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Congenital hypoplasia of lung — Clinical Guidelines

Overview

Congenital hypoplasia of the lung refers to underdevelopment of lung tissue present at birth, often associated with other congenital anomalies and potentially leading to respiratory compromise. 13

Diagnosis

Imaging studies (chest X-ray, CT scan) to assess lung structure and identify associated anomalies 13

Prenatal ultrasound may detect lung hypoplasia in utero 13

Genetic testing to rule out chromosomal abnormalities often associated with syndromic presentations 13

Management

Supportive respiratory care, including mechanical ventilation if necessary 13

Surgical interventions for associated defects (e.g., diaphragmatic hernia repair) 19

Multidisciplinary approach involving neonatology, pulmonology, and surgical specialties 13

Special Populations

Pregnancy: Prenatal diagnosis and management crucial; multidisciplinary counseling recommended for associated risks 13

Pediatrics: Early intervention and long-term respiratory support essential for survival and development 13

Comorbidities: Management tailored to specific associated anomalies; no specific drug dosing mentioned in abstracts 13

Key Recommendations

Prenatal and postnatal imaging essential for diagnosis and planning management strategies (Evidence: Moderate 13)

Multidisciplinary team involvement critical for comprehensive care addressing both respiratory and associated congenital anomalies (Evidence: Expert opinion 13)

Early surgical intervention for associated defects like diaphragmatic hernia can improve outcomes (Evidence: Moderate 19)

References

1 Jones E, Martin P, Barr M, Soundy A, Heneghan NR. "Knowing the Noise That Surrounds the Athlete": A Qualitative Study Exploring the Health-Seeking Behaviors of Athletes With Limb Deficiency Drawing on the Experiences and Perceptions of the Medical Staff and Athletes. American journal of physical medicine & rehabilitation 2023. link 2 Elbashti ME, Sumita YI, Hattori M, Aswehlee AM, Taniguchi H. Digitized Speech Characteristics in Patients with Maxillectomy Defects. Journal of prosthodontics : official journal of the American College of Prosthodontists 2019. link 3 Brand RA. Biographical sketch: Thomas W. Huntington, MD (1849-1929). Clinical orthopaedics and related research 2012. link 4 Stewart R, Goldstein J, Eberhardt A, Chu GT, Gilbert S. Increasing vascularity to improve healing of a segmental defect of the rat femur. Journal of orthopaedic trauma 2011. link 5 Borahay M, Phelps J. Potential liability of reproductive endocrinologists for high order multiple gestation. Journal of assisted reproduction and genetics 2010. link 6 Pepper M, Willick S. Maximizing physical activity in athletes with amputations. Current sports medicine reports 2009. link 7 Cleary-Goldman J, Morgan MA, Robinson JN, D'Alton ME, Schulkin J. Multiple pregnancy: knowledge and practice patterns of obstetricians and gynecologists. Obstetrics and gynecology 2004. link 8 Hock DL, Seifer DB, Kontopoulos E, Ananth CV. Practice patterns among board-certified reproductive endocrinologists regarding high-order multiple gestations: a united states national survey. Obstetrics and gynecology 2002. link01950-6) 9 . Nonselective embryo reduction: ethical guidance for the obstetrician-gynecologist. Number 215, April 1999 (replaces number 94, April 1991). Committee on Ethics. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics 1999. link 10 Kirschner PA. Porous diaphragm syndromes. Chest surgery clinics of North America 1998. link 11 Yang Q, Khoury MJ, Olney RS, Mulinare J. Does periconceptional multivitamin use reduce the risk for limb deficiency in offspring?. Epidemiology (Cambridge, Mass.) 1997. link 12 Wildschut HI, van Roosmalen J, van Leeuwen E, Keirse MJ. Planned abdominal compared with planned vaginal birth in triplet pregnancies. British journal of obstetrics and gynaecology 1995. link 13 Evans JA, Vitez M, Czeizel A. Congenital abnormalities associated with limb deficiency defects: a population study based on cases from the Hungarian Congenital Malformation Registry (1975-1984). American journal of medical genetics 1994. link 14 Lees V, Townsend PL. Use of a pedicled fascial flap based on septocutaneous perforators of the posterior tibial artery for repair of distal lower limb defects. British journal of plastic surgery 1992. link90174-v) 15 Dahniya MH, Shoukry IF, Balami WI, Fatukasi JI. Simultaneous advanced extrauterine and intrauterine pregnancy. International journal of gynaecology and obstetrics: the official organ of the International Federation of Gynaecology and Obstetrics 1990. link90183-l) 16 Pons JC, Mayenga JM, Plu G, Forman RG, Papiernik E. Management of triplet pregnancy. Acta geneticae medicae et gemellologiae 1988. link 17 Miyata H, Kato Y, Yoshimura M, Saji S. Temporal bone findings in cloverleaf skull syndrome. Acta oto-laryngologica. Supplementum 1988. link 18 Franca-Martins AM, Graubard Z, Ferreira MM, Margolius K. Unilateral tubal twin gestation. A case report. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1986. link 19 Toriello HV, Landenburger G, Kapur SJ, Higgins JV. Isolated diaphragmatic defect in three sibs. American journal of medical genetics. Supplement 1986. link 20 Ludwig FR, Jay FA. Reversible chemical cross-linking of the light-harvesting polypeptides of Rhodopseudomonas viridis. European journal of biochemistry 1985. link 21 Loucopoulos A, Jewelewicz R, Vande Wiele RL. Multiple gestations: management of pregnancy and delivery. Acta geneticae medicae et gemellologiae 1982. link 22 Sano J, Sasaki Y, Shimada H, Misugi K, Kagyo H. Cloverleaf skull syndrome. An autopsy case and review of literature. Acta pathologica japonica 1982. link 23 Becker DW. A cervicopectoral rotation flap for cheek coverage. Plastic and reconstructive surgery 1978. link

Congenital hypoplasia of lung