Ann M. Kosloske

Intralobar pulmonary sequestration: A clinical and pathological spectrum

Pulmonary sequestration is a mass of abnormal pulmonary tissue that does not communicate with the tracheobronchial tree and is supplied by an anomalous systemic artery. Whereas extralobar sequestration is clearly congenital, intralobar sequestration, which frequently presents in older children with pathological findings showing acute and chronic inflammation, may have an acquired origin secondary to frequent infections. Several large autopsy series support an acquired etiology of intralobar sequestration. Four cases of intralobar sequestration are presented that demonstrate a spectrum of inflammatory change that support its congenital, rather than acquired origin. Case 1 was a newborn who presented with tachypnea and a right lower lobe density. Resection at 3 weeks of age showed no inflammation in the sequestration specimen. Case 2 presented as a newborn infant with congestive heart failure. Pulmonary sequestration was confirmed by arteriogram. Resection at 3 months of age showed chronic inflammation. Case 3 presented at 7 months of age with chronic pneumonia. The resected specimen demonstrated moderately severe acute and chronic inflammation. Case 4 presented as a 6 year old. The operative specimen showed extensive bronchiectatic changes with marked acute and chronic inflammation. These cases support the congenital origin of intralobar sequestration and suggest a temporal progression from no inflammation to severe acute and chronic inflammation.

Debridement of Periumbilical Necrotizing Fasciitis: Importance of Excision of the Umbilical Vessels and Urachal Remnant

The operation of a neonate with periumbilical necrotizing fasciitis consisted of (1) excision of infected skin, fat, and fascia (including the umbilicus); (2) a limited laparotomy, with ligation and excision of the umbilical vessels and urachal remnant; and (3) placement of a temporary silastic patch over the central abdominal defect. Pathological sections confirmed the spread of infection along the vessels and urachal remnant. Excision of the vessels and urachal remnant may be crucial to survival from periumbilical necrotizing fasciitis.

Left mainstem bronchopexy for severe bronchomalacia

Severe bronchomalacia occurred in a 14-month-old boy, as a result of compression of the left mainstem bronchus by a bronchogenic cyst. After resection of the cyst, the bronchomalacia was corrected by suspension of the posterolateral bronchial wall to the ligamentum arteriosum. This method of bronchopexy may be of value for severe left mainstem bronchomalacia.

Perforated appendicitis in children: to drain or not to drain?

We studied 66 children with perforated appendicitis at the University of New Mexico to determine whether or not transperitoneal drainage has any advantage in the management of these children. Patients were assigned to one or the other treatment group on the basis of the call schedule of the attending surgeons, two of whom preferred drainage and two of whom did not. Other aspects of appendicitis management (e. g., supportive care, antibiotics) were the same for both groups. Thirty-two other children who had an abscess at the time of appendectomy were excluded from the analysis. The two study groups were similar in age and severity of illness. Postoperative complications (wound infection, abdominal abscess, small-bowel obstruction) had a similar incidence in the two groups: 6/32 (18.8%) for the drained group and 7/34 (20.6%) for the undrained group. The hospital stay was significantly longer for the drained group (mean 10.1 days, median 9 days) versus the undrained group (mean 7.0 days, median 7 days). The power of our study was 0.52; twice our sample size would have been required to achieve a power of 0.80. The evidence suggests that, unless an abscess is present, drainage may be abandoned for children with perforated appendicitis.

An Expandable Prosthesis for Stabilization of the Infant Mediastinum Following Pneumonectomy

One possible complication in infant pneumonectomy is mediastinal shift that can fatally kink or compress airways and vessels. Rigid prostheses have been used to prevent these problems; however, they cannot be adjusted as the child grows. We report a case of expandable prosthesis implantation in a 24-day-old infant. During the 18 months postimplantation, the prosthesis was periodically injected with a saline/contrast solution to maintain the mediastinum in a midline position as the child grew. At 24-month follow-up the prosthesis was still in place, and midline position of the mediastinum maintained.

Favorable prognosis of vasoactive intestinal peptide-secreting ganglioneuroblastoma

We report a 2.5-year-old girl with ganglioneuroblastoma, whose presentation was the watery diarrheal syndrome due to secretion of vasoactive intestinal peptide (VIP) by the tumor. The diarrhea subsided after resection of the tumor in two stages; the child is a long-term survivor. A review of the literature disclosed survival of 36/41 (88%) children with neural tumors that secreted VIP, including 22/26 (85%) with neuroblastoma or ganglioneuroblastoma. Thirty-five of 36 children who had resection of the tumor survived (97%), although resection was sometimes incomplete. VIP may be a marker predicting maturation of ganglioneuroblastoma and a favorable outcome. Aggressive surgery is warranted for control of the watery-diarrhea syndrome.

Normal Radiographic Findings After Foreign Body Aspiration When the History Counts

Foreign body aspiration in children is frequently associated with unilateral emphysema or atelectasis on chest x-ray. Two cases are reported of tracheal or bilateral foreign bodies in which the original chest x-rays were read as normal, but the history was suggestive of the foreign body aspiration. Early bronchoscopy can prevent the long-term morbidity that results from unrecognized tracheobronchial foreign bodies.

Acute abdominal emergencies associated with cytomegalovirus infection in the young infant

Gastrointestinal signs and symptoms have rarely been reported in association with cytomegalovirus (CMV) infection in young infants. However, in 1981 clear pathologic evidence was presented implicating this virus as a cause of hypoganglionosis and bowel dysmotility. We report our experience with four infants with CMV infection in whom gastrointestinal dysfunction was the reason for emergency abdominal operation. Since the association was made retrospectively, we were unable to demonstrate hypoganglionosis, but our experience underscores the need to include CMV intestinal infection in the differential diagnosis of the acute surgical abdomen in young infants.