Coexistence of meconium ileus with duodenal atresia and trisomy 21 in a newborn: a case report.

The coexistence of duodenal atresia (DA) may mask the antenatal ultrasound findings of meconium ileus (MI) and delay its postnatal diagnosis. We report a rare case of MI in a newborn infant diagnosed antenatally to have trisomy 21 and DA. The diagnosis of MI was only established intraoperatively after the patient showed persistent signs of intestinal obstruction following the surgical repair of the DA.

Rectal prolapse in autistic children.

Rectal prolapse in children is not uncommon, but surgery is rarely indicated. In mentally challenged adults and children, rectal prolapse occurs more frequently than in the general population and often requires surgical intervention in the second to third decade of life. The authors describe 3 children with autism and mental retardation who presented with rectal prolapse at an earlier age than would be anticipated with mental retardation alone. All 3 children required surgical intervention.

Splenic injuries in children: correlation between imaging and clinical management.

For non-operative management of splenic injuries in children, questions remain as regards imaging, length of hospital stay, and follow-up. We reviewed our experience concerning these issues over the past 15 years. The charts of all children with splenic injuries from January 1983 to December 1998 were reviewed. All computed tomography (CT) scans were reviewed and classified according to degree of splenic injury. Fisher's exact test and a linear regression model were used to analyze data. Sixty-nine males and 23 females under 16 years of age were identified; 57 (62%) had CT scans; 8 underwent a laparotomy; and 6 (6.5%) died. Age, on hemoglobin admission, and duration of intensive-care-unit stay were significantly related to hospital stay (median 8 days). Motor vehicle-pedestrian-related injuries correlated significantly with mortality. CT class did not correlate with mortality (P = 0.68) or hospital stay (P = 0.2). Follow-up data were available for 66 patients, 36 of them with imaging. Follow-up imaging did not impact on clinical decisions and no late sequelae were identified.

Congenital cervical teratoma in neonates. Case report and review.

Cervical teratomas are uncommon neoplasms. Although these lesions are histologically benign they are usually large and may cause airway obstruction. Cervical teratomas are usually diagnosed at birth. In-utero diagnosis is possible by prenatal ultrasound which assists in planning early airway management and surgical intervention. Mortality is significant but prognosis is good with airway control and complete surgical excision. However, pressure injury of contiguous structures can limit resectability and adversely affect outcome. Malignant cervical teratoma with metastasis has been reported mostly arising in adults with poor outcome. We present nine cases of neonatal cervical teratoma identified at two institutions between 1984 and 1996. One patient died before surgical intervention. All others underwent resection. There was one intraoperative death and one postoperative death. The remaining six patients did well postoperatively with no significant sequelae with 3 to 14 years follow-up.

Femoral hernia in children.

BACKGROUND/PURPOSE: Femoral herniae are uncommon in children and easily misdiagnosed. To evaluate performance with femoral hernia in children, the authors reviewed their experience for the past two decades. METHODS: All patients under 15 years of age with femoral hernia (January 1977 to January 1998) were reviewed. Age, gender, presentation, surgical findings and procedure, and previous repair were recorded. RESULTS: There were nine girls (53%) and eight boys (47%). Age range was 2 to 15 years. Thirteen were right side (77%), three were left side (18%), and one was bilateral (6%). All presented with a recurrent lump in the groin, one with incarceration. Duration of symptoms ranged from 1 day to 3 years (median, 3 months). Six cases were diagnosed correctly preoperatively (35%); the others were thought to be either an inguinal hernia or recurrent inguinal hernia. CONCLUSIONS: Femoral hernia in childhood is a challenging clinical problem because of its rarity and similar clinical presentation as indirect inguinal hernia. The frequency with which an incidental indirect inguinal hernia sac or patent processus vaginalis can be found at surgery can perpetuate a misdiagnosis. The absence of an expected indirect inguinal hernia sac or an apparent recurrence of an indirect inguinal hernia should lead to consideration of a possible femoral hernia.

Management of impalpable testes: indications for abdominal exploration.

Various approaches to the management of the impalpable testis in cases of cryptorchidism have been advocated. The authors' experience over the past 13 years was reviewed to try to determine an optimal approach. Of 1,305 patients with undescended testicles seen between February 1982 and December 1995, 157 boys (12.03%) had impalpable testes with 17 having bilateral impalpable testes for a total of 174 impalpable testes. A hernia sac was present in 155 impalpable testes with a testicle present in all cases. No hernia sac was found in 19 impalpable testes, five of which had no testicle present. This was confirmed by either open exploration or laparoscopy. One hundred forty-eight boys underwent groin exploration as initial treatment, 13 of these had bilateral impalpable testes. In addition to the five absent testicles with no hernia sac, one patient with a hernia sac and no testicle evident benefited from subsequent laparoscopy to identify an intraabdominal testicle. All other patients underwent routine orchidopexy or orchidectomy (one case with grossly malformed testicle). Nine boys underwent laparoscopy as initial treatment, four of these had bilateral impalpable testes. Two abnormal testicles were found and removed. Groin exploration and subsequent orchidopexy was definitive treatment in all other cases. The association of a hernia sac with an impalpable undescended testicle is very significant (P < .00001 Fisher's Exact test). The absence of a sac therefore may reflect an alternate diagnosis. When no sac is found with a testicle in the groin, this may represent an ectopic testicle. When no sac is found with no testicle, this may represent a vanishing testicle. From this experience the authors conclude that groin exploration should be the initial approach to impalpable testes. The presence of a hernia sac with an absent testicle demands further exploration; the absence of a hernia sac with an absent testicle suggests a vanishing testicle and may need no further exploration.

Disorders in foregut diverticulization: congenital hepaticoesophageal fistula in a baby with laryngotracheoesophageal cleft.

Congenital fistulae between the biliary tract and the proximal foregut are rare. Nineteen cases have been reported involving a fistula tract between the left hepatic duct and the distal trachea or proximal bronchus. Herein the authors describe a fistula between the hepatic duct and the esophagus. A baby girl had a type III laryngotracheoesophageal cleft repaired in the neonatal period. In the course of investigating her bilious vomiting a DISIDA scan showed radionuclide excretion into the distal esophagus. A percutaneous cholangiogram through the gallbladder showed no drainage from the common bile duct into the duodenum, while the proximal left hepatic duct drained into the distal esophagus. The fistula was divided adjacent to the esophagus and the biliary system was drained through a Roux-en-Y cholecystojejunostomy. Fourteen months later she is doing well with no evidence of hepatic compromise.

Perianal ependymoma presenting in the neonatal period.

Extraspinal ependymomas are a rare type of glioma that may arise in the sacrococcygeal region, presenting as a pelvic mass in an infant or child. Ependymoma presenting in the newborn period has not been described previously. Herein we describe a case of a newborn boy who presented with a perianal ependymoma, which was subsequently found to have presacral extension. The major diagnostic challenge this case presented was to rule out the alternative diagnosis of sacrococcygeal teratoma or a developmental malformation/heterotopia.

Routine neonatal circumcision?

Routine neonatal circumcision is still a controversial procedure. This article attempts to clarify some of the advantages and disadvantages of neonatal circumcision. The increased rate of penile cancer among uncircumcised men appears to justify the procedure, but that alone is not sufficient justification. The final decision on neonatal circumcision should be made by parents with balanced counsel from attending physicians.

Impact of diagnostic laparoscopy in the management of the unilateral impalpable testis.

OBJECTIVE: To evaluate the impact of laparoscopy on the management of children with a unilateral impalpable testis. PATIENTS AND METHODS: The study population consisted of 27 children who underwent a primary inguinal exploration for a unilateral impalpable testis. RESULTS: Twelve of 27 (44%) children had inguinal or 'peeping' testes and 10 of 27 (37%) had blind-ending vasa and vessels in the inguinal canal; four of these 10 had atrophic tubular tissue in the excised remant. Four of 27 (15%) had blind-ending vasa and vessels proximal to the internal ring. Only one child had a testis proximal to the internal ring. Only the four children (15%) with blind-ending vasa and vessels proximal to the internal ring would have benefited from a laparoscopy by avoiding an inguinal exploration. CONCLUSIONS: Because of the time, expense and limited usefulness of laparoscopy in altering the management of children with a unilateral unpalpable testis, we reserve laparoscopy for cases where inguinal exploration has failed.

Survival in neonatal congenital hernia without extracorporeal membrane oxygenation support.

The experience with high-risk congenital diaphragmatic hernia (CDH) at an institution that does not offer extracorporeal membrane oxygenation (ECMO) was reviewed. Between January 1, 1983 and December 31, 1993, 38 children presented with Bochdalek-type CDH. Excluded were two infants with lethal cardiac anomalies and four presenting after 4 hours of age. Thus, the authors identified 32 high-risk patients. All had early respiratory distress and were intubated within 5 hours of birth. Sixteen were inborn; 16 were referred to the Izaak Walton Killam Children's Hospital (IWK) within 24 hours of birth. There were 19 males and 13 females. Three died before surgery could be attempted. Twenty-two survived, giving an overall survival rate of 69% (22 of 32). For 28 of the 32, the best preoperative postductal Pao2 (BPDPao2) was recorded. Fifteen of the 28 children had a BPDPao2 of greater than 100 mm Hg. Survival in this group was 14 of 15 (93%). Thirteen of the 28 patients had a BPDPao2 of less than 100 mm Hg. Survival in this group was 5 of 13 (38%). These survival rates are comparable to those of centers offering ECMO. BPDPao2 appears to be a useful discriminating variable.

Omental-mesenteric inflammatory pseudotumor. Cytogenetic demonstration of genetic changes and monoclonality in one tumor.

BACKGROUND: Extrapulmonary inflammatory pseudotumor (plasma cell granuloma) is an uncommon lesion in adults and children, and little is known either of its etiology or clinical characteristics. However, it remains a significant source of morbidity to patients and confusion to clinicians. METHODS: Case reports are presented of three patients with intraabdominal inflammatory pseudotumor who recently underwent surgery. A review of the recent world literature is also presented. Clinical and laboratory characteristics of omental-mesenteric inflammatory pseudotumor are reviewed along with a discussion of its etiology. RESULTS: Cytogenetic data from cells of one patient show a derivative chromosome evolved from a translocation between the long arm of chromosome 2 and the short arm of chromosome 9 [(2;9)(q1,3;p2,2)]. CONCLUSIONS: The lesion is monoclonal, and genetic changes may play a crucial role in the development of this neoplasm. Omental-mesenteric inflammatory pseudotumor appears to represent a distinct clinicopathologic entity as a benign neoplasm in children.

Routine inguinal hernia repair in the pediatric population: is office follow-up necessary?

Routine inguinal hernia repair in the pediatric population has a low complication rate. Very few complications are identified at follow-up, which brings into question the necessity of the traditional postoperative visit. A retrospective review of patients who had undergone a routine inguinal hernia repair in 1991 at our institution was done in order to determine our current follow-up practices and complication rate. To determine the perceived necessity for the follow-up visit, parents were given a short telephone questionnaire. Of 175 eligible patients, questionnaires were completed on 145. Of these 145 patients, 77 were seen in follow-up by the pediatric surgeon only, 43 by the family doctor only, 12 were seen by both, and 13 patients had no physician follow-up. The sole complication was a stitch abscess (complication rate 0.7%). Results of the questionnaire showed that 90% of parents felt the follow-up visit was "helpful," 80% felt it was "necessary," and 35% would have been satisfied with telephone follow-up. The main purpose of the postoperative visit appears to be parental reassurance. Careful preoperative and postoperative instruction and reassurance may be sufficient in a significant number of cases.

Nipple discharge and breast lump related to Montgomery's tubercles in adolescent females.

Four adolescent girls, aged 12 to 14 years, were seen for evaluation of spontaneous nipple discharge, two of whom had associated breast lumps in the ipsilateral breast. Clinical examination showed the discharge to be arising from one or several of Montgomery's areolar tubercles, with the breast lumps localized to the subareolar region immediately beneath the discharging tubercle. The secretions were episodic, thin, varied in color from clear to brown, but were not milky. All discharges resolved within 3 to 5 weeks, and the associated breast lumps resolved within 4 months without treatment. There were no associated clinical complaints or physical findings and detailed endocrinologic assessments including serum prolactin, luteinizing hormone (LH), follicle-stimulating hormone (FSH), thyroid function tests, and 17 beta estradiol; they were all normal. None of these patients was pregnant, and follow-up from 4 to 18 months did not reveal evidence of recurrence or other pathology. Experience gained from these four cases suggests that cysts and spontaneous, non-milky discharge related to Montgomery's tubercles in otherwise healthy, nonpregnant adolescent females, represents a benign, self-limiting problem. Unless other indications are present, endocrinologic investigation is unnecessary and spontaneous resolution can be expected.

Neurenteric cysts--a spectrum.

This review encompasses seven patients with clinically important cystic lesions of the gastrointestinal (GI) tract, exhibiting a wide range of vertebral anomalies and connections to the neural canal. Three patients had mediastinal masses connected to lower cervical and upper thoracic anomalous vertebrae with intraspinal extensions. In addition, one of these patients had a separate, juxtapancreatic intestinal duplication cyst. One infant with colonic duplication had a lumbar vertebral anomaly and an epithelial-lined tract between the two. Another patient had a presacral cystic mass which was the site of recurrent infections and meningitis until a connection with the rectum was divided. A newborn baby had a completely split notochord syndrome with a large dorsal enteric fistula. Finally, one patient had a dorsal enteric cyst with a direct intraspinal connection. Four of the seven patients had associated significant congenital anomalies, two of whom died early in the neonatal period. The rest of the patients did well. This broad range of enteric lesions with associated vertebral and intraspinal abnormalities suggests that the clinical spectrum of neurenteric cystic lesions is much wider than is generally appreciated.

Paraesophageal hernia after Nissen fundoplication: a real complication in pediatric patients.

Eighty-nine pediatric patients aged 6 weeks to 20 years (mean, 3.8 years) who underwent Nissen fundoplication were reviewed. Follow-up, including upper gastrointestinal (GI) series, was obtained in 55 patients (61.8%). Fifteen patients developed paraesophageal hernia (PEH) (16.8%). PEH was diagnosed between 4 and 36 months following fundoplication (mean, 17 months). Patients were divided into three groups: A, those with significant mental dysfunction (45); B, those with previous tracheoesophageal fistula (12); and C, others (32). Incidence of PEH is 20% for group A, 16.8% group B, and 12.5% group C. Combining groups B and C, 5 of 25 patients (20%) who underwent fundoplication at less than 1 year of age developed PEH, whereas one of 19 older patients (5.3%) developed PEH. One of 25 patients (4%) who had crural repair at fundoplication developed PEH, whereas 14 of 64 patients without crural repair (21.9%) developed PEH. At surgery, PEH occurred at the left posterolateral aspect of esophagus. We conclude that (1) follow-up after fundoplication should continue for 36 months and include upper GI series; (2) patients under one year of age undergoing fundoplication may be at a higher risk for PEH; and (3) technical refinement including crural repair may be required to prevent PEH.

Neonatal ovarian torsion: report of three cases and review of the literature.

Ovarian cysts are common incidental findings in term infants and, if unusually large, may result in dystocia, torsion, or rupture. Torsion and infarction of a normal ovary tend to occur in older childhood. During a 4-month period, 3 cases of neonatal ovarian torsion were observed after antenatal ultrasonography had detected fetal pelvico-abdominal cystic lesions. The three infants were explored between 4 and 16 days of age. Ovarian torsion was right-sided in all 3, and 1 ovary had been autoamputated. The resected specimens were nontense, thin-walled cysts, filled with hemorrhagic fluid, that measured between 4.5 and 8 cm in diameter. Microscopically, focal calcification and widespread necrosis precluded recognition of underlying histologic landmarks. Neonatal ovarian cysts or cystic ovaries greater than 4 cm in diameter should be excised, even if asymptomatic, because they are prone to, or have undergone, torsion.

Fine needle aspiration cytology of metastatic malignant rhabdoid tumor.

The diagnosis and management of pediatric neoplasms using fine needle aspiration (FNA) has been neglected as compared to neoplasms occurring in adults. A case of metastatic malignant rhabdoid tumor diagnosed by FNA cytology is presented, and the cytologic morphology, which to our knowledge has not previously been reported, is described. The ultrastructural findings in the histologic specimen are also depicted. This case demonstrates the feasibility and need for further exploration of the use of FNA in pediatric oncology.