Case Report: Congenital nasal pyriform aperture stenosis with holoprosencephaly: Diagnosis and surgical management

Congenital nasal pyriform aperture stenosis (CNPAS) is a rare cause of neonatal nasal obstruction that clinically mimics choanal atresia. It occurs as a result of abnormal development of primary palate and maxilla, either in isolation or in association with other anomalies. Differentiation between CNSPAS and choanal atresia is critical as management approachers are quite different from one another. Diagnosis can be made clinically and with characteristics CT findings. Management options include both conservative and surgical approaches depending on the patient’s initial condition. Here is reported a case of 33 days infant who was admitted to our NICU with a working diagnosis of CNPAS after she presented with two days of fast breathing and failure to suckle, managed surgically and discharged improved.

Primary management of cleft lip and palate

Cleft lip and palate is a condition with the potential to impact the lifetime of a patient, affecting overall health, appearance, communication, facial growth, hearing, eating, dental health, and psychological well-being. To effectively manage these multiple effects, the treatment of cleft lip and/or palate requires involvement of multiple healthcare specialists. This multidisciplinary management is difficult to coordinate so is often delivered by extended multidisciplinary healthcare teams. The early management of a child with a cleft lip and/or palate centres on the well-being of the child. The focus is on feeding, breathing, and managing medical comorbidities. Care can require extensive input from paediatric medical specialists, and needs to occur prior to any surgical management of the cleft lip or palate. This chapter discusses the surgical and perisurgical management of the primary cleft lip and/or palate including manipulation of the cleft lip prior to definitive surgery, primary lip surgery, primary palate surgery, and primary closure of the alveolar cleft with an assumption of medical well-being.

A Closer Look at Delayed Primary Cleft Surgery and Unrepaired Cleft Lip and/or Palate in 5 UK Cleft Centers

Background: There may be many reasons for delays to primary cleft surgery. Our aim was to investigate the age of children undergoing primary cleft lip or primary cleft palate repair in 5 cleft centers within the United Kingdom. Identify the reasons for delayed primary cleft lip repair (beyond 6 months) and delayed primary palate repair (beyond 13 months). Identify children who had a cleft lip and/or palate (CL±P) that was intentionally unrepaired and the reasons for this. Methods: A retrospective, multicenter review of patients born with a CL±P between December 1, 2012, and December 31, 2016. Three regional cleft centers, comprising of 5 cleft administrative units in the United Kingdom participated. Results: In all, 1826 patients with CL±P were identified. Of them, 120 patients had delayed lip repair, outside the expected standard of 183 days. And, 178 patients in total had delayed palate repair, outside the expected standard of 396 days. Twenty (1%) patients had an unrepaired cleft palate. Conclusions: This large retrospective review highlights variations between centers regarding the timing of lip and palate surgery and details the reasons stated for delayed primary surgery. A small number of patients with an unrepaired cleft palate were identified. All had complex medical problems or comorbidities listed as a reason for the decision not to operate and 50% had a syndromic diagnosis. The number of patients receiving delayed surgery due to comorbidities, being underweight or prematurity, highlights the importance of the cleft specialist nurse and pediatrician within the cleft multidisciplinary team.

Descriptive Overview of Primary Cleft Palate Surgeries in the Low- and Middle-Income Countries

Objective: To increase access to high-quality and multiregional databases in global epidemiology of cleft surgeries through partnership with an NGO. Design: The study retrospectively analyzes 34 801 primary palate surgeries in 70+ countries from the 2016 electronic health records of an non-governmental organization (NGO). The study also utilizes the Kids’ Inpatient Database to compare the epidemiology of primary cleft palate surgeries in the United States. Participants: Patient records of those undergoing primary cleft palate surgeries only. Main Outcome Measures: Region, age, sex, type of cleft, laterality of cleft. Results: Key findings show that average age of those receiving primary cleft palate surgery in the low- and middle-income countries (LMICs) was 1.95 years. The distribution of males and females receiving surgery corresponds to the US national data. More hard cleft palates were on the left side (66.18%) than the right side (33.82%), independent of gender and region. Conclusions: Databases from an established NGO can be used to enhance our understanding of the disease characteristics in these regions. By increasing the information available regarding cleft surgeries in the LMIC, we hope to increase awareness of the similarities and differences in surgeries across various regions, as part of an effort to inform the goals set by Global Surgery 2030 initiative by the Lancet Commission.