Presentation
CPL can present antenatally as nonimmune hydrops with pleural effusions, which are frequently associated with polyhydraminos. CPL presents in the neonatal period with severe respiratory distress and can be complicated by chylothorax. [ncbi.nlm.nih.gov]
Entire Body System
- Developmental Disorder
Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder of the lung. CPL can present antenatally as nonimmune hydrops with pleural effusions, which are frequently associated with polyhydraminos. [ncbi.nlm.nih.gov]
Congenital pulmonary lymphangiectasia (CPL) is a rare developmental disorder that is present at birth (congenital). Affected infants have abnormally widened (dilated) lymphatic vessels within the lungs. [healthetreatment.com]
Congenital pulmonary lymphangiectasia Description, Causes and Risk Factors: Abbreviation: CPL.Congenital pulmonary lymphangiectasia is a rare developmental disorder that is present at birth (congenital). [medigoo.com]
A developmental disorder of the structures in the chamber angle may have caused the increased IOP. Unfortunately, we do not have any early description of the chamber angle and specimens from the trabeculectomies were not obtained. [healio.com]
Eyes
- Ectopia Lentis
الصفحة 896 - Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. N Engl J Med 1992;326:905-909. [books.google.com]
Face, Head & Neck
- Broad Nasal Bridge
bridge Broad nasal bridge Broad nasal root Broadened nasal bridge Increased breadth of bridge of nose Increased breadth of nasal bridge Increased width of bridge of nose Increased width of nasal bridge Nasal bridge broad Wide bridge of nose Widened nasal [rarediseases.info.nih.gov]
- Low Nasal Root
[…] bridge Depressed bridge of nose Flat bridge of nose Flat nasal bridge Flat, nasal bridge Flattened nasal bridge Low nasal bridge Low nasal root [ more ] 0005280 Facial edema Facial puffiness Facial swelling [ more ] 0000282 Flat face Flat facial shape [rarediseases.info.nih.gov]
Neurologic
- Neonatal Seizures
Arterial Stroke 1192 140 Neonatal Seizures 1199 141 The Timing of Neonatal Brain Damage 1208 142 Thrombosis in the Development of Newborn Brain Damage 1216 143 Epidemiology of Adverse Cerebral Outcome 1228 144 Neuromuscular Disorders 1232 145 Ocular [books.google.com]
Treatment
Ethiodized oil lymphangiography represents a new treatment modality for some patients with CPL. [ncbi.nlm.nih.gov]
85 Pathology and Treatment of Liver Diseases 641 86 Neonatal CholestasisConjugated Hyperbilirubinemia 650 87 Surgical Treatment of Biliary Tract Malformations 659 88 Orofacial Malformations 664 89 Esophageal Atresia 675 90 Gastrointestinal Malformations [books.google.com]
While it has been recognised in the literature since the 1950s, no standardised treatment has yet emerged and a definitive cause is still unknown. [journaloflymphoedema.com]
Prognosis
Congenital pulmonary lymphangiectasia is a rare condition that may present antenatally with pleural effusions and hydrops, and the prognosis is reported to be very poor. [ncbi.nlm.nih.gov]
This review seeks to draw together current information on diagnosis, treatment and prognosis, and to recommend that a systemic evaluation of such would provide direction for clinicians in dealing with patients aᰀected by this condition. [journaloflymphoedema.com]
Type 3 is associated with an aggressive course and poor prognosis. [8,9] Patients with type 1 CPL have better prognosis. Most of type 2 patients may die in fetal stage or only a few days after birth. [wjpch.com]
Etiology
Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. [ncbi.nlm.nih.gov]
Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion. Prenat Diagn. 2006 Aug 30; PMID: 16941717 Dempsey EM, Sant’Anna GM, Williams RL, Brouillette RT. [humpath.com]
A consistent chapter format enables you to rapidly and effortlessly locate the most current protocols on manifestations, etiologies, triggers, approaches to treatment, complications, and preventative strategies. [books.google.com]
Wilson RD, Pawel B, Bebbington M, et al: Congenital pulmonary lymphangiectasias sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion. Prenat Diagn 2006;26:1058–1061. [karger.com]
Epidemiology
The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly. [books.google.com]
EPIDEMIOLOGY: incidence: ? age of onset: neonatal period and after the neonatal period risk factors: Noonan's Syndrome Turner's Syndrome TYPES (after Noonon (1970))*: 1. [lymphedemapeople.com]
Pathophysiology
699 92 Meconium Plug Syndrome 704 93 Hirschsprungs Disease 708 94 Gastroenteritis and Intractable Diarrhea 713 95 Rehydration after Diarrhea 719 96 Necrotizing Enterocolitis 724 97 Surgical Treatment of Necrotizing Enterocolitis 731 Overview 735 99 Pathophysiology [books.google.com]
Prevention
The anomalies are organized by anatomical system and presented in a consistent manner, including details of the clinical presentation, epidemiology, embryology, treatment and prevention for each anomaly. [books.google.com]
Previous hypotheses suggested that CPL might be caused by conditions preventing normal regression of the lymphatics after the 18th-20th week of gestation. [ncbi.nlm.nih.gov]
How can Congenital Lymphangiectasia be Prevented? Current medical research has not established a method of preventing Congenital Lymphangiectasia. [dovemed.com]
High lines @T8-10, low lines below L3, to prevent major arterial thrombosis. Umbilical venous catheter -> left portal vein -> ductus venosis -> hepatic vein -> IVC. Ideally @ RA/IVC junction. [radnotes.co.nz]