°C. Elective support with nitric oxide was used postoperatively. Two years after the transplantation, the recipient doing well with normal lung function. Lung procurement from a 6-week donor with infectious complications and prolonged ventilation is a challenging undertaking but can be successful and should be attempted whenever possible given the paucity of organs available for pediatric recipients.

Medical Management of Children With Congenital/Infantile Cataract Associated With Microphthalmia, Microcornea, or Persistent Fetal Vasculature.

PURPOSE: To report the surgical outcomes of children with cataract associated with microphthalmia, microcornea, or persistent fetal vasculature (MMP) and children with isolated cataract. METHODS: The study included 111 children (cataract associated with MMP: n = 25) who underwent cataract surgery at younger than 16 years. Exclusion criteria were duration of follow-up less than 5 years, intraindividual differences in age at surgery, eye disorders other than MMP, secondary cataract, and syndromal diseases. Main outcome measures were proportion of eyes with glaucoma and best corrected visual acuity (BCVA). Both groups were dichotomized by age at surgery (early intervention group: ≤ 48 days). Descriptive analysis was performed throughout. RESULTS: Median age at surgery was 3.9 months for cataract associated with MMP and 23.3 months for isolated cataract. The median (interquartile range [IQR]) duration of follow-up was 95.9 months (range: 76.0 to 154.5 months). In children with bilateral cataract associated with MMP, the proportion of eyes with final BCVA worse than 0.3 logMAR was similar regardless of age at surgery (early intervention: 80%, later intervention: 78%). In bilateral isolated cataract, the proportions were 56% and 33%, respectively. Children with cataract associated with MMP had a high prevalence of glaucoma (28%). Glaucoma prevalence was lower in the later intervention group. CONCLUSIONS: In the presence of MMP, early cataract surgery is associated with a high risk of post-lensectomy glaucoma, but does not offer the benefit of better visual outcomes. [J Pediatr Ophthalmol Strabismus. 2019;56(1):43-49.].

Infants with Atypical Presentations of Alveolar Capillary Dysplasia with Misalignment of the Pulmonary Veins Who Underwent Bilateral Lung Transplantation.

OBJECTIVE: To describe disease course, histopathology, and outcomes for infants with atypical presentations of alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) who underwent bilateral lung transplantation. STUDY DESIGN: We reviewed clinical history, diagnostic studies, explant histology, genetic sequence results, and post-transplant course for 6 infants with atypical ACDMPV who underwent bilateral lung transplantation at St. Louis Children's Hospital. We compared their histology with infants with classic ACDMPV and compared their outcomes with infants transplanted for other indications. RESULTS: In contrast with neonates with classic ACDPMV who present with severe hypoxemia and refractory pulmonary hypertension within hours of birth, none of the infants with atypical ACDMPV presented with progressive neonatal respiratory failure. Three infants had mild neonatal respiratory distress and received nasal cannula oxygen. Three other infants had no respiratory symptoms at birth and presented with hypoxemia and pulmonary hypertension at 2-3 months of age. Bilateral lung transplantation was performed at 4-20 months of age. Unlike in classic ACDMPV, histopathologic findings were not distributed uniformly and were not diffuse. Three subjects had apparent nonmosaic genetic defects involving FOXF1. Two infants had extrapulmonary anomalies (posterior urethral valves, inguinal hernia). Three transplanted children are alive at 5-16 years of age, similar to outcomes for infants transplanted for other indications. Lung explants from infants with atypical ACDMPV demonstrated diagnostic but nonuniform histopathologic findings. CONCLUSIONS: The 1- and 5-year survival rates for infants with atypical ACDMPV are similar to infants transplanted for other indications. Given the clinical and histopathologic spectra, ACDMPV should be considered in infants with hypoxemia and pulmonary hypertension, even beyond the newborn period.

Unilateral anterior persistent fetal vasculature in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge.

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant genetic disease. It is clinically characterized by four major features; blepharophimosis, ptosis, epicanthus inversus, and telecanthus. We report a case of a 1-year-old female with BPES with unilateral anterior persistent fetal vasculature (PFV). On examination, she was found to have all the clinical features of BPES, along with calcified and partially absorbed cataract with elongated ciliary processes in her left eye. B-scan of left eye showed attached retina with no evidence of posterior PFV. Systemic examination was normal. She underwent cataract surgery with primary posterior capsulotomy with intraocular lens implantation under general anesthesia. Literature search did not reveal any previous reports of unilateral anterior PFV and BPES. The clinical features, other associations, and the difficulties in the surgical management of this condition are discussed.

Pulmonary Interstitial Glycogenosis: A Reversible Underlying Condition Associated With D-Transposition of the Great Arteries and Severe Persistent Pulmonary Hypertension.

Transposition of the great arteries with intact ventricular septum and persistent pulmonary hypertension (TGA-IVS PPHN) is a rare association with a poor prognosis. We report the case of a term newborn with TGA-IVS PPHN successfully managed with perioperative extracorporeal membrane oxygenation (ECMO) and aggressive pulmonary vasodilation therapy that underwent successful arterial switch procedure. A lung biopsy obtained during the surgical procedure showed pulmonary interstitial glycogenosis, a reversible condition. Concerns over left ventricle deconditioning after ECMO could be minimized with appropriate management and monitoring of the ductus arteriosus and appropriate timing of surgery.

Septo-optic dysplasia associated with congenital persistent fetal vasculature, retinal detachment, and gastroschisis.

PURPOSE: The purpose of this study was to report the association of septo-optic dysplasia, persistent fetal vasculature, retinal detachment, and gastroschisis in a preterm neonate. METHODS: This is a case report. RESULTS: A female preterm neonate was found to have septo-optic dysplasia, with optic nerve hypoplasia, tripartite splitting of the vessels at the optic nerve, an ectopic pituitary gland, and absence of the septum pellucidum associated with persistent fetal vasculature, a retinal detachment, and gastroschisis. CONCLUSION: Septo-optic dysplasia may also be associated with other ophthalmic findings and other developmental malformations as the authors report in this case. Follow-up should consist of a multidisciplinary approach with radiologic and endocrinology consultation.

Pulmonary interstitial glycogenosis: an unrecognized etiology of persistent pulmonary hypertension of the newborn in congenital heart disease?

BACKGROUND: Pulmonary interstitial glycogenosis (PIG) arises from a developmental disorder of the pulmonary mesenchyme and presents clinically with reversible neonatal respiratory distress and/or persistent pulmonary hypertension of the newborn (PPHN). OBJECTIVE: We report two cases of PIG in patients with congenital heart disease (CHD) and evidence of PPHN. RESULTS: Both cases demonstrated the hallmark PIG histologic finding of diffuse, uniform interstitial thickening due to the presence of immature interstitial cells containing abundant cytoplasmic glycogen. CONCLUSIONS: We report the second and third patients with PIG associated with CHD. Because histologic examination is required to establish the diagnosis, we speculate that PIG, although rare, may be underrecognized in neonates presenting with PPHN in the setting of CHD.

Nitric oxide in neonatal transposition of the great arteries.

UNLABELLED: Three newborn infants with transposition of the great arteries (TGA) and intact ventricular septum (IVS) developed postnatal persistent pulmonary hypertension of the newborn (PPHN) and were successfully treated with inhaled nitric oxide (iNO). Intervention with balloon atrial septostomy (BAS) was performed in two of the infants before the iNO treatment, but they continued to be severely hypoxic with metabolic acidosis. However, the iNO immediately improved oxygenation and the clinical condition. The third neonate had a moderately large atrial communication and echocardiographic signs of PPHN. He received iNO before BAS with dramatic clinical improvement, which therefore postponed BAS. CONCLUSION: Early diagnosis of PPHN and treatment with iNO may improve final outcome in neonates with TGA and IVS. In the presence of moderately large atrial communication and PPHN, treatment with iNO might be considered before BAS.

Congenital mydriasis combined with aneurysmal dilatation of a persistent ductus arteriosus Botalli: a rare syndrome.

Abstract. A child with congenital mydriasis and aneurismal dilatation of persistent ductus arteriosus is described. We discuss congenital mydriasis as a separate entity and in combination with heart diseases.

Clinical characteristics and follow up of Down syndrome infants without congenital heart disease who presented with persistent pulmonary hypertension of newborn.

We identified seventeen infants with Down syndrome without structural congenital heart disease who presented with persistent pulmonary hypertension in the newborn period. Respiratory distress with or without hypoxia was the presenting feature in these infants. Pulmonary hypertension resolved in the majority of the survivors. Two infants with refractory pulmonary hypertension benefited from patent ductus arteriosus ligation. Autopsies in two infants demonstrated structural lung immaturity. We suggest that infants with Down syndrome are at risk of developing persistent pulmonary hypertension even in the absence of structural heart disease and these infants should be followed up until resolution of the pulmonary hypertension.

Outcomes of surgical (pars plicata and limbal lensectomy, vitrectomy) and non-surgical management of persistent fetal vasculature (PFV): an analysis of 54 eyes.

PURPOSE: To identify clinical characteristics associated with outcomes of treatment for persistent fetal vasculature (PFV based on a modified classification. METHODS: In this noncomparative case series, 54 eyes of 47 consecutive patients with PFV managed from 1981 until 1998 at a tertiary eye hospital were reviewed. Pars plicata lensectomy and vitrectomy was performed in 34 eyes and translimbal lensectomy and vitrectomy in five. Fifteen eyes were managed non-surgically. The following outcomes were measured: 1) "Visual improvement", defined as at least one Zipf's category improvement of best corrected final vision as compared with initial visual acuity, provided that final vision of CSUM (central, steady, unmaintained) equal to 20/100-20/30 was obtained. 2) "Cosmetic acceptability", defined as the absence of the following: small orbit, externally small appearing eye with microcornea and hypotony, eye deviation more than 35 prism diopters without strabismus surgery and corneal opacity. RESULTS: Six eyes were inoperable. In 10 out of 33 operated eyes (30.3%) improvement of vision was obtained including four eyes with an anterior form and six eyes with a combined form of PFV. In univariate analysis, factors such as anterior form of PFV surgery in the combined form and having a normal retina, had a weak association with a higher chance of visual improvement. In multivariate regression analysis the likelihood of unacceptable cosmetic appearance was higher in females than males (OR = 10.5), and in bilateral cases (OR = 7.9). Offspring of consanguineous marriages had more severe forms of PFV. CONCLUSIONS: Although visual potential is limited in PFV some eyes with an anterior or combined form and normal retina achieved improvement of visual acuity after surgery. However, statistically no single factor except sex and bilateral PFV (for cosmetic unacceptability) reliably predicted the results of treatment. Therapy should be individualized based on clinical findings. Genetic studies in offspring of consanguineous marriages with PFV may prove informative.

[Coil embolization of persistent ductus arteriosus]. / Coillukking av persisterende ductus arteriosus.

From September 1994 to January 1996, 57 patients were admitted to Brompton Hospital for catheter closure of persistent ductus arteriosus. Umbrella closure was attempted in 22 patients and coil closure in 35. The duct was closed by a device in 55 of 57 patients (96.4%). Two patients were referred for surgical closure, one after failure of the umbrella closure and one after coil embolization to a branch of the pulmonary artery. Two additional coils embolized to pulmonary artery branches. Both coils were successfully retrieved by a snare, and the procedure then finished successfully. We recommend the use of coils for closure of small ducts, and for residual leak after previous umbrella closure. This is also a promising method for closure of larger ducts.

Orthotopic transplantation for total anomalous pulmonary venous connection associated with complex congenital heart disease.

BACKGROUND: When total anomalous pulmonary venous connection is associated with other complex cardiac malformations, early and late postsurgical morbidity and mortality are excessive. METHODS: In an attempt to modify this outcome, twelve children (4 days to 6.8 years of age) with total anomalous pulmonary venous connection and various congenital cardiac defects were treated with orthotopic heart transplantation. Associated cardiac diagnoses included the following: hypoplastic left heart syndrome (n = 2), unbalanced atrioventricular canal with pulmonary atresia (n = 2), and single ventricle with severe pulmonary stenosis (n = 3) or atresia (n = 5). Two patients had situs inversus, and two had dextrocardia with situs ambiguous. Eight patients had asplenia and one had polysplenia. Palliative pretransplantation procedures in five patients included the following: systemic to pulmonary artery shunt (n = 5), atrioventricular valve annuloplasty (n = 1) and classical Glenn shunt (n = 1). The donor left atrium was anastomosed directly to a common pulmonary venous pool in nine patients; whereas three children required complex reconstruction to baffle the pulmonary venous flow to the donor left atrium. RESULTS: There was one operative death related to an oversized heart and vena caval thrombosis. Follow-up ranged from 16 months to 4.5 years (average 3 years). In two patients (18%) pulmonary venous obstruction developed 3 and 4 months after transplantation. Reoperation to relieve the obstruction was successful in one patient. The second patient underwent three such reoperations and died of sepsis 10 months after orthotopic heart transplantation. CONCLUSION: Orthotopic transplantation is a viable option for children with complex total anomalous pulmonary venous connection that precludes a biventricular repair. Transplantation may improve the dismal prognosis of those children, but it does not eliminate the potential for late pulmonary venous obstruction.

Venovenous compares favorably with venoarterial access for extracorporeal membrane oxygenation in neonatal respiratory failure.

Traditional extracorporeal membrane oxygenation via the venoarterial route requires cannulation and ligation of the internal jugular vein and common carotid artery. Concerns about ligation of the common carotid artery prompted development of a 14F double-lumen internal jugular vein cannula for venovenous oxygenation for neonates with respiratory failure. We retrospectively compared 22 patients supported by venovenous bypass and 20 patients supported with traditional venoarterial bypass. The two groups of patients were selected to be comparable in terms of diagnosis and severity of respiratory insufficiency. The diagnoses in both groups were limited to meconium aspiration syndrome or persistent pulmonary hypertension of the newborn. The average oxygenation indexes in the two groups were similar (46.6 venovenous, 47.2 venoarterial, p = not significant). Venovenous access allowed flow rates of more than 100 ml/kg per minute, which were adequate for gas exchange support. One patient required conversion from venovenous to venoarterial bypass because of hemodynamic instability. The average time of bypass support was 115 hours (range 24 to 338 hours) for venovenous bypass and 134 hours (range 47 to 361 hours) for venoarterial bypass (p < 0.05). The time to extubation after decannulation from extracorporeal membrane oxygenation was 133 hours (range 38 to 720 hours) for venovenous support and 100 hours (range 27 to 192 hours) for venoarterial support (p = not significant). One patient supported with venoarterial bypass had an intracranial hemorrhage. There were no documented neurologic injuries in the patients managed with venovenous bypass. There were no deaths in either group. Venovenous extracorporeal membrane oxygenation through a double-lumen cannula can adequately provide respiratory support for neonates with pulmonary failure and effectively avoids ligation of the common carotid artery.

Reconstruction of the arteria carotis communis in newborn following extracorporeal membrane oxygenation (ECMO).

With the help of ECMO it is possible to save the lives of newborn infants suffering from severe respiratory distress syndrome not responding to conservative treatment. Using Bartlett's classic venous-arterial perfusion technique in ECMO the right arteria carotis communis had to be sacrificed. Thus, despite the life-saving character of this new method, the ligation of the carotid with all its possible complications had often been a major argument against using this therapy. We are now therefore trying to reconstruct the arteria carotis after decannulating the vessel after extracorporeal membrane oxygenation. In our 8 cases so far, post-op examinations showed no obstruction of blood flow in the vessel. No neurological deficiencies were recorded.

Congenital diaphragmatic hernia: impact of prostanoids in the perioperative period.

A prospective study of 10 neonates with congenital diaphragmatic hernia and five controls to determine the importance of prostanoid concentrations perioperatively and the relation with persistent pulmonary hypertension (PPH) is reported. In neonates with congenital diaphragmatic hernia postoperative concentrations of the vasoconstrictor thromboxane B2 rose significantly and were higher during episodes of PPH; this rise may provoke PPH and subsequent right to left shunting.

Pregnancy and delivery after bone marrow transplantation (BMT) for severe aplastic anemia (SAA). A case report.

A 23 years old multiple pretransfused female with severe aplastic anemia (SAA) underwent bone marrow transplantation (BMT). Cyclophosphamide (200 mg/kg) was used for conditioning, donor buffy coat cells were given as rejection prophylaxis. Seven months after BMT regular menses started in the absence of exogenous hormonal manipulation and 21 months after BMT the patient became pregnant. Pregnancy was complicated by mumps in the 14th week. It was terminated at term by cesarean section. The normally developed newborn girl (3,450 gm) presented with a "persistent fetal circulation syndrome". After surgical correction of the patent ductus arteriosus Botalli the girl baby recovered quickly.

[PFC syndrome (persistent fetal circulation). Central cyanosis in a full-term newborn infant without congenital heart defect]. / PFC-Syndrom (Persistenz der fetalen Zirkulation). Zentrale Zyanose eines reifen Neugeborenen ohne angeborene Herzmissbildung.

In a case report the main features of the PFC-syndrome (persistent fetal circulation) are demonstrated: 1. general central cyanosis, 2. pulmonary hypertension, 3. right-to-left shunting via foramen ovale and (or) ductus arteriosus. At postmortem analysis in some of these patients the muscular thickness of the pulmonary vessels was found to be increased: primary PFC-syndrome. Hypoxia, shock, and many pulmonary disturbances of the newborn can produce the clinical picture of "persistent fetal circulation": secondary PFC-syndrome. Under adequate therapy with O2-insufflation, vasodilatory drugs, and if necessary mechanical ventilation the prognosis in the majority of cases is good.