Congenital Arhinia: A Neonatal Case Report.

Congenital arhinia or nasal absence is a rare condition, with only less than 100 cases published in the literature to date. It is a rare condition that causes respiratory distress during the neonatal period. Although stabilization of the airway is the priority, management is not clearly defined, given the rarity of the malformation. We report a case of arhinia in a female newborn and briefly review the literature.

[Computerized three-dimensional reconstruction of the retrohepatic segment of inferior vena cava of a 20 mm human embryo]. / Reconstruction tridimensionnelle informatisée du segment rétrohépatique de la veine cave inférieure d'un embryon humain de 20mm.

The subdiaphragmatic venous drainage of the embryo is provided by the two caudal cardinal veins to which is added the subcardinal vein system, draining the mesonephros, the perispinal supracardinal veins and the umbilical and vitelline venous system. The anastomosis of certain segments of the embryonic venous structures and the disappearance of others are at the origin of the inferior vena cava. Since the 19th century, three-dimensional reconstruction of solid models from histological sections were developed. At present, the development of computerized three-dimensional reconstruction techniques allowed to operate a multitude of techniques of image processing and modeling in space. Three-dimensional reconstruction is a tool for teaching and research very useful in embryological studies because of the obvious difficulty of dissection and the necessity of introducing time as the fourth dimension in the study of organogenesis. This method represents a promising alternative compared to previous three-dimensional reconstruction techniques including Born technique. The aim of our work was to create a three-dimensional computer reconstruction of the retrohepatic segment of the inferior vena cava of a 20mm embryo from the embryo collection of Saints-Pères institute of anatomy (Paris Descartes university, Paris, France) to specify the path relative to the liver and initiate a series of computerized three-dimensional reconstruction that will follow the evolution of this segment of the inferior vena cava and this in a pedagogical and morphological research introducing the time as the fourth dimension.

High tie versus low tie vascular ligation of the inferior mesenteric artery in colorectal cancer surgery: impact on the gain in colon length and implications on the feasibility of anastomoses.

BACKGROUND: There is no demonstrated benefit of high-tie versus low-tie vascular transections in colorectal cancer surgery. OBJECTIVE: The aim of this study was to compare the effects of high-tie and low-tie vascular transections on colonic length after oncological sigmoidectomy, the theoretical feasibility of colorectal anastomosis at the sacral promontory, and straight or J-pouch coloanal anastomosis after rectal cancer surgery with total mesorectal excision. DESIGN: This study is an anatomical study on surgical techniques. SETTINGS: This study was conducted in a surgical anatomy research unit. PATIENTS: Thirty fresh nonembalmed cadavers were randomly assigned to high-tie and low-tie groups (n = 15). INTERVENTIONS: Oncological sigmoidectomy followed by total mesorectal excision was performed. MAIN OUTCOME MEASURES: The distances from the proximal colon limb to the lower edge of the pubis symphysis were recorded after each step of vascular division. RESULTS: The successive mean gains in length in high-tie vs low-tie vascular transections were 2.9±1.2 cm vs 3.1 ± 1.8 cm (p = 0.83) after inferior mesenteric artery division, 8.1 ± 3.1 cm vs 2.5 ± 1.2 cm (p = 0.0016) after inferior mesenteric vein division at the lower part of the pancreas, 8.1 ± 3.8 cm vs 3.3 ± 1.7 cm (p = 0.0016) after sigmoidectomy. The mean cumulative gain in length was significantly higher in high-tie vs low-tie vascular transections (19.1 ± 3.8 vs 8.8 ± 2.9 cm, p = 0.00089). After secondary left colic artery division, the gain in length was similar to that of the high-tie group (17 ± 3.1 vs 19.1 ± 3.8 cm) (p = 0.089). Colorectal anastomosis at the promontory and straight and J-pouch coloanal anastomosis feasibility rates were 100% in the high-tie group, 87%, 53%, and 33% in the low-tie group, but 100%, 100%, and 87% after secondary left colic artery division. LIMITATIONS: This anatomical study, based on cadavers rather than live patients, does not evaluate colon limb vascularization. CONCLUSIONS: The gain in colonic length is 10 cm greater for high-tie vascular transections. With low-tie vascular transections, high inferior mesenteric vein division produced a small additional gain in length, and secondary left colic artery division produced the same length gain as high-tie vascular transections.

[Ocular findings in Alport syndrome: 32 case studies]. / Atteinte oculaire au cours du syndrome d'Alport. A propos de 32 cas.

BACKGROUND: Alport syndrome is an inherited disease resulting in kidney failure, hearing loss, and ocular abnormalities. The purpose of this study was to describe the incidence and type of ocular abnormalities and to determine inheritance of this syndrome in our population. PATIENTS AND METHODS: A total of 32 patients, from ten different families in South Tunisia, underwent a complete ocular examination. Inheritance was determined using pedigrees and genotyping. RESULTS: The best corrected visual acuity was 7.6/10. Biomicroscopy showed polymorphous dystrophy in 3%, anterior lenticonus in 28%, lens opacities in 3%, cataract in 19%, and retinal flecks in 37%. The genetic survey found five families with X-linked Alport syndrome, four families with recessive autosomal disease, and one family with dominant autosomal disease. DISCUSSION: Ocular abnormalities have been reported in 9%-82% of Alport syndrome patients. They are rare in childhood and increase in frequency and severity with age. The types of ocular defects described mostly involve the lens, the retina and more rarely the cornea. The most common changes are anterior lenticonus and perimacular retinal flecks. In approximately 85%, Alport syndrome is X-linked. In the remaining 15%, the transmission is autosomal recessive and exceptionally autosomal dominant. CONCLUSION: Ocular examination is a precious help for Alport syndrome diagnosis. It can also determine the prognosis of nephropathy.

[Ocular manifestations associated with nephronophthisis and genetic study in three Tunisian families]. / Analyse des manifestations oculaires associées à la néphronophtise et étude génétique chez trois familles tunisiennes.

PURPOSE: Nephronophthisis is a familial interstitial nephropathy with an autosome recessive mode of transmission. In some cases, it is associated with ocular manifestations such as retinitis pigmentosa in Senior-Løken syndrome. We report ocular abnormalities and genetic results in three affected Tunisian families. PATIENTS AND METHODS: Twenty-two members of these three families underwent a complete ophthalmologic examination (visual acuity, slit lamp biomicroscopy, ophthalmoscopy, and retinal electrophysiology). For genetic study, all individuals were genotyped and underwent a genomic sequence. RESULTS: Twenty-two subjects, nine of whom presented nephronophthisis, were included in this study. Retinitis pigmentosa was found in three cases. Our genetic study demonstrated that patients belonging to family 1 had homozygous deletions in NPHP1, all affected individuals from family 3 were linked to NPHP4 and presented a deletion in exons 2 and 3. Results are pending for patients in family 2. CONCLUSION: Senior-Løken syndrome is a rare hereditary disease that combines familial juvenile nephronophthisis and retinitis pigmentosa. This association was described in the literature in 39%-43% of cases. In our study, it was approximately 33% of cases. The genetic study can sometimes obviate the need for renal puncture, especially when the homozygous deletion of NPHP1 gene is confirmed.

[Recurrent toxoplasmic retinochoroiditis after clindamycin treatment]. / Récurrence de la rétinochoroïdite toxoplasmique après traitement par clindamycine.

PURPOSE: To evaluate the efficacy and safety of subconjunctival injections of clindamycin associated with oral corticotherapy in the treatment of toxoplasmic retinochoroiditis (TRC) and its effect on recurrence. PATIENTS AND METHODS: This study investigated 21 patients (10 males and 11 females) who presented to an ophthalmological department from 1995 to 2000, aged 14-55 years (mean, 28 years), with unilateral TRC. They were treated with subconjunctival injections of clindamycin and systemic corticotherapy at a dose of 1 mg/kg/day. Clinical follow-up consisted of assessment of visual acuity and biomicroscopic fundus examination, completed by retinal angiography if necessary. RESULTS: We observed clinical improvement in 68.75% of patients within 4 days and in all patients healing of the chorioretinal area within 1.6 months. During the follow-up period ranging from 6 to 52 months (mean, 22 months), there have been two recurrences (9%) over the first 18 months. Tolerance of the injections was good. Serious side effects were noted in two patients: one case of conjunctival necrosis and one case of corneal and conjunctival erosion. CONCLUSION: In treating ocular toxoplasmosis, clindamycin appears to be a safe and effective substitute for the classic protocol (pyrimethamine-sulfadiazine, pyrimethamine-azithromycin), for which a 50% recurrence rate has been reported. It provides relatively good protection against recurrence and warrants testing in further controlled studies.

Radio-anatomic study of the pancreatic duct by MR cholangiopancreatography.

To compare the performance of MR-cholangiopancreatography (MRCP) and that of classical anatomy in the depiction of the main pancreatic duct, 50 MRCP examinations were done in patients free of pancreatic disease. Axial and coronal sections 20 mm thick were obtained in a Single Shot Fast Spin Echo (SSFSE) sequence. The following were analyzed: (1) visibility of pancreatic duct structures, (2) form of the main pancreatic duct, (3) various angulations of the duct and (4) diameter of the duct. Anatomic variants were noted. These findings were compared with anatomic and radio-anatomic (ERCP) data in the literature. The main pancreatic duct was visualized in 100% of cases and the accessory pancreatic duct in 61%. The form, diameter and angulations of the various segments of the pancreatic duct were similar to those reported in the literature. These findings are reported in the axial and coronal planes. Comparison with major anatomic classifications was not possible. MRCP enables in vivo anatomic exploration of the main pancreatic duct. Horizontal sections provided new radio-anatomic information. The technique nevertheless remains limited by poor spatial resolution.

Double hepatomesenteric artery: a rare anatomical variant.

A case of a "double hepatomesenteric artery", i.e. two persisting hepatic arteries arising from the superior mesenteric artery, is reported. Also observed was a lack of systematisation of the pancreaticoduodenal arteries. The variant, which does not appear to have been previously reported in the literature, was observed when dissecting the vessels of the duodenal bulb in a preserved male cadaver injected with neoprene latex. Its embryological origin, as well as the clinical, radiological (diagnostic or interventional) and surgical implications, are discussed.

Arterial supply of the duodenal bulb: an anatomoclinical study.

An anatomic and radio-anatomic study of 15 specimens enabled us to reconsider the arterial vascularization of the duodenal bulb and to propose a new classification based on anatomoclinical criteria. The two arterial pedicles (infra- and supraduodenal) reach the bulb on its posterior aspect; each pedicle is made up of two sorts of blood currents (right and left); the posterior aspect of the bulb seems to be the most vascularized one, explaining, apart from bleeding from gastroduodenal a. erosion, the hemorrhagic character of ulcers of the posterior aspect of the bulb. The predominance of the left-hand currents explains the possible ischemia of the duodenal bulb and/or rupture of the duodenal stump after their interruption.

Anatomic study of the deep plantar artery: potential by-pass receptor site.

The deep plantar artery is the main anastomosis between the dorsal and plantar arterial systems of the foot; with no change of calibre it unites the dorsalis pedis artery to the deep plantar arch, the second segment of the lateral plantar artery, passing into the first intermetatarsal space. Our anatomic study of 20 dissections of the deep plantar artery allowed us to verify the near-constancy of its descriptive anatomy and relationships. The deep plantar artery, when present, only found in 16 of our 20 cases, is totally linked to the dorsalis pedis artery. Its easy accessibility via the dorsal route, its medium calibre and the absence of collateral branches make this artery a possible anatomic site for the performance of a distal bypass, the last option before an amputation of the foot becomes necessary, although such a case remains exceptional.

Anatomy of the arterial vascularization of the lips.

The findings from 12 dissections of previously injected facial masks, 8 dissections of the face following intraarterial injection of a red solution of Latex Neoprene, and a corrosion cast specimen allowed us to study the arterial supply of the lips. The arterial supply of the upper lip arises mainly from the superior labial arteries, but also from the subseptal arteries and from the subalar arteries. There is a figure of 8 shaped anastomotic system between these arteries lying on the upper lip. The arterial supply of the lower lip arises from the inferior labial arteries and from branches of the mental artery. A constant inferior labial arterial network was shown at the level of the lower lip arising in a fifth of cases from a T-shaped inferior labial artery. All these recent anatomic findings help us to improve our understanding of plastic surgery of the lips.

An anatomical study of the motor distribution of the mandibular nerve for a masseteric-facial anastomosis to restore facial function.

On account of the complex anatomy at the base of skull, surgery here may result in post operative cranial n. deficits. Facial palsy is often feared and its effects upon the patient's psychological and emotional well-being can be catastrophic. The modest results and the side effects of the facio-hypoglossal anastomosis used for facial rehabilitation have led us to consider an anastomosis between a motor branch of the trigeminal n. and the facial n. Dissection has allowed us to demonstrate that the masseteric n. offers the characteristics and the relationships which should make such an anastomosis feasible.

Predictibility of the cervical origin of the anterior spinal artery.

SUMMARY: The anatomic study (under a surgical microscope) of 25 brains that had previously been injected with colored latex confirmed the fact that in the majority of cases, the anterior spinal artery results from the merging of two arterioles which both derive from two vertebral arteries. This bilateral nature is determined by the symmetric origin of the posterior inferior cerebellar arteries. When the conformation is asymmetrical, a single anterior spinal artery branches off from the vertebral artery that supplies the closest posterior inferior cerebellar artery.