Shaking infant trauma induced by misuse of a baby chair

A 2 month old infant presented with bilateral subdural haemorrhages and bilateral subhyaloid haemorrhage. The parent admitted to forceful bouncing of the child in a baby rocker. Experiments showed that violent rocking in the chair could produce extreme alternating acceleration/deceleration forces in excess of those induced by shaking alone. Such handling could not be interpreted as accidental mismanagement and the abusive nature of the process was graphically shown in video recordings of the experiment. Prosecution resulted in a conviction for cruelty, and a suspended sentence.     

隐睾儿童血清苗勒管抑制物质的研究

目的探讨苗勒管抑制物质(MIS)在隐睾的诊断以及睾丸发育评价中的作用。方法选择54例单纯性隐睾患儿为观察组,36例无生殖器病变的儿童为对照组,分别检测两组儿童血清MIS水平,并比较两者之间的差异。结果观察组与对照组按年龄分组,各组间年龄比较,差异无统计学意义(P>0.05)。观察组血清MIS为48.1±26.2 ng/ml,对照组为76.3±22.8 ng/ml,两组比较,差异有统计学意义(P<0.05)。双侧隐睾患儿血清MIS为39.2±28.3 ng/ml,单侧隐睾患儿为56.5±30.1 ng/ ml,两者比较,差异有统计学意义(P<0.05)。MIS水平与睾丸发育情况呈正相关(r=0.35,P<0.05),与治疗年龄相关(r=0.19,P<0.05)。结论MIS可作为反映隐睾患儿睾丸发育状况的一项重要指标。     

Bilateral pheochromocytoma. Is there stimulation of an extra-adrenal adrenaline liberation after bilateral adrenalectomy?]

BACKGROUND: In order to determine plasma and urine epinephrine levels after bilateral adrenalectomy, we examined a 16 year old young man with bilateral pheochromocytoma. Bilateral removal of the adrenals was performed because of a left sided pheochromocytoma relapse. An extra-adrenal pheochromocytoma could be excluded by scintigraphy. Macroscopically all adrenal tissue was removed during surgery. METHODS: A modified radioenzymatic determination of the free catecholamines epinephrine, norepinephrine and dopamine was used according to Peuler and Johnson. Sulfoconjugated catecholamines were measured after addition of 60 microU arylsulfatase type VI. Urine catecholamine levels were determined fluorometrically. RESULTS: The elevated plasma andurine norepinephrine levels before surgery returned to normal after surgery. In contrast, plasma epinephrine levels returned to subnormal values thereafter but increases 5-fold at the end of anesthesia suggesting an extra-adrenal source of epinephrine. Urine epinephrine levels remained in the lower normal range. An insulin induced hypoglycemia was performed resulting in adrenergic symptoms of hypoglycemia and a subnormal increase of epinephrine. CONCLUSIONS: We conclude, that epinephrine mediated physiological regulations occur inspite of bilateral adrenalectomy probably by a regulated extra-adrenal source of epinephrine. The kidney could be the site of extra-adrenal epinephrine production.     

Testicular volume and function in adolescence after breech delivery

Testicular volume and pubertal development were investigated in 108 boys born by breech delivery aged 14 1/2 to 19 1/2 years. Mean testicular volume was slightly but not significantly lower than in a control group of the same age. Pubertal development was normal. One boy had unilateral and two boys bilateral testicular atrophy (one with azoospermia and one with oligospermia). In a control group of 135 boys delivered from vertex position ther were three cases with unilateral and none with bilateral testicular atrophy. In both groups all cases with unilateral testicular atrophy could be explained by postnatal testicular injury, whereas the two cases of bilateral atrophy in the group of breech delivered boys remained unexplained. They may have originated from local trauma during breech delivery. It is concluded that testicular atrophy as a complication of breech delivery is seen with a frequency of only 2% or less.     

Congestive heart failure, hypertension, and hyperreninemia in bilateral Wilms' tumor: successful medical management

Congestive heart failure is an unusual complication of the hyperreninemia of Wilms' tumors. Cases with bilateral tumors present a difficult management problem. This is a report of the successful medical management of a child with congestive heart failure secondary to hyperreninemia from bilateral Wilms' tumor. Hypertension and hyperreninemia were extensively documented. Their etiologic relation to the congestive heart failure was supported by the patient's improved cardiac function following specific renin-angiotensin blockade. With unilateral tumors, surgical excision corrects the hypertension; however, with large bilateral tumors, excision is out of the question. A unique feature of this case is the ability to control the blood pressure with saralasin. With subsequent antitumor therapy, renin concentrations decreased proportional to tumor size, and renin angiotensin blocking therapy could be discontinued.     

Epididymal and vasal abnormalities in undescended testes and azoospermia

Epididymal and vasal abnormalities (EVA) have long been known to be associated with cryptorchidism. In order to provide data concerning the incidence of EVA in undescended testicles, we operatively examined the anatomic relationship of the epididymis, vas, and testis in 456 cryptorchid patients (390 unilateral and 66 bilateral, over-all 522 undescended testes). As a control group we examined 50 adults postmortem and 96 pediatric patients operated upon for inguinal hernia or hydrocele. We divided the operative findings into two groups: simple variants of normal, and forms of complete anatomic disconnection of the spermatic ducts.We were unable to find any anatomic disconnection in either control group. In contrast, in 99 of the 522 maldescended testes operated upon (19%) we found some form of anatomic disconnection along the proximal spermatic ducts. The incidence was 17% in unilateral cryptorchidism and 26% in bilateral cases, in 16% of whom the EVA was bilateral. According to the literature and our previous study, azoospermia is present in about 18%–20% of adults operated upon for bilateral cryptorchidism. Our present study may suggest that in bilateral cryptorchid patients who were operated upon in the pediatric age range, the azoospermia in adulthood could be partially related to some form of bilateral occlusion or interruption of the spermatic ducts. Correspondence to: G. Belloli     

Reproductive risk factors in unilateral and bilateral renal agenesis

ABSTRACT  Renal agenesis (RA) appears to be a multifactorial condition with combined genetic and environmental influences. We performed a retrospective case-control study of reproductive history of 26 isolated RA live births cases referred to Sicilian Registry of Congenital Malformations. A statistical significant association for birth weight if we considered all RA together and for bilateral RA alone, an increasing risk for maternal age only in the bilateral RA subgroup and a male predominance both for unilateral and bilateral RA was found. Our results show that some reproductive risk factors may be associated with RA, moreover differences found between subgroups indicate that some risk factors may be different in unilateral and bilateral RA. The association between reproductive risk factors and RA may reflect pathogenetic interaction between genetic and environmental factors. Nevertheless further studies are needed to clarify these associations and to explore the role of perinatal factors in the etiology of renal agenesis. In fact if prenatal or perinatal risk factors are in a causal chain influencing the risk for developing RA, then these data could have important implications in the prevention or treatment of this condition.     

Pneumoperitoneum in the ventilated infant

Pneumoperitoneum was diagnosed in a 3-month-old infant following bilateral diaphragmatic hernia repair. The patient was ventilated and treated for bilateral pneumothorax with underwater seal drainage. Percutaneous drainage of the pneumoperitoneum was carried out. Oxygen partial pressure in the drained air was used to determine the origin of the pneumoperitoneum, which could be lung or a perforated viscus. Continuation of the drainage over more than 24 h served to alleviate the added respiratory insult caused by abdominal distension. Continuous percutaneous peritoneal drainage can serve both diagnostic and therapeutic purposes in selected patients. Offprint requests to: R. Udassin     

Massive bilateral nephroblastomatosis in a 13-year-old girl

A 13-year-old girl on chronic hemodialysis with renal failure thought to be due to polycystic renal disease, underwent bilateral nephrectomy as a pretransplant procedure. Microscopic examination of the grossly enlarged nodular kidneys revealed a bilateral diffuse tumor infiltration which was not Wilms tumor. Eventually the diagnosis of bilateral nephroblastomatosis was established. This is apparently unique at this age without coexistent Wilms tumor. Four months after nephrectomy metastases-exceedingly rare in nephroblastomatosis-developed. Local radiation and cytostatic therapy with Actinomycin D, Vincristine and Adriamycin were initiated. All drugs were administered 24 h before the beginning of hemodialysis; only Actinomycin D was reduced to 70% of the usual dosage. Therapeutic side effects remained within the usual limits. Renal transplantation was performed 34 months after metastases had developed, i.e. 10 months after cessation of cytostatic therapy.Massive bilateral nephroblastomatosis can also occur in older children and can easily be mistaken for polycystic renal discase. It's early clinical and microscopic recognition could enable appropriate management, which should consist of open biopsy, chemotherapy and scrupulous follow-up procedures, rather than aggressive therapy. The latter probably had to be administered in this patient, initially uremic and on chronic hemodialysis.     

Urological anomaly in an infant with Soto's syndrome: report of a case

OBJECTIVE: To discuss the diagnosis and the etiology of a bilateral urologic anomaly in a patient with Soto's Syndrome (cerebral gigantism). To alert the pediatric physicians and urologists about the coexistence of these two malformations. RESULTS: A three month old boy, born of a 35 week uncomplicated first pregnancy by cesarian, with phenotypic, radiologic and metacarpophalangeal profile of Soto's Syndrome was admitted to the Hospital because of hyperpnea, tachycardia and 39o C recurrent fever. The culture of urine disclosed 500.000 col/ml. Cistouretrographic study showed bilateral megaloureter and hydronephrosis. Vesico-ureteral bilateral reflux was diagnosed and antibiotic therapy was administered. Patient's follow up was excellent and was discharged taking preventive nitrofurantoin. CONCLUSIONS: As in other cases of urinary tract infection, pediatric physicians must consider urologic anomalies in children with cerebral gigantism who present the clinical presentation of Soto's Syndrome. Autonomic Nervous System development failure in ureteral muscles could be an alternative etiology.     

Course and outcome of otitis media in early infancy: a prospective study

We determined the course of otitis media in a prospective, longitudinal study of infants who were enrolled at birth and followed to age 1 year. Bilateral chronic otitis media with effusion developed in eight of 24 (33%) with onset of otitis media before age 2 months, compared to two of 30 (7%) with later onset (P = 0.012). Infants with bilateral chronic otitis media with effusion could be identified early: eight of 15 (53%) infants with bilateral middle ear effusion at age 2 months subsequently had bilateral chronic otitis media with effusion, compared to two of 55 (4%) infants without bilateral effusions at age 2 months (P = 0.000007). The onset of otitis media was symptomatic in 29 of 54 (54%), and asymptomatic in 25 of 54 (46%). If regular well-child examinations with otoscopy had not been performed in asymptomatic infants, bilateral chronic otitis media with effusion would not have been detected in six of 10 infants. Infants with otitis media in early infancy may be asymptomatic and are at high risk for chronic otitis media with effusion.     

Diagnosis of veno-occlusive disease of the liver by color-coded Doppler sonography

In a girl with bilateral Wilms' tumor veno-occlusive disease occurred during chemotherapy. The diagnosis was established by reversed flow in the portal vein displayed by color and pulsed Doppler sonography. After successful conservative treatment normalization of flow in the portal vein could be shown by both Doppler techniques.     

双侧发育性髋关节脱位一期Salter骨盆截骨术

目的探讨双侧发育性髋关节脱位一期Salter骨盆截骨术的安全性和近期疗效。方法双侧同时行Salter骨盆截骨术的发育性双侧髋关节脱位患儿48例（96髋）,为观察组,取同期单侧分次Salter骨盆截骨术患儿66例（76髋）,为对照组。比较两组术后关节功能、髋臼的x线表现、并发症发生率等。结果随访2年至5年6个月,按Mckay髋关节功能评价标准,观察组术后优良率为96．88％,对照组为98．68％;按Severin标准评定x线表现,病例组术后优良率为94．79％,对照组为97．37％;股骨头无菌性坏死率观察组为4．17％,对照组为3．95％;髋关节功能不良的发生率在观察组及对照组分别为3．13％和1．32％。两组各项指标比较均无统计学意义。结论双侧同时行Salter骨盆截骨与单侧Salter骨盆截骨疗效相似,双侧Salter骨盆截骨治疗发育性双侧髋关节脱位安全有效。远期疗效有待进一步观察。     

Bilateral frontal polymicrogyria and Ehlers-Danlos syndrome.]

The authors describe the case of a six-year-old girl with Ehlers-Danlos syndrome associated to bilateral symmetrical frontal polymicrogyria. Several extracellular matrix components, including collagen, are directly implicated in the neuronal migration. We think that a defect in collagen or in another extracellular matrix protein during fetal development could result in this association.     

Bilateral, multifocal orbital cysticercosis

Orbital cysticercosis is a benign infection with a variety of clinical presentations. Awareness of all of the possible clinical manifestations could aid early diagnosis and prevention of late sequelae. We report a case of bilateral, multifocal, intraconal orbital cysticercosis, which is an extremely rare presentation.     

Thromboangiitis obliterans and distal gangrene in a 5-year-old child

Distal gangrene associated with arterial hypertension but without systemic manifestations or muco-cutaneous eruptions was noted in a 5 years old child. Gangrene first appeared in toes and then in fingers. Progression in a 7 months period led to bilateral midleg amputation and amputation of several fingers. Angiography revealed complete obliteration of popliteal and humeral arteries by thrombi and histologic examination of amputated material showed organized thrombi with phlebothrombosis and without vasculitis. Biopsies of liver, lung, kidneys and skin were unremarkable and immunofluorescence studies with various antisera were negative. Also no contributing factor or etiologic agents were found, similarities to adult type Buerger's disease are striking. Vasodilators were of no help and bilateral lumbar and cervical sympathectomy could only demarcate the already present gangrene.     

Oral converting enzyme inhibitor in malignant hypertension.

Malignant hypertension, which developed in a 9-year-old boy after an episode of haemolytic uraemic syndrome, could not be controlled with antihypertensive agents. However, treatment with oral converting enzyme inhibitor (captopril) was effective in controlling the blood pressure and it averted bilateral nephrectomy. No adverse effects from the drug were noted.     

Vascular air embolism: A rare complication of nasal CPAP

Abstract: A preterm infant developed bilateral tension pneumothoraces and extensive vascular air embolism 6 h after being commenced on nasal continuous positive airway pressure (CPAP). Neonatal clinicians should be aware that catastrophic vascular air embolism could occur in infants receiving nasal CPAP, a modality of respiratory support conventionally considered non-invasive and 'safe'.     

Neonatological and pulmonological management of bilateral pulmonary sequestration in a neonate

Background  Bronchopulmonary sequestration is a lung malformation characterized by nonfunctioning lung tissue without primary communication with the tracheobronchial tree. Intrauterine complications such as mediastinal shift, pleural effusion or fetal hydrothorax can be present. We present the case of a newborn with bilateral intralobar pulmonary sequestration. Methods  Prenatal ultrasonography in a primigravida at 20 weeks of gestation revealed echogenic masses in the right fetal hemithorax with mediastinal shift towards the left side. Serial ultrasound confirmed persistence of the lesion with otherwise appropriate fetal development. Delivery was uneventful and physical examination revealed an isolated intermittent tachypnea. Chest CT scan and CT angiography showed a bilateral intrathoracic lesion with arterial supply from the aorta. Baby lung function testing suggested possible multiple functional compartments. Results  Right and left thoracotomy was performed at the age of 7 months. A bilateral intralobar sequestration with vascularisation from the aorta was resected. Pathological and histological examination of the resected tissue confirmed the surgical diagnosis. At the age of 24 months, the child was doing well without pulmonary complications. Conclusions  Bilateral pulmonary sequestration requires intensive prenatal and postnatal surveillance. Though given the fact of a bilateral pulmonary sequestration, postnatal outcome showed similar favourable characteristics to an unilateral presentation. Baby lung function testing could provide additional information for optimal postnatal management and timing of surgical intervention.     

Isolated hypoglossal paralysis

A 5 year old girl suffering from chronic tonsillitis and bilateral tympanic effusion developed atrophy of the right half of the tongue. With the exception of the twelfth cranial nerve (right) no neurological signs could be detected. We are reporting a case of an isolated hypoglossal nerve paralysis.