Emphysema as a possible complication of infant respiratory distress syndrome leading to lung transplantation.

Infant respiratory distress syndrome (IRDS) develops among premature infants due to structural immaturity of the lungs and insufficient production of pulmonary surfactant. Nowadays, treatment takes place under conditions of intensive care and includes oxygen therapy, mechanical ventilation, exogenous supplementation of pulmonary surfactant and antenatal corticosteroid therapy. The treatment of IRDS, especially mechanical ventilation, may lead to complications which can contribute to developing a severe dysfunction of the respiratory system. Unavailability of pharmacological treatment of IRDS and development of pulmonary barotrauma due to mechanical ventilation in our patient led to the forming of severe pulmonary interstitial emphysema. In this case report, lung transplantation was performed as an only successful therapeutic option.

Clinical and surgical aspects of congenital lobar over-inflation: a single center retrospective study.

BACKGROUND: Congenital lobar overinflation (CLOI) is one of the most important causes of infantile respiratory distress (RD). We aim to evaluate our experience in CLOI management emphasizing on clinical features, diagnostic modalities, surgery and outcomes. METHODS: This is a retrospective study for all CLOI cases undergoing surgical management at Qena University Hospital. Demographic data, clinical data, radiographic findings, surgery and postoperative follow-up were reviewed. RESULTS: A total of 37 neonates and infants with CLOI were presented to our center between January 2015 and January 2019; their mean age was 111.43 ± 65.19 days and 22 were males. All cases presented with RD; and cyanosis in 19 cases. 15 cases presented with recurrent pneumonia and fever. Diminished breath sounds on the affected side and wheezes were the main clinical findings in 30 and 22 cases respectively. On CXR, emphysema was detected in all cases. A confirmatory CT chest was done for all cases. Left upper lobe was affected in 23 cases, right middle lobe in 7 and right upper lobe in 7 cases. Lobectomy was done in thirty-one cases; their mean age at surgery was 147.58 ± 81.49 days and 19 were males. Postoperative complications were noted in 5 cases and postoperative ventilation was required for 2 of them. No morbidity or mortality was reported. The follow-up duration ranged from 3 months to 1 year and all patients were doing well except one case that lost follow up after 3 months. CONCLUSION: CLOI is a rare bronchopulmonary malformation that requires a high index of clinical suspicion, especially in persistent and recurrent infantile RD. CT chest is the most useful diagnostic modality. Early management of CLOI improves outcome and avoid life-threatening complications. Surgical management is the treatment of choice in our center without recorded mortality.

Clostridium difficile causante de síndrome de distrés respiratorio agudo tras pancreatectomía total / Acute respiratory distress syndrome due to clostridium difficile after total pancreatectomy

La diarrea nosocomial, que es la adquirida en el ámbito hospitalario, suele ser producida por Clostridium difficile. Sin embargo, en raras ocasiones puede ocasionar un síndrome de distrés respiratorio. Por ello, el diagnóstico de dicha patología es difícil si no se sospecha. El tratamiento se basa en el uso de antibiótico vía oral. Se expone el caso de una paciente de 66 años con dicha patología tras la realización de pancreatectomía total.

Nosocomial (hospital-acquired) diarrhea is usually caused by Clostridium difficile. On rare occasions it can cause acute respiratory distress syndrome (ARDS). Therefore, this condition should be suspected in order to make a diagnosis. Treatment is based on oral antibiotics. We report the case of a 66-year-old female patient with ARDS secondary to Clostridium difficile colitis after total pancreatectomy.

Fetal lung interstitial tumor: Prenatal presentation of a rare fetal malignancy.

Fetal lung interstitial tumor (FLIT) is a rare fetal malignancy that is typically diagnosed in the postnatal period, or, if recognized prenatally can mimic a benign lesion such as congenital pulmonary airway malformation. We present the earliest case of a FLIT tumor described by ultrasound and MRI at 26 weeks of gestation. Our case highlights features suggestive of FLIT including presentation later in gestation in combination with findings on fetal MRI such as a solid appearance with radiating curved bands of high signal within and along the periphery of the lesion (not as intensely high signal as the typical CPAM), possibly detailing a radiographic signature for these tumors. The role of betamethasone for these tumors is not known.

Persistent respiratory distress in a neonate: a diagnostic dilemma.

We present a 17-day-old term, female baby who was referred to our centre for persistent respiratory distress. She was managed for pneumonia and pneumothorax at the primary care centre. On detailed clinical examination at admission, a possibility of congenital lobar emphysema (CLE) was considered. A CT chest was performed, and diagnosis of CLE was confirmed. The infant was managed with lobectomy. The respiratory distress settled within a few hours after the surgery, and the baby was discharged in stable condition.

Atypical pulmonary sequestration causing respiratory distress in a 2-month-old male infant.

Pulmonary sequestrations are non-functional masses of lung tissue lacking normal communication with the tracheobronchial tree and systemic-circulation-derived blood supply. The disconnection between the normal airway and pulmonary arterial supply prevents pulmonary sequestrations from participating in respiration while their aberrant circulation increases the potential for hemodynamic imbalance. Extralobar sequestrations are prenatally diagnosed, congenital lesions that may become symptomatic before or after birth, whereas intralobar sequestrations are usually identified during adulthood. This report describes a 2-month-old boy with respiratory distress caused by an anomaly characterized by a systemic artery feeding a large segment of the left lower lobe lacking normal ventilation due to absent bronchial connections. This segment that was surrounded by its own visceral pleura, and separated from the normal lower lobe by a fissure, drained through a large vessel into the left inferior pulmonary vein, causing left-to-left shunting and high output failure. Symptoms disappeared immediately following thoracoscopic division of the aberrant vessels and excision of only the sequestered segment. This case accentuates the hemodynamic imbalance set off by a left-to-left shunt across an atypical sequestration that although intralobar was separated by an accessory fissure from the normal parenchyma and also the benefits of video-assisted thoracoscopic surgery.

Congenital lobar emphysema.

Congenital lobar emphysema is a rare variety of congenital malformation of lung characterized by over distension of a lobe of the lung due to partial obstruction of the bronchus. It is one of the rare causes of infantile respiratory distress requiring surgical resection of affected lobe. We are reporting the case of an 8-week-old baby who presented with respiratory distress related to CLE affecting the left upper lobe. Lobectomy was performed under general anaesthesia followed by an uneventful recovery.

Genotype-phenotype correlations for infants and children with ABCA3 deficiency.

RATIONALE: Recessive mutations in the ATP-binding cassette transporter A3 (ABCA3) cause lethal neonatal respiratory failure and childhood interstitial lung disease. Most ABCA3 mutations are private. OBJECTIVES: To determine genotype-phenotype correlations for recessive ABCA3 mutations. METHODS: We reviewed all published and unpublished ABCA3 sequence and phenotype data from our prospective genetic studies of symptomatic infants and children at Washington and Johns Hopkins Universities. Mutations were classified based on their predicted disruption of protein function: frameshift and nonsense mutations were classified as "null," whereas missense, predicted splice site mutations, and insertion/deletions were classified as "other." We compared age of presentation and outcomes for the three genotypes: null/null, null/other, and other/other. MEASUREMENTS AND MAIN RESULTS: We identified 185 infants and children with homozygous or compound heterozygous ABCA3 mutations and lung disease. All of the null/null infants presented with respiratory failure at birth compared with 75% of infants with null/other or other/other genotypes (P = 0.00011). By 1 year of age, all of the null/null infants had died or undergone lung transplantation compared with 62% of the null/other and other/other children (P < 0.0001). CONCLUSIONS: Genotype-phenotype correlations exist for homozygous or compound heterozygous mutations in ABCA3. Frameshift or nonsense ABCA3 mutations are predictive of neonatal presentation and poor outcome, whereas missense, splice site, and insertion/deletions are less reliably associated with age of presentation and prognosis. Counseling and clinical decision making should acknowledge these correlations.

Urgent bilateral endoscopic marsupialization for respiratory distress due to bilateral dacryocystitis in a newborn.

We describe an infant with respiratory distress due to bilateral dacryocystoceles and dacryocystitis who was successfully treated with urgent bilateral endoscopic marsupialization. A male infant was brought to our outpatient department 7 days after birth, with red, acutely inflamed swellings near the medial canthal area of both eyes. From birth, there had been bluish swelling near the medial canthal area, and redness and swellings developed within 3 days. On physical examination, the child was afebrile but showed respiratory distress with coarse breathing sound. That day, the infant was admitted and treated with intravenous cefotaxime 150 mg. After withholding oral intake for appropriate preoperative fasting, urgent bilateral probing with endoscopy was done. On endoscopy, huge bilateral congenital dacryocystoceles were found. Because of its huge size, the inferior surface of the cyst was touching the nasal floor, which made probe unable to perforate the wall of dacryocystocele. Therefore, an endoscopy-assisted marsupialization of dacryocystoceles and bicanalicular silicone intubation were performed. Both swellings and erythema subsided within 48 hours postoperatively, and the patient was discharged after 72 hours from treatment.

Fast and early mandibular osteogenetic distraction in a 24-day-old female newborn with Larsen syndrome.

BACKGROUND: Larsen syndrome (LS) is a rare bone dysplasia characterized by multiple dislocations affecting large and small joints, progressive scoliosis, accessory and early ossifying carpal/tarsal bones, and characteristic craniofacial features. CASE PRESENTATION: A newborn with a clinical diagnosis of LS is presented. Shortly after birth, she had respiratory distress due to retrognathia. Such a life-threatening complication was resolved by mandibular distraction osteogenesis at 24 days of age. CONCLUSION: Fast and early mandibular osteogenetic distraction could represent an optimal tool to avoid tracheostomy and to improve oral feeding in patients with rare conditions, such as LS.

Image-guided transoral resection of recurrent parapharyngeal space glial heterotopia.

Intranasal glial heterotopia is an uncommon congenital nasal lesion of neuroectoderm origin. Involvement of the parapharyngeal space is extremely rare. We present a case report of a newborn with life-threatening respiratory distress and feeding difficulty caused by a nasal glial heterotopia in a rare location involving the nasopharynx and parapharyngeal space. Surgical treatment was done in a staged fashion, involving image guidance for recurrence. Other diagnostic and treatment options are reviewed in the light of current literature.

Hairy polyp can be lethal even when small in size.

A case of sudden cardiopulmonary arrest in a 3-month-old girl is presented. The patient had barely recovered from hypoxic encephalopathy when she presented with repeated respiratory distress. Computed tomography and endoscopic analysis revealed a shiny polyp in the lateral wall of the nasopharynx, and this polyp was suspected to be the main cause of respiratory distress. After referral to our hospital, surgical removal was performed, and the histopathological diagnosis was hairy polyp. Hairy polyp is a rare congenital benign tumor that sometimes induces respiratory distress. This polyp can potentially induce a life-threatening event. In a systematic review of 40 reported cases, polyps of ≤ 3.0 cm in diameter have a higher risk of respiratory distress than do those >3.0 cm in diameter (P = 0.01). Small hairy polyps may be lethal because of delayed diagnosis. To locate small hairy polyps, physicians should not hesitate to perform further examination because there is the possibility of oversight with only physical examination.

Comentarios al artículo «Airtraq como dispositivo de primera elección para un lactante con vía aérea difícil conocida» / Comments on the article "Airtraq as a first-choice device for an infant with known difficult airway"

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Neonatal airway lesions: our experience and a review of the literature.

OBJECTIVES: This paper reports on two rare cases of neonatal airway lesions with differing aetiology that were successfully managed by surgery, and provides a review of the literature on neonatal stridor and airway lesions. CASE REPORTS: In the first case report, a newborn presented with a nasopharyngeal teratoma. In the second case report, a newborn presented with a congenital laryngeal saccular cyst. Difficulties in the diagnosis of these lesions, and surgical and anaesthetic challenges in their management are discussed. CONCLUSION: Every case of neonatal airway distress must be evaluated and the cause of stridor needs to be established. It is important that rare lesions such as teratomas and laryngeal cysts are not overlooked; a high index of suspicion for these congenital anomalies is necessary. These airway lesions should be managed in an institutional setting by a multidisciplinary team.

An interesting finding in a congenital cystic adenomatoid malformation.

Here we describe a case of antenatally diagnosed congenital cystic adenomatoid malformation. Due to increasing cyst size and fetal hydrops an antenatal drain was inserted. The lesion was excised surgically soon after birth due to significant respiratory distress. During histological assessment an area of squamous metaplasia was found within the cyst wall. This developed as a result of the drain placement. At 4-year follow-up the child remains well and has experienced no long-term respiratory sequalae.

Not all infantile respiratory distress in winter is bronchiolitis: congenital lobar emphysema.

The authors report the case of a 4-week-old male infant presented during the winter period with respiratory distress. He had a 3 day history of cough and coryza, and a 2 day history of breathlessness and reduced feeding. He had evidence of tachypnoea, subcostal recession and hypoxia on examination. An initial diagnosis of bronchiolitis was made. The authors explore how the correct diagnosis of congenital lobar emphysema (CLE) was reached, highlighting key clinical signs and investigations. He had evidence of a hyperinflated right middle lobe, with collapse of right upper and lower lobes and left upper lobe with associated mediastinal shift on chest x-ray (CXR) and CT scan. He was referred to the regional Paediatric Cardiothoracic Centre where right middle lobectomy was performed with complete resolution of his respiratory distress and re-expansion of the compressed lobes on CXR. Current literature concerning CLE is reviewed.

Acute respiratory distress secondary to severe compression of the left main bronchus by the ductus arteriosus.

Two newborn infants presented with acute respiratory distress. In both cases, the left lung was opaque, hyperinflated, and associated with a rightward shift of the mediastinum. A diagnosis of retained fetal fluid secondary to vascular compression of the left bronchus by the ductus arteriosus was made by combining various imaging methods including chest radiograph, computed tomography (CT), and echocardiography. Although the initial chest radiographs were similar, the mechanisms of obstruction were different. The imaging emphasizes the importance of CT angiography to understanding the three-dimensional relationships resulting in bronchial compression.