Idiopathic disorders of fecal continence in children.

A group of 176 patients aged 2 to 15 years was investigated for idiopathic disorders of bowel function other than Hirschsprung's disease. Anorectal motility, as well as colorectal transit of radiopaque markers, were investigated. Before the end of the first month of life, 70 of the patients were constipated. Resting pressure was more unstable (P less than 0.001) and higher than normal in the rectal ampulla and upper anal canal (P less than 0.01). Retardation of markers occurred in the proximal and/or distal large bowel of 61% of the patients. The existence of functional abnormalities was demonstrated in the majority of children with idiopathic disorders of fecal continence.

Testicular descent: when to interfere?

Spontaneous descent of testes after birth can occur in up to 70% of cases, yet the factors contributing to it are still controversial. This study aims to evaluate factors contributing to spontaneous descent of palpable undescended testes. Eighty-four newborns with 126 palpable undescended testes (42 unilateral and 42 bilateral) were followed up for a period of one year to study the occurrence and time of testicular descent and its relation to gestational age, birth weight, uni- or bilaterality and levels of FSH, LH and testosterone. A total of 58 testes (46%) descended between 3 and 6 months. Spontaneous descent occurred in 10 premature patients (14 testes 63%) compared to 44 testes of full-term patients (43%). Descent occurred in 14 unilateral undescended testes (33%) compared to 44 (52%) in bilateral cases. In patients with spontaneous testicular descent there was postnatal peak of LH and testosterone at 2 to 3 months of age which returned to basal level at 6 months of age. In patients with permanent undescended testes the peak of LH and testosterone was very low and almost absent in some of them; no significant difference was found between the mean values of FSH in both groups. No spontaneous testicular descent occurred after the 4th month in the full-term group, whereas in the pre-term group spontaneous descent occurred up to 6 months of age. This study concluded that spontaneous descent of palpable undescended testes is closely related to the presence of LH and testosterone surge. Therapy of undescended testes should start at 4 months of age in a full-term baby and at 6 months of age in a pre-term baby.

[Prenatal diagnosis of congenital cystic adenomatoid malformation of lung: perinatal management. Apropos of 4 cases]. / Malformation kystique adénomatoïde pulmonaire congénitale de diagnostic anténatal: prise en charge périnatale. A propos de quatre observations.

BACKGROUND: Cystic adenomatoid malformation of the lung can be seen by ultrasonography during pregnancy. Surgical excision of the affected lobe is indicated during the first days of life. CASES REPORT: Four neonates were admitted to an intensive care unit from March 1988 to February 1992, due to cystic adenomatoid malformation of the lung, that had been diagnosed by ultrasonography at 19, 22, 34 and 37 weeks of gestational age, respectively. These malformations were not associated with other abnormalities and were type I (three cases) and II (one case) according to Stocker's classification. Only one patient became symptomatic, requiring intubation by 72 hours of age. Surgical excision of the affected lobe was performed in three patients at 4 hours, 2 and 7 days of life, respectively, with a normal long-term survival. A segmental resection was performed at 5 days of life in the remaining symptomatic patient but persistence of cystic lesions required lobectomy at 10 months. CONCLUSIONS: Early perinatal management of cystic adenomatoid malformations of the lung is necessary as surgical excision is indicated as soon as possible, even in asymptomatic patients.

[Clinical heterogeneity of Townes-Brocks syndrome]. / Hétérogénéité clinique du syndrome de Townes-Brocks.

BACKGROUND: Townes-Brocks syndrome (TBS) is a rare autosomal dominant entity mainly characterized by ano-rectal, ear and extremities abnormalities with variable clinical expression. CASE REPORTS: The first case had ear and extremities, but not anorectal, abnormalities; a Pierre-Robin sequence with esophageal atresia was also observed. The second case had the classical triad of abnormalities also associated with tetralogy of Fallot which has been only once reported in the literature. CONCLUSIONS: Both cases are other examples of the frequent clinical variability observed in this syndrome explaining diagnostic difficulties in the absence of a specific marker.

[Anorectal traumas by impalement in children. Apropos of 3 cases]. / Les traumatismes ano-rectaux par empalement chez l'enfant. A propos de trois observations.

Traumatic impalement is an exceptional cause of anorectal lesions in the child. The clinical diagnosis is sometimes difficult and associated lesions raise the problem of the most appropriate exploratory examination. Based on our experience with three cases, we would emphasize the usefulness of hydrosoluble enema study prudently performed by the surgeon during the preoperative work-up. The precise localization of the lesions must be obtained with careful emergency exploration before repair. Treatment of the trauma includes lavage, drainage and sometimes colostomy. Antibiotics must always be prescribed. Early complications are dominated by infection and long-term sequellae, requiring a long follow-up.

[Congenital obstructive valves and diverticula of the anterior urethra]. / Valves et diverticules congénitaux obstructifs de l'urètre antérieur.

Obstructive lesions of the anterior urethra (valves, saccular diverticula) are rare, and can be difficult to diagnose. Three recent cases have led us to review the existing french and english literature and we have found case histories for 81 cases, encountered over a twenty year period. The anatomic interpretation of the lesions is far from being univocal; however many authors clearly distinguish between valves and diverticula, the basic difference residing in the contiguity between the anomaly and the corpus spongiosum. Where the clinical presentation depends on age, the diagnosis depends essentially on the voiding cysto urethrography, which must objectify the whole of the urethra. Generally speaking the treatment for the valves is simple, consisting in endoscopic resection. In the case of diverticula, it is not always necessary or desirable to remove the diverticulum itself; if there is a well-formed distal obstructing lip, removing it may be enough to cure the obstruction.

[Primary intussuception of appendix (author's transl)]. / Invagination primaire de l'appendice.

A 2 years old female presents abdominal pain and rectal bleeding roentgenological and endoscopic investigations lead to the diagnosis of intussusception of the appendix. Surgically resected, the clinical course was uneventful and the patient promptly discharged. Primary intussusception of the appendix is a rare event (less than 200 cases recorded since 1858). The particularity of it, according the age of the patient, the clinical symptoms, and the endoscopic diagnosis are emphasized.

[Results of the treatment of acute intestinal invaginations by radiopaque enemas. Apropos of 70 cases]. / Résultat du traitement des invaginations intestinales aiguës par lavement opaque. A propos de 70 cas.

Since May 1982, when the pediatric surgery group of Rouen has published their study in the 39th congress of "Societe Française de Chirurgie Infantile", regular surgical attitude in treatment of intussusception was abandoned by our team. Seventy cases were admitted in our section in the period (06-82/06-86), age ranged from one month to nine years. All except one were subjected to radio-opaque enema as a method of treatment with success rate of 89% reduction. Optimum conditions, failures (7 case), perforation (one case) and recurrence are presented. We conclude the efficacy of this treatment in condition of a rigorous technic.

[Constipation in the infant (author's transl)]. / Les constipations de l'enfant.

The authors studied 102 cases of constipation in children. 76 of them went through a protocol of investigations : clinical, psychological, radiological and manometric. The authors suggest that so-called functional constipation is more often organic than is commonly thought.

[Digestive hemorrhage and bacterial overgrowth in children]. / Hémorragie digestive et pullulation bactérienne chez l'enfant.

Two cases of gastrointestinal bleeding due to bacterial overgrowth syndrome are presented. The microbial contamination was confirmed by quantitative studies of the intestinal microflora and by therapeutic test; the association colistin-metronidazole could stop the digestive hemorrhage.

Quantitative analysis of anorectal pressures in Hirschsprung's disease.

Anorectal motility was investigated in 146 children with Hirschsprung's disease and 89 normal control subjects. Pressures were recorded in the rectum and anal canal at rest and during rectal distention. The rectoanal inhibitory reflex was absent in all but four patients. Intraluminal rectal pressure was higher than normal (16.5 vs. 14.6 cm H2O, P less than 0.03), with more frequent (41 per cent vs. 18 per cent, P less than 0.01) pressure waves. In the upper anal canal, there were more frequent (62 per cent vs. 18 per cent, P less than 0.001) spontaneous variations of pressure of lower frequency (9.5 vs. 12.8 cycles/min P less than 0.001) and greater amplitude (5.2 vs. 3.6 cm H2O, P less than 0.001) than normal. The rectoanal contractile reflex occurred in 47 per cent of the patients but in only 21 per cent of the control subjects (P less than 0.001). Aganglionosis was associated with the presence of a rectoanal inhibitory reflex in three patients. This study confirms the value of anorectal manometry in diagnosing Hirschsprung's disease in a large group of patients, and demonstrates other abnormalities that may be useful in cases in which histologic and manometric data are in conflict.

Measuring the anorectal angle.

Lateral radiographs of the rectum and anus were taken in 90 children with chronic idiopathic constipation. A computer program was developed to obtain an objective quantitative assessment of the anorectal angle; it averaged 120 degrees at rest, decreased during forceful anal contraction and increased during defecation. It was noted that 24% of the children, when asked to defecate, decreased their anorectal angle as normally observed during forceful anal contraction and, that 21% when asked to contract the anus, increased the angle as if they were defecating. This 'sphincteric disobedience' is probably of major clinical relevance to an understanding of the symptoms of these patients.

[Epiphyseal fracture-avulsion of the distal extremity of the clavicle. Apropos of a case]. / La fracture-décollement épiphysaire de l'extrémité distale de la clavicule. A propos d'une observation.

Fracture of the clavicle is the commonest fracture in children. It is generally due to direct trauma to the shoulder. Orthopaedic usually ensures consolidation within three weeks. Fractures of the lateral third is uncommon and unusual. It corresponds to an epiphyseal avulsion of the distal extremity of the clavicle, equivalent to an acromioclavicular "pseudodislocation". The coracoclavicular ligaments remain intact. Surgical reduction of the clavicle into its periosteal sheath is necessary. The authors report a case of fracture of the lateral third of the clavicle in a 5 and a half year old child with spontaneous formation of a "neoclavicle".

[Giant cell tumor of the tendon sheath of the long extensor muscle of the thumb in a 7-year-old child]. / Tumeur à cellules géantes de la gaine du tendon du long fléchisseur du pouce chez une enfant de 7 ans.

The authors report the case of a giant cell tumour of the flexor pollicis longus tendon sheath in a child. This benign tumour, usually observed in women between the ages of 30 to 50 years, has a highly controversial aetiopathogenesis; the existence of initial trauma is found in 50% of cases. The present case concerns a 7-year-old girl with swelling of the right thumb for two years. Surgical resection established the definitive diagnosis by histological examination of the specimen. The follow-up is currently two years, with no local recurrence.