Dataset Information

Pneumonectomy in an elderly patient with congenital unilateral lung hypoplasia and recurrent Aspergillus: a case report.

ABSTRACT:

Background

Congenital lung disorders encompass a spectrum of several conditions, one of which is lung hypoplasia. While hypoplasia is typically identified and intervened upon in the neonatal period, some patients can remain asymptomatic or even be diagnosed as adults. Given the rarity of the condition persisting in adults though, it is not clear what medical or surgical interventions may be helpful if respiratory status declines.

Case description

In this report, we describe an elderly man with a history of right lung hypoplasia, pulmonary artery agenesis, and bronchial atresia who developed progressive dyspnea and worsening cough. His condition was complicated by multiple Aspergillus infections for which he received prolonged courses of anti-fungal therapy. He was also treated for bacterial pneumonia many times over a 10-year period. However, as his symptoms remained refractory to medical management, he underwent pneumonectomy, which revealed diffuse cystic changes in the right lung. He is currently doing well post-operatively with resolution of his dyspnea.

Conclusions

Although hypoplastic lung disorders have been described in asymptomatic adults, this report highlights the successful utilization of pneumonectomy in an individual with refractory symptoms much later in adulthood. This case additionally describes possible complications of cystic lung disease in this patient population, serving as further rationale for aggressive intervention.

SUBMITTER: Ramesh N

PROVIDER: S-EPMC9929657 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Pneumonectomy in an elderly patient with congenital unilateral lung hypoplasia and recurrent <i>Aspergillus</i>: a case report.

Ramesh Navneet N Velotta Jeffrey B JB

AME case reports 20221104

<h4>Background</h4>Congenital lung disorders encompass a spectrum of several conditions, one of which is lung hypoplasia. While hypoplasia is typically identified and intervened upon in the neonatal period, some patients can remain asymptomatic or even be diagnosed as adults. Given the rarity of the condition persisting in adults though, it is not clear what medical or surgical interventions may be helpful if respiratory status declines.<h4>Case description</h4>In this report, we describe an eld ...[more]

PMID: 36817703

Similar Datasets

Project description:UnlabelledIntroductionX-linked congenital adrenal hypoplasia is a rare developmental disorder of the human adrenal cortex and is caused by deletion or mutation of the dosage-sensitive sex reversal adrenal hypoplasia congenita critical region of the X chromosome, gene 1 (DAX-1) gene. Most affected children present with failure to thrive, salt wasting and hypoglycemic convulsions in the first months of life. Hypospadias affects approximately one in 250 live male births. Mutations in the mastermind-like domain-containing 1 (MAMLD1) gene have been implicated as one of the causes of hypospadias in children. To the best of our knowledge, an association between congenital adrenal hypoplasia due to a DAX-1 mutation and hypospadias due to mutation of the MAMLD1 gene has not previously been reported in the literature.Case presentationA 35-day-old male Egyptian baby was referred to our institution for the evaluation of a two-week history of recurrent vomiting associated with electrolyte imbalance. On examination, our patient was found to have hypotension and dehydration. A genital examination showed distal penile hypospadias with chordee and normal testes. He had hyponatremia, hyperkalemia, hypoglycemia and metabolic acidosis. Endocrinological investigations revealed low levels of cortisol, 17-hydroxyprogesterone and aldosterone, with a high level of adrenocorticotrophic hormone. A provisional diagnosis of congenital adrenal hypoplasia associated with hypospadias was made. A molecular genetics study confirmed the diagnosis of X-linked congenital adrenal hypoplasia due to DAX-1 mutations and hypospadias due to MAMLD1 mutation. He was started on hydrocortisone and fludrocortisone treatment. After three weeks of treatment, his symptoms improved and his blood sugar, sodium, potassium and cortisol levels normalized.ConclusionsWe report the case of an Egyptian baby with an association of congenital adrenal hypoplasia due to DAX-1 mutation and hypospadias due to MAMLD1 mutation. Early diagnosis of this association and determining its optimal treatment are vital in helping to avoid its fatal course.

Project description:BackgroundSurgical resection of locally advanced or borderline pancreatic ductal adenocarcinoma is a recognized procedure with curative intent performed in specialized oncology centers. Postoperative dysautonomia such as gastroparesis, mild hypotension, and diarrhea are common in elderly patients undergoing pancreaticoduodenectomy. A distinctive feature of our case, is the severing of an important sympathetic chain by the surgical procedure, leading to recurrent severe neurogenic shock. Locally advanced borderline tumor extension, aggressive maximal local tumor resection, and advanced age of the patient were the combined factors that explained the observed postoperative complication.Case descriptionAn 80-year-old woman underwent an elective R0 pancreaticoduodenectomy with total mesopancreas excision, distal gastrectomy and portal vein resection without relevant intraoperative and immediate postoperative complication. Pathology confirmed a 5.0 cm × 3.2 cm × 1.9 cm ductal adenocarcinoma in the head of the pancreas. After discharge, the patient returned to the emergency room complaining of nonspecific malaise, lipothymia, and cold sweating that was exacerbated by bowel movement attempts. During hospitalization, the patient experienced two additional severe hypotensive episodes with identical clinical presentation that required resuscitative measures in the intensive care unit (ICU). Because the third hypotensive episode developed without an obvious causal factor, apart from evacuation attempts, the hypothesis of neurogenic shock due to secondary splanchnic dysautonomia caused by extensive resection of the celiac plexus nerve structures after duodenopancreatectomy was considered.ConclusionsThis discussion is important, as it enables the care team to recognize this differential diagnosis and provide the best care for the patient. The patient was treated with sympathomimetics, fludrocortisone, and mechanisms to increase venous return when clinical improvement promptly occurred, allowing discharge from the hospital. Despite the challenging prognosis of the disease, we were able to provide the patient with moments at home with their family.