Bartsocas-Papas syndrome: The first case report of severe autosomal recessive form from Indonesia.

INTRODUCTION: Bartsocas-Papas syndrome (BPS) is an autosomal recessive form of Popliteal Pterygium syndrome (PPS). It is a very rare disease characterized by congenital craniofacial anomalies, popliteal webbing, and genitourinary and musculoskeletal anomalies. Almost all of the cases were reported in dead intrauterine pregnancies. PRESENTATION OF CASE: We present a 10-month-old boy with bilateral complete cleft lip and palate, abnormal scalp hair, an absence of both upper eyelids, choanal atresia, syndactyly of the third and fourth fingers of the right hand, agenesis fingers on the left hand, bilateral popliteal pterygia, bilateral talipes equinovarus, agenesis of the toes of both lower extremities, intercrural webbing, an absence of testis, and scrotal anomaly. Multistage surgical correction was performed for the multiple congenital malformations. CONCLUSION: We report the first case of BPS from Indonesia. Gradual management should be performed according to the patient's age and available facilities.

Anthropometric Outcome of Primary Unilateral Cleft Lip Repair in Indonesia.

OBJECTIVE: This study aimed to measure and analyze the outcome of primary unilateral cleft lip repair. DESIGN: Observational cohort study. SETTING: Surabaya Cleft Lip and Palate (CLP) Center, a major referral center for the Eastern part of Indonesia, affiliated with a tertiary center. PATIENTS, PARTICIPANTS: From 69 patients who met the inclusion criteria, we excluded 31 patients who were more than 2 years of age and were operated on by junior residents under supervision. INTERVENTIONS: We performed anthropometric measurements of the patients on photographs taken before, immediately after, and a year after the surgery. MAIN OUTCOME MEASURE(S): This study measured nasal width, vertical lip height, horizontal lip length, and philtral height ratios. RESULTS: While nasal width and philtral height ratios decreased significantly (P = .000 and P = .000, respectively) reaching symmetry immediately after surgery, the horizontal lip length, and vertical lip height ratios remained unchanged (P = .862 and P = .981, respectively). A year after surgery, the nasal width and horizontal lip length ratios increased significantly (P = .017 and P = .006, respectively), while philtral height and vertical lip height ratios remained unchanged (P = .927 and P = .138, respectively). There was no difference in the ratios based on the initial size and completeness of the cleft. CONCLUSION: In Surabaya CLP Center, the symmetry of nasal width, philtral height, horizontal lip length, and vertical lip height were achieved by the unilateral cleft lip repair despite the initial size and completeness of the cleft.