The defect in gastroschisis is:
Gastroschisis represents a congenital anomaly characterized by a defect in the anterior abdominal wall through which the intestinal contents freely protrude. Unlike omphalocele, there is no overlying sac and the size of the defect is usually <4 em. The abdominal wall defect is located at the junction of the umbilicus and normal skin and is almost always to the right of the umbilicus (Fig. below), whereas for omphalocele the umbilical cord inserts into the sac. The umbilicus becomes partly detached, allowing free communication with the abdominal cavity. The appearance of the bowel provides some information with respect to the in utero timing of the defect. The intestine may be normal in appearance, suggesting that the rupture occurred relatively late during the pregnancy. More commonly, however, the intestine is thick, edematous, discolored, and covered with exudate, implying a more longstanding process.
Gastroschisis in a newborn. Note the location of the umbilical cord, and the edematous, thickened bowel.
The initial treatment for a pure EA (no fistula) is:
Primary EA (type A) represents a challenging problem, particularly if the upper and lower ends are too far apart for an anastomosis to be created. Under these circumstances, treatment strategies include placement of a gastrostomy tube and performing serial bougienage to increase the length of the upper pouch. This occasionally allows for primary anastomosis to be performed. Occasionally, when the two ends cannot be brought safely together, esophageal replacement is required, using either a gastric pull-up, reverse gastric tube, or colon interposition.
The most common branchial cleft fistula originates from the:
Paired branchial clefts and arches develop early in the fourth gestational week. The first cleft and the first, second, third, and fourth pouches give rise to adult organs. The embryologic communication between the pharynx and the external surface may persist as a fistula. A fistula is seen most commonly with the second branchial cleft, which normally disappears, and extends from the anterior border of the sternocleidomastoid muscle superiorly, inward through the bifurcation of the carotid artery, and enters the posterolateral pharynx just below the tonsillar fossa. In contrast, a third branchial cleft fistula passes posterior to the carotid bifurcation. The branchial cleft remnants may contain small pieces of cartilage and cysts, but internal fistulas are rare. A second branchial cleft sinus is suspected when clear fluid is noted draining from the external opening of the tract at the anterior border of the lower third of the sternomastoid muscle. Rarely, branchial cleft anomalies occur in association with biliary atresia and congenital cardiac anomalies, an association that is referred to as Goldenhar complex.
Which of the following is characteristic of congenital lobar emphysema?
Congenital lobar emphysema is a condition manifested during the first few months of life as a progressive hyperexpansion of one or more lobes of the lung. It usually occurs in the upper lobes of the lung (left greater than right), and is caused by intrinsic bronchial obstruction from poor bronchial cartilage development or extrinsic compression. Treatment is resection of the affected lobe, which can be safely performed using either an open or thoracoscopic approach. The other choices describe bronchiectasis, pulmonary sequestration, and congenital pulmonary airway malformation, respectively.
An infant is referred to your clinic for evaluation of a neck mass. On physical examination, the patient has a left lateral neck mass with his head rotated to the opposite side. Which of the following is the most likely diagnosis?
The management of neck masses in children is determined by their location and the length of time that they have been present. Neck lesions are found either in the midline or lateral compartments. Midline masses include thyroglossal duct remnants, thyroid masses, thymic cysts, or dermoid cysts. Lateral lesions include branchial cleft remnants, lymphatic malformations (previously known as cystic hygroma), vascular malformations, salivary gland tumors, torticollis, and lipoblastoma. The presence of a lateral neck mass in infancy in association with rotation of the head toward the opposite side of the mass indicates the presence of congenital torticollis. This lesion results from fibrosis of the sternocleidomastoid muscle, and is histologically characterized by the deposition of collagen and fibroblasts around atrophied muscle cells. In the vast majority of cases, physical therapy based on passive stretching of the affected muscle is of benefit. Rarely, surgical transection of the sternocleidomastoid may be indicated.
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