Don't let a common manifestation of one major anomaly blinds us to another

مقدمه/ بیان مساله : Hypertrophic pyloric stenosis is the most common cause of gastric outlet obstruction in young infants. polycystic kidney disease(PKD) is characterized by the progressive development and growth of numerous bilateral renal cysts, resulting in numerous abnormalities. It is unclear whether the relationship between PKD and IHPS is due to genetics, a pathophysiological link or a chance phenomenon.معرفی بیمار :The patient is a ۲۶-day-old infant, In the initial examination and evaluation of the newborn, no obvious congenital anomalies were observed. Due to breathing problems, the baby was admitted to the neonatal ward. ۳ days after the start of oral feeding, the baby had non-bilious vomiting. On examination, the abdomen was distended and masses with ill-defined borders were palpated in both flanks. After initial resuscitation of fluids and elimination of dehydration, due to food intolerance, sepsis workup and abdominal ultrasound were performed . On ultrasound study, The length of the left kidney was ۷۵ mm and the right kidney was ۷۳ mm. Hyper echogenicity was seen in the medulla of both kidneys, and numerous small cysts were evident throughout the parenchyma of both kidneys and corticomedullary differentiation was lost. At first, the infant's vomiting was attributed to the mass effect of polycystic kidneys. After reviewing the patient's history, with the suspicion of stenotic pyloric hypertrophy, an abdominal ultrasound was performed. the thickness of the pylorus was measured as ۴.۴ mm. According to the results of imaging and tests, a definitive diagnosis of congenital hypertrophic pyloric stenosis and a possible diagnosis of ARPKD were made for the patient. he underwent pyloromyotomy surgery with Ramstedt technique to correct pyloric stenosis. Also, during the operation, biopsies were taken from liver.

کلیدواژه ها:

Hypertrophic pyloric stenosis ، polycyctic kidney disease ، projectile vomiting