OBJECTIVETo discuss the treatment and diagnosis of congenital median perineal cleft in children.

METHODSBetween January 2009 to February 2013, 7 cases were diagnosed as congenital median perineal cleft according to the symptoms. Among them, 4 cases underwent surgery to correct cleft with double triangular perineal flaps. The other 3 cases with minor cleft did not receive surgery management.

RESULTSThere is an median cleft from the perineum to the anus with mucosa on the cleft surface. Primary healing was achieved in all the four patients with satisfactory appearance. The patients were followed up for 1-4 years with almost normal perineal appearance.

CONCLUSIONSCongenital perineal median cleft can be diagnosed according to the symptoms. Double triangular perineal flaps can be effectively correct the cleft to attain normal perineal appearance.

Adolescent ; Anal Canal ; Child ; Humans ; Perineum ; abnormalities ; surgery ; Surgical Flaps ; Treatment Outcome ; Wound Healing

Objective To investigate the relationship between the nerve growth factor ( NGF ) induced hippocampal neuroregeneration and homeobox gene Lhx 8.Methods Seventy-two SD rats were divided into control group , transected group, NGF group, transected combined with NGF group after right fimbria-fornix transection and NGF intracerebroventricular injection . Real-time PCR and Western blotting were applied to detect the gene and protein expression of Lhx8 in each group.The choline acetyltransferase ( ChAT)/Lhx8 double labeled cells in subgranular zone ( SGZ) of hippocampus in each group were detected by immunofluorescence .Results The expression of Lhx8 gene and protein in the transected , NGF group and especially in the transected combined with NGF group was obviously higher than in the control group .The number of ChAT/Lhx8 double labeled cells in the NGF group and the transected combined with NGF group was obviously more than in the control group and transected group . Conclusion The hippocampal neuroregeneration which induced by NGF intracerebroventricular injection was associated with the higher expression of Lhx8.

Objective To investigate the effect of Jagged1 on hippocampal radial glial cells (RGCs) proliferation and neuronal differentiation in vitro.Methods Hippocampal RGCs were cultured in vitro, the agonist Jagged1 and(or) inhibitor DAPT of Notch signaling were added into the culture medium , and then the cells were divided into control group , Jagged1 group, Jagged1 combined with DAPT group and DAPT group .CCK-8 regent was used to detect cells ’ vitality;immunofluorescent was used to detect the number of BLBP /Ki67 double labeled cells and differentiated microtubule associated protein-2(MAP-2) positive cells.Results Cell vitality in Jagged1 group was obviously higher than that of the other groups .The number of BLBP/Ki67 double labeled cells and differentiated MAP-2 positive cells were more than other groups.Conclusion Jagged1 promotes the proliferation and neuronal differentiation of hippocampal RGCs in vitro.

Objective: To investigate the clinical outcome of distraction osteogenesis in the treatment of pediatric maxillofacial fractures. Methods: From November 2017 to November 2018, 6 cases of maxillofacial fractures were treated, including 3 cases of maxillary fracture and 3 cases of mandibular fracture. All of them were associated with facial asymmetry of different severity, disordered occlusion and displacement of fracture segments. The distraction osteogenesis device was used to fix the fracture during the operation. The distraction was initiated after 1 day latency period, and proceeded at approximately 0.5 mm each time, twice a day, until the normal occlusal relationship was achieved. Distractor was maintained for 1 month after distraction, and then removed. The degree of fracture healing, the mouth opening and the occlusal status at the fracture site were evaluated during follow-up, and the corresponding curative effect was observed and evaluated. Results: All fractures were healed well, without infection, dislocation, or pseudo-joint formation after 1 month. The distraction duration is 15-20 days and the distraction distance is 15-20 mm. At the end, the occlusal relationship was normal, and the mouth opening was not limited. The follow-up period was from 1 month to 1 year. The maxillofacial appearance of the children was normal, and the maxillofacial development was not significantly affected. Conclusions Distraction osteogenesis is an effective and controllable method for child maxillofacial fracture. It can be used as a supplement to the conventional treatment of maxillary and mandibular fracture in children.

Objective: To investigate a new method to treat unilateral coronal synostosis. Methods: 2 cm-wide osteotomy was performed over the fused unilateral coronal suture.Oblique osteotomy was performed along the supraorbital rim to do a frontal suture of the glabella (patients less than one year old) or on the opposite side of the supraorbital rim (patients over one year old) after performing a suturectomy of the effected coronal suture. Two internal distraction devices were subsequently placed across the osteotomized, fused coronal suture. At the top of the unfused metopic suture, bring two holes and fixing with 4-0 absorbable suture.Finally, the cranium pieces were divided into 2 pieces and placed in the middle of the frontal bone using biological glue or titanium screws. Five days after the operation, a 0.6 mm distraction was performed twice per day. The distraction was removed 6 months after distraction reaching 30—60 mm. Results: Internal distraction osteogenesis with supraorbital oblique osteotomy was performed in eleven patients suffering from unilateral coronal synostosis from 2014.1 to 2017.1. All the patients had no abnormalities in nerve system, such as cephalomeningitis/ fistaul of cerebrospinal fluid/ epidural hematoma and so on.The distractor was not exposed. The width of extension ranged from 25—39 mm. One patient had infection in the rods around the distraction during the period of fixed, but was cured with antibiotic treatment and got an ideal extension eventually. The rest of 10 patients obtained the expected result . During a mean follow-up period of 12 months (5 to 26 months), all patients were presented with satisfactory cosmetic and functional result . CVAI were close to normal. Conclusions Treating unilateral coronal synostosis by internal distraction osteogenesis with supraorbital oblique osteotomy reduces the necessity offrontal and supraorbital osteotomy, and the exposure of the base of anterior cranial fossa, renders a safe and effective approach.

Objective: To evaluate the effect of three-dimensional Z-shaped osteotomy with cranium distraction osteogenesis for unilateral lambdoid synostosis. Methods: Imaging the skull as a cuboid, we designed the affected lambdoid suture as the middle line of letter Z, the osteotomy line paralleling to the sagittal suture or extending downward posterior cranial fossa as the two horizontal lines of Z to obtain the three-dimensional Z-shaped osteotomy lines. Two or three distraction devices were installed after removing the cranial bone at the premature fused suture with a width of 2.0-2.5 cm. Since the 5th day after operation, distraction was performed at the rate of 0.6 mm each day, twice a day, until the distraction distance reached 2.0-4.5 cm. Finally, we removed the distractors after fixation for three months. Results: Eleven cases of unilateral lambdoid synostosis were treated successfully. No infection or bleeding happened except for one case with distractor entrance scalp infection. No complications, including the fixed screws displacement, penetrating the cranium and the dura mater or distraction devices retracting, occurred. During a mean follow-up period of 24 months (5 to 36 months), all patients were satisfied with the cosmetic and functional result. Conclusions Three-dimensional Z-shaped osteotomy with cranium distraction osteogenesis can not only extend upward to raise the cranial height, but also lengthen backward to singularize the occipital carina, forming a well-pleasing occipitalia. Z shape stabilizes the whole plate, making the separated cranial bones not easy to retract. Therefore, using this procedure is effective and mini-invasive, and especially suitable for young infant.

Objective: To assess the therapeutic effect of management for lymphatic malformation(LM)in infants. Methods: This retrospective study recruited clinical data of 996 patients with LM from June 2004 to July 2015 in our center. All patients were diagnosed as LM after ultrasound, CT or MR scan. All patients were divided into Group 1 (427 patients, treated by endoscopic LM partial resection combined with cautery and postoperative intratumoral negative pressure and absolute ethyl alcohol), Group 2(239 patients, treated by combined pinyangmycin and dexamethasone injection), Group 3 (330 patients, treated by surgical resection only). The clinical effects were observed in three groups, and therapeutic effect differences in gender, age, maximum diameter, location, range, histological typing, lymph property and treatments were analysed in cervicofacial LM. Results: Group 1: 333 patients were cured (78.0%), Group 2: 165 patients were cured(69.0%), Group 3: 238 patients were cured (72.1% ). The difference in cure rate between Group 1 and Group 2 or between Group 1 and Group 3 was significant(P<0.05). The number of patients with ≥2 treatments in Group 3 was significantly less than that in other two groups. The cure rate of LM in cervicofacial area was significantly lower than that in other parts of body (P<0.05). In cervicofacial LM patients, the therapeutic effect differences in maximum diameter, range, histological typing, lymph property were statistically significant (P<0.05). At the multivariable logistic regression analysis, LM range as well as histological typing were independent factors influencing the therapeutic effect (P<0.05). Conclusions Both treatment of endoscopic LM partial resection combined with cautery and postoperative intratumoral negative pressure and absolute ethyl alcohol, and surgical resection have good therapeutic effect on LM in infants. LM range as well as histological typing are important factors independently influencing the therapeutic effect of cervicofacial LM in infants.

Objective: To investigate the treatment of nasal deformity in patients with unicoronal craniosynostosis. Methods: In patients over 6 months old, the nasal bones were wedge-removed without fixation. The management of all patients with unicoronal craniosynostosis was distraction osteogenesis of pedicled unilateral frontal bone flap. Results: Postoperative extended distance of the frontal bone was 28—41 mm (mean, 35.4 mm). After extension, three-dimensional reconstruction of cranial CT was carried out, which showed that CVAI was 0.8—1.2 (mean, 0.98), tending to normal. After discharge, dynamic cranial braces were put on for 1 year. Postoperatively, the children were followed up for 8—36 months (mean, 28 months). The shape and nasal deformity of all children were improved compared with those before surgery. Conclusions Nasal wedge resection should be used to correct nasal deformity in children over 6 months with unicoronal craniosynostosis.

We treated a neonate who was diagnosed as rapidly involuting congenital hemangioma (RICH). With a review on the clinical manifestation, differential diagnosis, and treatment of RICH that associated with thrombocytopenia(TP)and coagulopathy(CP)and received platelet transfusions and embolization therapy including absolute ethanol and polidocanol in our hospital in March of 2015. The platelets and coagulation function soon returned to normal, the tumor involuted significantly. Surgical excision was proceeded subsequently. The platelets returned to normal level after a one-time platelet transfusion, meanwhile, multiple reexaminations of blood coagulation function were normal. Postoperatively, incision wound healed well. There was no recurrence, and the functional recovery of upper limb was satisfactory. RICH is a rare type of vascular tumor which may present with TP and CP similar to KHE-KMP in the neonatal period. More attention should be addressed to identify these two diseases, because the treatment and prognosis of which have significant differences. Consumption of coagulation factors with milder platelet decrease may also be a complication of slow flow venous or venolymphatic malformations.

Objective: To explore the feasibility of mandibular distraction for the treatment of airway obstruction in neonates with Braddock-Carey syndrome. Methods: From August 2007 to November 2017, 6 neonates with Braddock-Carey syndrome were treated in our center. All patients experienced the combined thrombopoietic treatment before operation. Bilateral oblique mandibular osteotomy was performed by extraoral approaches and the distraction was initiated on postoperative day 3 at a rate of 1.2 mm/d. It usually took around 2 weeks to get required length of distraction. Distractor was removed after 3-months fixation at second stage. Results: The distraction distance of 6 cases was from 12 to 20 mm. The CT scan showed a widening gap of the airway by 6-10 mm, and the average width was around 7 mm. 5 patients had no bleeding and infection after the operation, while one patient experienced bleeding for 4 times treated by regional compression. All neonates felt painful during distraction, and got relieved after reduction of the frequency and pace. With mandibular advancement, all patients′ face type improved, while paroxysmal bruising, inspiratory dyspnea and difficulty in breastfeeding disappeared. After 6 months of follow-up, all patients′ weight and face type were basically the same as normal children. Conclusions With the treatment of thrombocytopenia before operation, complete hematischesis during the operation, and postoperative treatment of thrombocytopenia, mandibular distraction osteogenesis was a feasible and safe management of upper airway obstruction in neonates with Braddock-Carey syndrome.